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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
