development in hearing impaired.rev
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Development in the Development in the Hearing Impaired ChildHearing Impaired Child
Dr Varsha Atul ShahDr Varsha Atul Shah
Associate ConsultantAssociate Consultant
Department of Neonatal & Department of Neonatal & Developmental Medicine, SGHDevelopmental Medicine, SGH
Visiting ConsultantVisiting Consultant
K K Hospital,Child Development K K Hospital,Child Development UnitUnit
Colorado Newborn Screening Colorado Newborn Screening ProgramProgram
Per 100 000 birthsPer 100 000 births
Congenital HLCongenital HL 260 (200 bilateral)260 (200 bilateral)
HypothyroidismHypothyroidism 2525
PKUPKU 77
CFCF 5050
Pediatrics Jan 1998Pediatrics Jan 1998
SeveritySeverity dBdB
MildMild 27 - 4027 - 40
ModerateModerate 41 - 5541 - 55
Moderately-severeModerately-severe 56 - 7056 - 70
SevereSevere 71 - 9071 - 90
ProfoundProfound 91 91American National Standards Institute American National Standards Institute 19691969
Classification of Classification of Severity of Hearing Severity of Hearing
ImpairmentImpairment
Hearing Impairment In InfancyHearing Impairment In Infancy
Environmental Environmental CausesCauses (50%)(50%)
Genetic Causes Genetic Causes (HHI)(HHI) (50%)(50%)
Syndromic (30%)
Non Syndromic (70%)
Environmental CausesEnvironmental Causes
Perinatal infectionsPerinatal infections CMV, Rubella, other IUICMV, Rubella, other IUI
MeningitisMeningitis bacterialbacterial
PrematurityPrematurity multiple risk factorsmultiple risk factors
HyperbilirubinaemiaHyperbilirubinaemia OtotoxicityOtotoxicity
aminoglycosides, loop diureticsaminoglycosides, loop diuretics
Hereditary Hearing Hereditary Hearing Impairment (HHI)Impairment (HHI)
CongenitalCongenital HHI HHIPresent at birthPresent at birth
Delayed onsetDelayed onset HHI HHIDevelop later in lifeDevelop later in life
Syndromic:
both conductive & SNHL
Non Syndromic:
most often SNHL
Syndromic HHISyndromic HHI
Chromosomal disordersChromosomal disorders Down SyndromeDown Syndrome TrisomiesTrisomies
Non Chromosomal Non Chromosomal disordersdisorders Noonan’s syndrome Noonan’s syndrome CraniosynostosisCraniosynostosis Pendred SyndromePendred Syndrome Osteogenesis Osteogenesis
ImperfectaImperfecta Charcot-Marie ToothCharcot-Marie Tooth Waardenburg SyndromeWaardenburg Syndrome
Non-Syndromic HHI: the Non-Syndromic HHI: the geneticsgenetics
Gene mutation Gene mutation
Genetic heterogeneityGenetic heterogeneity
““DDeaeaFNFNess” gene lociess” gene loci AR (77%)AR (77%) DFNB loci (1-33)DFNB loci (1-33)
AD (22%)AD (22%) DFNA loci (1-41)DFNA loci (1-41)
X-linked (1%)X-linked (1%) DFN loci (1-8)DFN loci (1-8)
Mitochondrial (< 1%)Mitochondrial (< 1%) maternal maternal inheritance inheritance with with susceptibility to susceptibility to aminoglycosidesaminoglycosides
Undetected Hearing LossUndetected Hearing Loss
Hearing loss is a Hearing loss is a
hidden disability hidden disability
which has long which has long
term consequences term consequences
on development if on development if
the appropriate the appropriate
intervention is not intervention is not
instituted earlyinstituted early
Screening
Identification
Follow up
Intervention
Outcome
The Importance of Hearing The Importance of Hearing
Hearing is important forHearing is important for
1.1. LanguageLanguage development development especially in the first especially in the first
5 years of life5 years of life
2.2. SpeechSpeech development development Speech perceptionSpeech perception Speech production Speech production
(including (including
articulation, timing articulation, timing
and prosody)and prosody)
Unilateral or Mild Bilateral HLUnilateral or Mild Bilateral HL Relatively minor disabilityRelatively minor disability Speech recognition & understanding under Speech recognition & understanding under
adverse conditions (e.g. classroom noise)adverse conditions (e.g. classroom noise)
AttentionAttention
BehaviourBehaviour
Overall educational Overall educational achievementachievement
Psychosocial well beingPsychosocial well being
Children with minimal sensorineuralChildren with minimal sensorineural
hearing loss:hearing loss:
Bess FH, Ear Hear Oct 1998Bess FH, Ear Hear Oct 1998
Poorer comprehension & communicationPoorer comprehension & communication
Impairment in functional statusImpairment in functional status
37% failed at least 1 grade37% failed at least 1 grade
Moderate-profound HLModerate-profound HL
Impairment of speech/language Impairment of speech/language developmentdevelopment
Reduction of academic achievementReduction of academic achievement
Disturbance in social & emotional Disturbance in social & emotional development development
Problems faced by HI Problems faced by HI ChildrenChildren
HearingImpairedChildren
LanguageIssues
Non – LanguageIssues
ConcomitantDisabilities
Language Related Language Related IssuesIssues
1.1. When adults speak with the HI childWhen adults speak with the HI child They tend to give less verbal praise and They tend to give less verbal praise and
become less stimulatingbecome less stimulating
2.2. HI children have reciprocal HI children have reciprocal communication issuescommunication issues They use attention-seeking statements or They use attention-seeking statements or
methods to enter a conversation methods to enter a conversation They have difficulty deciding when to enter a They have difficulty deciding when to enter a
conversationconversation
Language Related Language Related IssuesIssues
3.3. When HI children seek clarification,When HI children seek clarification, They tend to repeat rather than reviseThey tend to repeat rather than revise
4.4. HI children have limited vocabulary:HI children have limited vocabulary: Vocabulary of the HI is 0 - 9Vocabulary of the HI is 0 - 9
words at 18 monthswords at 18 months Vocabulary of normally-hearingVocabulary of normally-hearing
children = 20 – 50 words children = 20 – 50 words
Language Related Language Related IssuesIssues
5.5. HI children have poor sentence structureHI children have poor sentence structure
6.6. Children with language difficulties Children with language difficulties
face difficulty in face difficulty in
Learning new skills & conceptsLearning new skills & concepts
Making sense of events aroundMaking sense of events around
Solving problemsSolving problems
Literacy – reading & writing skillsLiteracy – reading & writing skills
Non-Language Non-Language IssuesIssues
1.1. More More physically–tiredphysically–tired (more effort (more effort required)required)
2.2. Emotional outcomes-Emotional outcomes- Poor self-esteem, Poor self-esteem, motivationmotivation
3.3. Socialization skillsSocialization skills Difficulty in making friends & being accepted Difficulty in making friends & being accepted
sociallysocially Often feel rejected & isolatedOften feel rejected & isolated Weaker play skills (especially those associated Weaker play skills (especially those associated
with language and social skills)with language and social skills)
4.4. Behaviour problemsBehaviour problems AggressionAggression ImpulsivityImpulsivity ImmaturityImmaturity Inattention, frustrationInattention, frustration
5.5. Family dynamicsFamily dynamics Challenges in family adjustment (emotional, Challenges in family adjustment (emotional,
financial and time factors) will impact on the financial and time factors) will impact on the child and his/her family - child relationshipchild and his/her family - child relationship
Non-Language IssuesNon-Language Issues
Assessment of Language Assessment of Language
Proper diagnostic hearing assessmentProper diagnostic hearing assessment Proper treatment i.e. aids / CI / AVT / Proper treatment i.e. aids / CI / AVT /
signsign Hearing Age importantHearing Age important Appropriate non verbal and verbal Appropriate non verbal and verbal
language assessments:language assessments: Receptive language skillsReceptive language skills Expressive language skillsExpressive language skills
Concomitant DisabilitiesConcomitant Disabilities
Several disabilities occur more commonly Several disabilities occur more commonly in HI childrenin HI children
Intellectual / cognitive disability (8%)Intellectual / cognitive disability (8%) Based on generalized developmental Based on generalized developmental
delay with poor adaptive or functional delay with poor adaptive or functional skillsskills
Emotional / behavioural problems (4%)Emotional / behavioural problems (4%) Inappropriate, disruptive, aggressive behaviourInappropriate, disruptive, aggressive behaviour
Concomitant DisabilitiesConcomitant Disabilities
Learning disability (9%)Learning disability (9%) Diagnosis based on Diagnosis based on discrepancydiscrepancy
between intellectual potential and between intellectual potential and achievement in reading and writing skillsachievement in reading and writing skills
Average / above average IQ with specific Average / above average IQ with specific deficits in reading/writing (dyslexia)deficits in reading/writing (dyslexia)
Neurological problemsNeurological problems
Visual impairmentVisual impairment
Hearing Impairment & Hearing Impairment & LearningLearning
Previous studies showed that HI children havePrevious studies showed that HI children have Linguistic / language problemsLinguistic / language problems Cognitive / thinking / learning problemsCognitive / thinking / learning problems
Hearing impairment alone Hearing impairment alone does NOT does NOT equate to low intellectual ability or inability to equate to low intellectual ability or inability to learnlearn
With early intervention, children can With early intervention, children can develop good language skillsdevelop good language skills
Early intervention is Early intervention is essentialessential
Neural circuits of auditory system Neural circuits of auditory system rewired by external stimulations rewired by external stimulations very early in lifevery early in life
Pediatric Clinics of North America Feb Pediatric Clinics of North America Feb 19991999
If such stimulations not received at that If such stimulations not received at that time, circuits will not develop fullytime, circuits will not develop fully
Screening
Identification
Follow up
Intervention
Breakdown at any stage
jeopardizes entire effort
Breakdown at any stage
jeopardizes entire effort
Outcome
The UNHS The UNHS programme was programme was piloted at the piloted at the Singapore General Singapore General Hospital in 1998Hospital in 1998
In 2002 the project become In 2002 the project become the National UNHS the National UNHS programme involving all programme involving all public hospitals, polyclinics public hospitals, polyclinics and some private hospitalsand some private hospitals
Echocheck version 2.13.3 ILO, Otodynamics
(July 2000 - )
Echoport ILO 288, Otodynamics
(Nov 1998 - )
Do not only hear me Do not only hear me but also listen to me…but also listen to me…