beyond the basics: the impact of zika virus on vision and hearing€¦ · · 2017-09-07beyond the...
TRANSCRIPT
Beyond the Basics The Impact of Zika Virus on Vision and Hearing
AAP Webinar Series on Zika Virus SyndromeTuesday September 5 2017 at 100pm ET1200pm CT
OBJECTIVES
After completion of this webinar participants will be able to
1 Describe the vision and hearing findings seen in infants born with Congenital Zika Virus Syndrome
2 Understand the landscape of research on the Zika virusrsquo impact on vision and hearing
3 Know what guidance for evaluation treatment and long term care a pediatrician can use when seeing a patient with possible or confirmed Congenital Zika Virus Syndrome
TECHNICAL SUPPORT
bull Type issue into the chat feature
bull Call 800-843-9166
bull Email supportreadytalkcom
Q amp Abull Submit questions at any time through the chat box
bull Over the phone call 888-632-5004 ID 987450
bull Dial 1 on your phone to ask a live question
PRA CREDITS STATEMENTbull The American Academy of Pediatrics (AAP) is accredited by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing medical education for physicians
bull The AAP designates this live activity for a maximum of 10 AMA PRA Category 1 Credit(s)trade Physicians should claim only the credit commensurate with the extent of their participation in the activity
bull This activity is acceptable for a maximum of 10 AAP credits These credits can be applied toward the AAP CMECPD Award available to Fellows and Candidate Members of the American Academy of Pediatrics
bull The American Academy of Physician Assistants (AAPA) accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credittrade from organizations accredited by ACCME Physician assistants may receive a maximum of 10 hours of Category 1 credit for completing this program
bull This program is accredited for 10 NAPNAP CE contact hours of which 0 contain pharmacology (Rx) content (0 related to psychopharmacology) (0 related to controlled substances) per the National Association of Pediatric Nurse Practitioners (NAPNAP) Continuing Education Guidelines
bull Successful completion of this CME activity which includes participation in the activity with individual assessments of the participant and feedback to the participant enables the participant to earn 1 MOC points in the American Board of Pediatricsrsquo (ABP) Maintenance of Certification (MOC) program It is the CME activity providerrsquos responsibility to submit participant completion information to ACCME for the purpose of granting ABP MOC credit
FACULTY
Lisa Hunter PhD FAAAScientific Director for AudiologyCommunication Sciences Research CenterCincinnati Childrenrsquos Hospital Medical CenterCincinnati OH
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OBJECTIVES
After completion of this webinar participants will be able to
1 Describe the vision and hearing findings seen in infants born with Congenital Zika Virus Syndrome
2 Understand the landscape of research on the Zika virusrsquo impact on vision and hearing
3 Know what guidance for evaluation treatment and long term care a pediatrician can use when seeing a patient with possible or confirmed Congenital Zika Virus Syndrome
TECHNICAL SUPPORT
bull Type issue into the chat feature
bull Call 800-843-9166
bull Email supportreadytalkcom
Q amp Abull Submit questions at any time through the chat box
bull Over the phone call 888-632-5004 ID 987450
bull Dial 1 on your phone to ask a live question
PRA CREDITS STATEMENTbull The American Academy of Pediatrics (AAP) is accredited by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing medical education for physicians
bull The AAP designates this live activity for a maximum of 10 AMA PRA Category 1 Credit(s)trade Physicians should claim only the credit commensurate with the extent of their participation in the activity
bull This activity is acceptable for a maximum of 10 AAP credits These credits can be applied toward the AAP CMECPD Award available to Fellows and Candidate Members of the American Academy of Pediatrics
bull The American Academy of Physician Assistants (AAPA) accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credittrade from organizations accredited by ACCME Physician assistants may receive a maximum of 10 hours of Category 1 credit for completing this program
bull This program is accredited for 10 NAPNAP CE contact hours of which 0 contain pharmacology (Rx) content (0 related to psychopharmacology) (0 related to controlled substances) per the National Association of Pediatric Nurse Practitioners (NAPNAP) Continuing Education Guidelines
bull Successful completion of this CME activity which includes participation in the activity with individual assessments of the participant and feedback to the participant enables the participant to earn 1 MOC points in the American Board of Pediatricsrsquo (ABP) Maintenance of Certification (MOC) program It is the CME activity providerrsquos responsibility to submit participant completion information to ACCME for the purpose of granting ABP MOC credit
FACULTY
Lisa Hunter PhD FAAAScientific Director for AudiologyCommunication Sciences Research CenterCincinnati Childrenrsquos Hospital Medical CenterCincinnati OH
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
TECHNICAL SUPPORT
bull Type issue into the chat feature
bull Call 800-843-9166
bull Email supportreadytalkcom
Q amp Abull Submit questions at any time through the chat box
bull Over the phone call 888-632-5004 ID 987450
bull Dial 1 on your phone to ask a live question
PRA CREDITS STATEMENTbull The American Academy of Pediatrics (AAP) is accredited by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing medical education for physicians
bull The AAP designates this live activity for a maximum of 10 AMA PRA Category 1 Credit(s)trade Physicians should claim only the credit commensurate with the extent of their participation in the activity
bull This activity is acceptable for a maximum of 10 AAP credits These credits can be applied toward the AAP CMECPD Award available to Fellows and Candidate Members of the American Academy of Pediatrics
bull The American Academy of Physician Assistants (AAPA) accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credittrade from organizations accredited by ACCME Physician assistants may receive a maximum of 10 hours of Category 1 credit for completing this program
bull This program is accredited for 10 NAPNAP CE contact hours of which 0 contain pharmacology (Rx) content (0 related to psychopharmacology) (0 related to controlled substances) per the National Association of Pediatric Nurse Practitioners (NAPNAP) Continuing Education Guidelines
bull Successful completion of this CME activity which includes participation in the activity with individual assessments of the participant and feedback to the participant enables the participant to earn 1 MOC points in the American Board of Pediatricsrsquo (ABP) Maintenance of Certification (MOC) program It is the CME activity providerrsquos responsibility to submit participant completion information to ACCME for the purpose of granting ABP MOC credit
FACULTY
Lisa Hunter PhD FAAAScientific Director for AudiologyCommunication Sciences Research CenterCincinnati Childrenrsquos Hospital Medical CenterCincinnati OH
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
PRA CREDITS STATEMENTbull The American Academy of Pediatrics (AAP) is accredited by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing medical education for physicians
bull The AAP designates this live activity for a maximum of 10 AMA PRA Category 1 Credit(s)trade Physicians should claim only the credit commensurate with the extent of their participation in the activity
bull This activity is acceptable for a maximum of 10 AAP credits These credits can be applied toward the AAP CMECPD Award available to Fellows and Candidate Members of the American Academy of Pediatrics
bull The American Academy of Physician Assistants (AAPA) accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credittrade from organizations accredited by ACCME Physician assistants may receive a maximum of 10 hours of Category 1 credit for completing this program
bull This program is accredited for 10 NAPNAP CE contact hours of which 0 contain pharmacology (Rx) content (0 related to psychopharmacology) (0 related to controlled substances) per the National Association of Pediatric Nurse Practitioners (NAPNAP) Continuing Education Guidelines
bull Successful completion of this CME activity which includes participation in the activity with individual assessments of the participant and feedback to the participant enables the participant to earn 1 MOC points in the American Board of Pediatricsrsquo (ABP) Maintenance of Certification (MOC) program It is the CME activity providerrsquos responsibility to submit participant completion information to ACCME for the purpose of granting ABP MOC credit
FACULTY
Lisa Hunter PhD FAAAScientific Director for AudiologyCommunication Sciences Research CenterCincinnati Childrenrsquos Hospital Medical CenterCincinnati OH
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FACULTY
Lisa Hunter PhD FAAAScientific Director for AudiologyCommunication Sciences Research CenterCincinnati Childrenrsquos Hospital Medical CenterCincinnati OH
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FACULTY
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FACULTY
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FACULTY
Marcia Tartarella MD PhD FAAPPediatric OphthalmologistFederal UniversitySatildeo Paulo Brazil
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FACULTY
Meg Fisher MD FAAPInfectious Disease PediatricianMedical DirectorUnterberg Childrenrsquos Hospital at MonmouthMedical Center
Long Branch New Jersey
MemberAAP Disaster Preparedness Advisory Council
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
DISCLOSURES
bull The presenters have no relevant financial relationships with the manufacturers(s) of any commercial products(s) andor provider of commercial services discussed in this activity
bull The presenters do not intend to discuss an unapprovedinvestigative use of a commercial productdevice in this presentation
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
WELCOME REMARKS
Meg Fisher MD FAAP
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OPHTHALMOLOGICAL FINDINGS IN CONGENITAL
ZIKA SYNDROME
Marcia Beatriz Tartarella MD PhD FAAPSOPLA President 2015-2017
Satildeo Paulo - Braziltartarellayahoocom
wwwmarciatartarellacombr
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OPHTHALMOLOGICAL FINDINGS
bull First report January 2016
bull Macular Atrophy
bull Microcephaly
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
Zika Embryopathy Evaluation and Management Recommendations for
Ophthalmologists SOPLA Guidelines
February 2016
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
REFERENCES IN BRAZIL
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OPHTHALMOLOGICAL FINDINGS DETECTED IN 36 OF PATIENTS
1 Chorioretinal Atrophy
(circular whitish lesions or colobomatous-like) 60
2 Focal retinal pigment mottling 70
3 Optic Nerve anomalies hypoplasia double-ring sign pallor cupping 40
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJournal of American Association for Pediatric Ophthalmology and Strabismus Volume 21 Issue 4 August 2017 Pages 300-304e1J AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024 [Epub ahead of print]
Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB4
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
Infants without microcephaly ocular findings
OCULAR FEATURES
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
CHORIORETINAL ATROPHY
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
MACULAR ATROPHY
CHORIORETINAL ATROPHY
Macular Atrophy Colobomatous-like
whitish circumscribed flat lesionsSharp edges
No inflammatory signsORIORETINAL ATROPHY
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull Characterization of Congenital Zika Syndrome (CZVS) = Major Sign of CZVS
bull Macular atrophy is an important clinical criteria for CZVS diagnosis when laboratory testing is not available
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FOCAL RETINAL PIGMENT MOTTLING
RETINAL PIGMENTARY MOTTLING
RETINAL PIGMENTARYMOTTLING
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OPTIC NERVE ABNORMALITIES
Optic nerve findings include
bull Hypoplasia with the double-ring signbull Pallorbull Increased cup-disc ratio
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
CONGENITAL ZIKA SYNDROME
Red Reflex Test - RRT is NORMAL in cases of CZVS
bull 72 patients with microcephaly tested normal RRT
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OPHTHALMOLOGICAL FINDINGS
bull Congenital Cataract = 2
bull Microftalmia = 1
bull Congenital Glaucoma = 1
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
VISUAL IMPAIRMENT LOW VISION
bull All children tested in this group presented visual impairment
bull In case of ocular involvement or cerebralcortical visual impairment the baby is referred to Early Visual Intervention and Visual Rehabilitation Centers
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull Corticalcerebral visual impairment may occur from brain damage without ophthalmologic lesions
bull Low vision may occur without microcephaly
bull Strabismus may occur without microcephaly and without ocular abnormalities Strabismus and Nystagmus are predictors of visual impairment
bull Strabismus and Nystagmus have to be observed in all pediatric and ophthalmic visits
VISUAL IMPAIRMENT LOW VISION
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
STRABISMUS X CZVS
Strabismus
IMPORTANT Early onset Strabismus mean age = 4 months
14 CZVS x 4 normal children
9 Nystagmus
The visual system in infants with microcephaly related to presumed congenital Zika syndromeJ AAPOS 2017 Jun 23 pii S1091-8531(16)30736-4 doi 101016jjaapos201705024Verccedilosa I1 Carneiro P1 Verccedilosa R1 Giratildeo R1 Ribeiro EM2 Pessoa A2 Almeida NG3 Verccedilosa P1 Tartarella MB
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
RECOMMENDATIONS
Protection for all medical personal should be instituted for the ophthalmological examination to minimize the risk of tear-born contamination or spread
J Sun Zhong H Wu et alPresence of Zika virus in conjunctival fluid
JAMA 134 (2016) pp 1330-1332
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
In newborns or babies with microcephaly or Presumed Congenital Zika Syndrome the ophthalmologic evaluation should include Indirect Ophthalmoscopy or Digital imaging with Retcam
RECOMMENDATIONS
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
Evaluation of Visual Acuity and refraction during the first year of life
Prescription glasseseye patch if necessary
RECOMMENDATIONS
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
RECOMMENDATIONS
Ophthalmological Evaluation repeat every 6 months
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull First eye evaluation within 30 days of life
bull Ophthalmic evaluation every 6 months
bull Check refractive status and the need of glasses
bull Evaluation of ocular abnormalities macular atrophy optic nerve hypoplasia strabismus nystagmus refraction and visual acuity
bull Refer to early visual intervention or visual rehabilitation centers
SUMMARY
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
Congenital Zika Virus and Hearing Loss
Lisa L Hunter PhD FAAACincinnati Childrenrsquos Hospital and the University of
Cincinnati Departments of Otolaryngology and Communication Sciences and Disorders
American Academy of AudiologyLisahuntercchmcorg
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
FIRST CASE OF CONGENITAL ZIKA-RELATED
SENSORINEURAL HEARING LOSS
LEAL MUNIZ NETO ET AL BRAZ J OTO 2017
bull Newborn from twin pregnancy delivered by C-section in 37th week in Pernambuco Brazil Capture ELISA was positive for ZIKV IgM in CSF
bull Microcephaly and clubfoot were present Hearing screening with otoacoustic emissions were absent
bull Auditory Brainstem Response (ABR) to clicks was absent in the left ear and present at a profound level in the right ear
bull CT scan showed diffuse bilateral cerebral parenchyma reduction ventriculomegaly simplified gyri and calcifications in the basal ganglia and subcortex
httpswwwnytimescom20161122healthzika-microcephaly-babieshtml
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
ESTABLISHING GUIDELINES IN THE FACE OF
UNCERTAINTY ABOUT HEARING LOSS AND RISK
FOR PROGRESSIVE AND DELAYED ONSET LOSSbull In July 2016 a working group met at CDC in Atlanta convened by the AAP The
goal was to set preliminary guidance for surveillance of newborns who tested positive for the Zika virus
bull Guidance was considered based on knowledge of similar viruses (eg CMV) and effect on hearing No studies were yet available beyond sporadic cases
bull Guidance was developed to be cautious and consider the risk for delayed onset and progressive loss in infants with and without signs of CZVS
bull The Joint Committee on Infant Hearing Guidelines were examined and followed to a large extent with additions for infants who tested positive but had no signs of Zika syndrome at birth
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
MANY QUESTIONS REMAINbull Should infants who have CZVS but pass screening at birth be followed with
repeat hearing testing in the first year Is there a risk for progressive hearing loss
bull Should infants who test positive for Zika but have no symptoms at birth be monitored with an additional hearing test and when
bull What form should the later hearing screens take (OAEs ABR behavioral)
bull Considerations for developmental delay and age possible cortical and neural effects on auditory system
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
DUAL SENSORY IMPAIRMENT IN INFANTS
(DEAF-BLIND)
bull CZVS is now one of the causes of combined visual and hearing disability along with CMV Rubella Usher and other syndromes
bull In many cases speech is unlikely to develop to a functional level or to be the most effective means of communication The acquisition of symbolic understanding necessary for any form of language development is also a challenge
bull Resources httpwwwdeafblindinformationorgaucongenital-deafblindness
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
DUAL SENSORY IMPAIRMENT COMMUNICATION
SYSTEMS
bull The causes of congenital deaf-blindness are varied thus cognitive and motor abilities need to be considered
bull Choices include modified and tactile sign language body signs touch cues
bull Avoiding sudden and startling movements providing repetition consistency schedule are important
bull Providing enhanced visual cues watching expression for acknowledgment asking caregiver for input on typical responses
bull Provide parent resources httpwwwdeafblindinformationorgauwp-contentuploads201601communication-fact-sheets-for-parentspdf
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
Audiological Aspects
Mariana Leal MD PhDOtolaryngologist and ProfessorDepartment of SurgeryFederal University of PernambucoRecife Brazil
Lilian Muniz MDProfessor of AudiologyFederal University of PernambucoRecife Brazil
munizliliangmailcommarianaclealhotmailcom
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
PERNAMBUCO BRAZIL
Pernambuco population 9410336
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
MICROCEPHALY
PERNAMBUCO 2015-2017
bull MICROCEFALIA NO ESTADO
201520162017
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull Screening bull Hearing Assessment
HEARING LOSS
American Academy of Pediatrics Joint Committee on Infant Hearing (2007) Multiprofessional Committee on Hearing Health (2010 Brazil)
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
CROSS-CHECK PRINCIPLEJerger and Hayes 1976
OBJECTIVETESTS
BEHAVIORAL
TESTS
HEARING ASSESSMENT
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
PUBLISHED DATA
469 (58)
One excluded ototoxic exposure
Screening ABRn=70
FAILED16
NORMAL54
FAILED8
NORMAL8
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
OUR POPULATION
Congenital Zika Virus Syndrome
ZIKV-specific IgM in CSF (ELISA)
Characteristic radiologic findings (CTMRI)
Exclude the main differential diagnoses of CZS
(STORCH)
89 with microcephaly
More Severe CZVS
400255
139
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
NOT PUBLISHED DATA AUGUST 2017
bull Confirmed CZS
bull Excluded other causes of microcephaly and hearing loss risks indicators
138
bull 6 Sensorineural hearing loss
bull 3 Conductive hearing loss
6138 (43)
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
SENSORINEURAL HEARING LOSS
Case No Diagnosis
1 Bilateral profound SNHL
2 Unilateral profound SNHL
3 Bilateral mild SNHL
4Bilateral SNHL
(Moderate on right profound on left)
5 Bilateral moderate SNHL
6 Bilateral moderate SNHL
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
VARIABLES SNHL Normal P value
Mean head circumference 273 cm 291 cm 0040
Small for gestational age 333 123 0181
Maternal rash duringpregnancy
667 767 0628
SexMale 667 421
0402Female 333 579
Mean age at test (days) 14257 1375 0762
HEARING LOSS
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CONGENITAL ZIKA VIacuteRUS
SYNDROME (CZVS)
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Inclusion criteria (N=73138)
ABR wave five present at 35 dB nHL
(Passed at newborn hearing screening)
bull Evaluation method
ABR at 80 dB nHL ndash Verify latencies
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
N=73
N=3 First ABR
SecondABR
ThirdABR
02 Abnormal Normal Normal
01 Abnormal Abnormal Abnormal
AUDITORY NERVE CONDUCTION IN CHILDREN WITH CZVS
bull Neural conduction seems to be normalthrough Brainstem and the stimulus isprobably going towards primaryauditory cortex
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull Population (N=88)
Gender (male 591 - female 409 )
Age ( 6 ndash 12 months)
bull Inclusion criteria
Detectable wave V at 35 dB nHL ABR
(Passed at newborn hearing screening)
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
bull Procedures
Secondary data from a principal study
Questionnaire applied to parents or caregivers
(Questionnaire to monitor auditory and language
developing during the first year of life
[Alvarenga et al 2013])
AUDITORY AND LANGUAGE DEVELOPMENT IN CHILDREN WITH CZVS
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
RESULTS
Variables n
TOTAL 88 1000
Auditory Abilities
Normal 47 534
Altered 41 466
Language Abilities
Normais 11 125
Alterados 77 875
Positive association = microcephaly severity and motor abilities
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
DATA FOLLOW-UP
bull 1 child with HL on the 1st hearing assessment
Results had normalized after one year (maturation)
bull Progressive hearing loss was not found
bull Delayed hearing loss was not found
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
INTERVENTION
bull Hearing Aids
bull Cochlear Implant
bull Speech Therapy
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
NEXT STEPS
bull Cortical brainstem potential
bull Correlation between auditory assessment and auditory cortex images (MRI)
bull Hearing follow up expanded until 5 years of age
Normal image (MRI) CZS image (MRI)
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
SUMMARY REMARKS
Meg Fisher MD FAAP
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
CMEMOC CREDIT
CMEMOC Credit
bull Complete the post activity survey
bull Only physicians can claim MOC Part 2
bull Physicians must identify ABP ID number
AAP staff will email each person claiming CMEMOC 2 credit with their certificate of completion Email DisasterReadyaaporg with any questions
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
QUESTIONS
bull Dial 1 on your phone to ask a live question
bull Phone 888-632-5004
bull Conference ID 987450
bull Can ask questions through chat box in lower left corner AAP staff or presenters will address unanswered questions via e-mail after the call
Please e-mail DisasterReadyaaporg to
receive info on future events or
follow-up as needed
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services
This webinar is supported by cooperative agreement number 6NU38OT000167-04-06 funded by the Centers for Disease Control and
Prevention Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease
Control and Prevention or the Department of Health and Human Services