christine yoshinaga-itano, ph.d. professor university of colorado ... 2013 ear foundation az... ·...

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Christine Yoshinaga-Itano, Ph.D.

Professor

University of Colorado, Boulder

Department of Speech, Language & Hearing Sciences

Allison Sedey, Ph.D.

Rosalinda Baca, Ph.D.

Molly Dalpes, AuD

Kristin Uhler, Ph.D.

University of Colorado Denver Medical Campus

Department of Ear, Nose & Throat & Audiology

At the conclusion of this presentation, the participant will be able to:

1. Identify the prevalence and etiologies of progressive hearing loss by age (from birth through 7 years) and hearing loss.

2. Identify the prevalence and etiologies of acquired hearing loss by age (from birth through 7 years) and hearing loss.

3. Identify the longitudinal language development of children with progressive hearing loss from birth through 7 years as compared to children with stable losses

33 1/3%

1 IN 3 CHILDREN WITH HEARING LOSS HAVE A

ACQUIRED, LATE ONSET OR PROGRESSIVE OR

ACQUIRED HL

Most present AFTER THE AGE OF SIX

NO IDENTIFIED ETIOLOGY 30-40% OF CASES

1) The audiologic report stated that the child

had a progressive loss (or that “the loss had

progressed compared to previous testing”),

2) or if we had multiple audiograms:

2) Two or more frequencies (in the better ear)

were 15dB or more poorer than previous testing

indicated, or

3) One or more frequencies (in the better ear)

was 20 dB or more poorer than previous testing

indicated.

Children whose “worse ear” progressed are not included in this count if there was no progression in the better ear.

Children whose ABR thresholds were significantly different from behavioral thresholds unless their behavioral thresholds progressed or there were multiple ABRs were not included

If differences between ABR and behavioral thresholds were very significant e.g. mild to severe/profound, were recorded as progressive.

Children with acquired hearing loss after the age of 3 years were not included (they could not be included in the longitudinal study which required enrollment in early intervention services

Children with UNHL who progressed to bilateral HL after the age of 3 years

N=135 children

No additional disabilities impacting language/communication development

N=89 children with moderate-severe to profound hearing loss

PROGRESSIVE HEARING LOSS

31% children with hearing aids had progressive HL

34.7% children with cochlear implants had progressive HL

30 of the 146 (20.5%) children had progressive hearing by age 7

AMPLFICATION:

12 of the 30 children used conventional amplification, hearing aids and

18 had cochlear implants.

Degree of HL by age 7: 83% or 25 of the 30 children had either a moderate-severe (20%, N=9), severe (30%, N=9) or profound hearing loss (33.3%, N=10).

0

5

10

15

20

25

30

35

Number

Percent

Genetic: N=6 20%

Cytomegalic Virus: N=4 12%

Unknown N=16 53.3%

Ototoxicity N=2 (6.6%)

Multiple etiologies: N=1 3.3%

Mondini N=1 3.3%

Treacher Collins N=1 3.3%

**Expressive One Word Picture Vocabulary

Test

92 months (25.6 SD) – progressive hearing loss

hearing loss

81 months (27 SD) – stable hearing loss same

degree

11 month difference AT AGE 7

Test of Auditory Comprehension of Language

NO SIGNIFICANT DIFFERENCE

85.6 mo. (19 SD) progressive HL

84.6 mo. (15 SD) stable HL

17 of the 17 children had information about the age of acquisition of the hearing loss.

**58.8% or 10 of the 17 children acquired hearing loss between .5 months of age to 12 months of age.

Of these 10 children 4 children (40%) (23.5% of total) acquired the hearing loss between .5 and 6 months, and

Of the 10 children, 6 (60%) (35% of total) of the children acquired the hearing loss between 7 and 12 months of age.

Missed or acquired?? – most have etiology reported

Another 35.3% or 6 children acquired their hearing losses between 13 and 24 months of age and

1 child acquired hearing loss (5.9%) between 25 and 36 months of age.

0

10

20

30

40

50

60

70

0-6 mo. 7-12 mo. 13-24 mo. 25-36 mo.

Percent

Number

14 of17 children with acquired hearing loss had information about the degree of hearing loss at enrollment into early development/intervention services.

5 of the 17 children had a profound hearing loss (29.4%),

1 had a severe hearing loss (5.9%),

4 had a moderate-severe hearing loss (23.5%),

2 had a moderate hearing loss (11.8%),

1 had a mild hearing loss (5.9%)

1 had a low or high frequency hearing loss (5.9%), and

3 had missing data.

Only 1 of the 17 children had both an acquired and progressive HL

Genetic N=1 7.1%

Cytomegalic virus: N=1 7.1%

Meningitis N=2 14.3%

Unknown N=7 50%

Ototoxicity N=3 21.4%

Missing N=3

Total: 17 acquired hearing losses of 146

participants

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

mild moderate severe profound

Present at birth

Progressive

Acquired

Total P & A

0

5

10

15

20

25

30

35

40

45

Mild Moderate Severe Profound

Percent Acquired+ Progressive HL by degree of HL (4 to 7 years)

Acquired

Progressive

Total

all children with bilateral hearing loss, excludes children with unilateral permanent hearing loss and auditory neuropathy, but includes permanent conductive, sensori-neural and mixed hearing losses, and all degrees of hearing loss between the ages of birth to 36 months who received early intervention services when the children were birth to 36 months of age.

Statistics include children across all cognitive levels including children with additional disabilities.

All children in this database are in English-speaking homes.

10% of all ages had a progressive hearing loss

Ages 1 to 42 months: 6.4% had a progressive

HL

Ages 43-89 months: 18.5% had a progressive

HL

768 of 853 children had stable HL

85 had progressive HLs

0

5

10

15

20

25

30

35

40

45

mild mod mod-sev sev profound

0-42 mo

43-89 mo.

Of the 795 children in the database, 521 had

a reported onset of hearing loss.

380 of the 521 (72.9%) children had onset of

hearing loss from birth.

72 of the children (13.8%) had an acquired

hearing loss

Of the 72 with reported acquired hearing loss, the following etiologies were reported:

hereditary (N=3, 4.2%)

CMV (N=4, 5.6%)

other syndrome (N=1, 1.4%)

meningitis (N=35, 49.3%) *note diff with longitudinal study

high fever (N=1, 1.4%)

trauma (N=1, 1.4%)

unknown etiology (N=13, 18.3%)

ototoxicity (N=6, 8.5%)

other (N=5, 7%). In the “other” category, 1 was reported to be due to EVA (enlarged vestibular aqueduct), 1 from otosclerosis, and 3, the information was not provided.

0

5

10

15

20

25

30

35

40

45

all ages

1 to 42 mo.

43- 89 mo

Centers for Disease Control

AUCD #433

Developmental Outcomes Feasibility Study

AUCD

Association of University Centers on

Disability

Longitudinal data collection also funded

previously: NIH-NIDCD, Office of Education,

Maternal and Child Health

CMV titers at referral or diagnosis (urine

sample – pad in diaper)

Genetic testing of all/most pediatric hearing

loss

Early intervention- diagnostic audiology

collaboration- daily parent Ling 6 sound test

Screening in preschool

EHDI databases include longitudinal

audiological information, acquired at any age

in childhood

Failed NBHS in both ears

Mild SNHL confirmed at 3 weeks by ABR

Since it was a “mild” hearing loss, no follow-up occurred

At age 2;4, referred to EI services for significant speech delay

Hearing loss progressed to flat, moderate SNHL

Began intervention at 2;4

Received amplification at 2;11

CT scan at age 3;6 revealed EVA

CHIP facilitator 1 hour/week

05/16/00 (age 2;5)

Speech, Amplification, Auditory training/listening, Speechreading, Natural gestures, Manually coded English

Mother noted strong desire to use sign because her child, “doesn’t have aids yet so we don’t know if they will help or not. Using signs as a precautionary measure because we will need to communicate with my child if hearing aids don’t help.”

Receptive & Expressive Language = 11-13 months

At this time, mother was doing most of the talking (“teachable moment” for communication strategies)

Mother had 32 “needs” from the family needs survey

12/09/00 (age: 2;11)

Fit binaurally with amplification

Expressive Language: 19 months

64 utterances, 49 sign & 33 spoken MLU = 1.00 for speech & sign

Poor intelligibility

Areas of Concern: (from Minnesota CDI) General development

Gross Motor

Expressive Language

Comprehension

Personal/Social

01/30/03 (age: 4;11)

Expressive One Word Vocabulary-10th

percentile

258 utterances, 53 sign & 248 spoken

Good intelligibility

Mother has learned 389 out of 400 signs

04/26/06

58dB 62dB

08/31/04

Right: 48dB

Left: 57dB

03/19/07

60dB

65dB

10/11/07

67dB

68dB

04/16/08

70dB

72dB

RE: 22dB

LE: 15dB

0

10

20

30

40

50

60

70

80

Left Ear

Right Ear

02/01/05 (age: 7;1)

In DHH classroom 15 hr./wk. & regular

classroom with interpreter 13 hr./wk.

Expressive One Word Vocabulary Test-5th

percentile

Test of Auditory Comprehension of language

total score-39th percentile

Excellent intelligibility

Mother has learned 398 out of 400 signs

01/31/00 (age: 2 years)

Approximates a flat, moderate hearing loss bilaterally

02/04/98 (age 1.5 months)

Approximates a mild hearing loss bilaterally

57 dB 63dB

55 dB 63dB

57dB 65dB

68 dB 72 dB

02/07/02 04/09/02

08/06/02 02/15/05

RE: 13dB

LE: 11dB

0

10

20

30

40

50

60

70

80

Left ear

Right ear

Failed newborn hearing screening

Bilateral SNHL confirmed at 5 weeks

Intervention services & amplification by 3

months

Etiology: CMV

CHIP facilitator 1 hour/week in the home

Communication Approach:

Auditory Oral

Supplemented with some sign language

Goal was spoken language

Click ABR (1.5 months) + 500Hz tone burst (5 months)

Severe rising to moderate SNHL in L

Profound SNHL in R

03/07/02

50dB

04/04/02 05/08/02

07/10/02 07/22/02

04/16/03

62dB 48dB

75dB 48dB 80dB

LE: 32dB

0

10

20

30

40

50

60

70

80

90

3/6/98 4/6/98 5/6/98 6/6/98 7/6/98 8/6/98 9/6/98 10/6/98 11/6/98 12/6/98 1/6/99 2/6/99 3/6/99 4/6/99

Left ear

Left ear

Right CI implanted on 05/23/03

Left CI implanted on 09/26/05

Before Right CI, emphasis placed on total

communication with more sign language

incorporated

After Right CI, AV therapy techniques used to

focus on auditory training/listening with a

cochlear implant

Referred on NBHS

Bilateral SNHL confirmed at 2 weeks

Received amplification at 8 months

Intervention began at 10 months

Child was in NICU for an unspecified time

Part of CHIP program and is seen in home for 2 hr./wk.

Aspects of intervention: Speech

Amplification

Auditory training/listening

Natural gestures

Pidgin Sign English

Total communication approach

Spoken language and simultaneous sign

Intelligibility a big

Slight delay in expressive language

04/26/06

58dB 62dB

08/31/04

Right: 48dB

Left: 57dB

03/19/07

60dB

65dB

10/11/07

67dB

68dB

04/16/08

70dB

72dB

RE: 22dB

LE: 15dB

0

10

20

30

40

50

60

70

80

Left Ear

Right Ear

69

Morton and Nance NEJM 2006

Etiology of Pediatric Hearing Loss

Incidence at

Birth

(186 per

100,000)

Prevalence at 4

Years

(270 per 100,000)

GjB2 mutation,

21%

Syndromic,

14%

Nonsyndromic,

30%

Other genetic causes,

44%

Other environ-

mental causes,

14%

Pendred’s

syndrome

, 3%

CMV,

21%

Other

genetic

causes,

33%

Nonsyndromic,

22%

Syndromic,

11%

GjB2 mutation,

15%

Other environ-

mental causes,

14%

CMV,

25%

EVA

SLC26A4+

, 7% EVA

SLC26A4-,

5%

mtA1555

G, 1%

Total genetic

contribution

68% 54% Clinically

inapparent

infection,

11%

Clinically

apparent

infection, 10%

Clinically

apparent

infection, 7%

Clinically

inapparent

infection,

8%

Late-onset,

10%

Infant death 10% 0

Hearing loss 60% 7–15%

Mental retardation 45% 2–10%

Cerebral palsy 35% <1%

Chorioretinitis 15% 1–2%

4 Million - Annual Birth Rate

1 Percent - Average CMV Infection Rate

40,000 - Children Infected

4,000 -Symptomatic CMV (40.7% with HI)

36,000 -Asymptomatic CMV( 7.4 % with HI)

4,292 -Children born annually

with/develop HI from CMV

3/1,000 - Hearing loss in newborn

population

35.76 - % of hearing loss due to CMV

Adapted from Dahle et al,

2000

4 Million - Annual Birth Rate

1 Percent - Average CMV Infection Rate

40,000 - Children Infected

4,000 -Symptomatic CMV (40.7% with HI)

36,000 -Asymptomatic CMV( 7.4 % with HI)

4,292 -Children born annually

with/develop HI from

CMV

3/1,000 - Hearing loss in newborn

population

35.76 - % of hearing loss due to CMV

Adapted from Dahle et al,

2000

1960s - CID/symptomatic CMV infection and hearing loss first reported. Medearis, l964; McCracken, et al. 1969

1970s - inapparent / asymptomatic CMV infection and HL was first reported. Reynolds, et al. 1974; Dahle, et al. 1974; Hanshaw, et al. 1976; Stagno, et al. 1977

1970s & 1980s, progression and delayed onset HL first described. Dahle, et al, 1979; Williamson et al. 1982

3.9% at birth will have hearing loss; 8.3% at 6 yrs

Symptomatic infection – 16.5% HL at birth; 36.4% at 6 yrs

Asymptomatic infection – 2.9% HL at birth; 11.3% at 6 yrs

• 95% clinically inapparent

• 35% transmitted to fetus

• No clear relationship between

gestational age and transmission

• Fetal damage more likely in first 26

weeks, (32%) than later (15%)

United States Primary

N=176

Sero +

N=124

SN Hearing Loss 10% 11%

Bilateral HL 42% 23%

Late Onset HL 53% 31%

Progressive HL 63% 15%

Severe/Profound HL 63% 23%

UAB-Ross et al. J Pediatr 2006

Characteristics of CMV-Related HL

Total Number of Children 651 209

Fluctuating Loss 26 (54.1%) 25 (29.4%)

Improvement of Loss 23 (47.9%) 18 (21.2%)

Asymptomatic Symptomatic

Dahle, et. al., 2000

High-Frequency Only 18 (37.5%) 11 (12.9%)

(4000-8000 Hz)

•Fluctuating HL, High Frequency HL and Improvement of HL all occur

in CMV-Related HL –more often in asymptomatic infants

SUBJECTS ASYMPTOMATIC SYMPTOMATIC

SUBJECTS 651 209

Subjects with HL

48(7.4%) 85(40.7%)

Unilateral HL 25(52.1%) 28(32.9%)

Bilateral HL 23(47.9%) 57(67.1%)

High Frequency 18(37.5%) 11(12.9%)

Delayed Onset 18(37.5%) 23(27.1%)

Age Range 24-182 Months 6-197 Months

Progression 26(54.2%) 46(54.1%)

Age Range 3-186 Months 2-209 Months

Asymptomatic CMV – 53% unilateral

40% with progression of hearing loss

63% of loss in the Right ear

Symptomatic CMV - 35% unilateral

52% with progression of hearing loss

55% of loss in the Right ear

11% developed some degree of HL

in their good ear

Asymptomatic CMV – 53% unilateral

40% with progression of hearing loss

63% of loss in the Right ear

Symptomatic CMV - 35% unilateral

52% with progression of hearing loss

55% of loss in the Right ear

15% of congenital hearing loss

Associated with acquired

Progression

Fluctuation

Frequently only children with CI candidacy

have imaging

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