Christine Yoshinaga-Itano, Ph.D. Professor University of Colorado, Boulder Department of Speech, Language & Hearing Sciences Allison Sedey, Ph.D.
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1 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
2 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
3 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
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5 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.
6 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
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8 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
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10 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).
11 Number Percent
12 Genetic: N=6 20% Cytomegalic Virus: N=4 12% Unknown N= % Ototoxicity N=2 (6.6%) Multiple etiologies: N=1 3.3% Mondini N=1 3.3% Treacher Collins N=1 3.3%
13 **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
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15 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.
16 Percent Number mo mo mo mo.
17 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
18 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
19 100% 90% 80% 70% 60% 50% 40% 30% Present at birth Progressive Acquired Total P & A 20% 10% 0% mild moderate severe profound
20 Percent Acquired+ Progressive HL by degree of HL (4 to 7 years) Acquired Progressive Total Mild Moderate Severe Profound
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22 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 Englishspeaking homes.
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24 10% of all ages had a progressive hearing loss Ages 1 to 42 months: 6.4% had a progressive HL Ages months: 18.5% had a progressive HL 768 of 853 children had stable HL 85 had progressive HLs
25 mo mo mild mod mod-sev sev profound
26 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
27 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.
28 all ages 1 to 42 mo mo 5 0
29 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
30 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
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45 Failed NBHS in both ears Mild SNHL confirmed at 3 weeks by ABR Since it was a mild hearing loss, no followup 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
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47 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 = 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
48 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
49 01/30/03 (age: 4;11) Expressive One Word Vocabulary-10 th percentile 258 utterances, 53 sign & 248 spoken Good intelligibility Mother has learned 389 out of 400 signs
50 08/31/04 Right: 48dB Left: 57dB 58dB 62dB 04/26/06 65dB 60dB 03/19/07
51 RE: 22dB LE: 15dB 68dB 72dB 67dB 70dB 10/11/07 04/16/08
52 Left Ear Right Ear
53 02/01/05 (age: 7;1) In DHH classroom 15 hr./wk. & regular classroom with interpreter 13 hr./wk. Expressive One Word Vocabulary Test-5 th percentile Test of Auditory Comprehension of language total score-39 th percentile Excellent intelligibility Mother has learned 398 out of 400 signs
54 02/04/98 (age 1.5 months) Approximates a mild hearing loss bilaterally 01/31/00 (age: 2 years) Approximates a flat, moderate hearing loss bilaterally
55 RE: 13dB LE: 11dB 57 db 63dB 57dB 65dB 02/07/02 04/09/02 55 db 63dB 68 db 72 db 08/06/02 02/15/05
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57 Left ear Right ear
58 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
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60 Click ABR (1.5 months) + 500Hz tone burst (5 months) Severe rising to moderate SNHL in L Profound SNHL in R
61 LE: 32dB 50dB 62dB 48dB 03/07/02 04/04/02 05/08/02 75dB 48dB 80dB 07/10/02 07/22/02 04/16/03
62 90 Left ear Left ear /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
63 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
64 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
65 Total communication approach Spoken language and simultaneous sign Intelligibility a big Slight delay in expressive language
66 08/31/04 Right: 48dB Left: 57dB 58dB 62dB 04/26/06 65dB 60dB 03/19/07
67 RE: 22dB LE: 15dB 68dB 72dB 67dB 70dB 10/11/07 04/16/08
68 Left Ear Right Ear
69 Etiology of Pediatric Hearing Loss Clinically apparent infection, 10% Other environmental causes, 14% Incidence at Birth (186 per 100,000) Clinically inapparent infection, 11% CMV, 21% Pendred s syndrome, 3% Other genetic causes, 44% Nonsyndromic, 30% GjB2 mutation, 21% Syndromic, 14% Total genetic contribution 68% 54% mta1555 G, 1% Syndromic, 11% Nonsyndromic, 22% Prevalence at 4 Years (270 per 100,000) GjB2 mutation, 15% Other genetic causes, 33% EVA SLC26A4+, 7% CMV, 25% EVA SLC26A4-, 5% Other environmental causes, 14% Late-onset, 10% Clinically inapparent infection, 8% Clinically apparent infection, 7% Morton and Nance NEJM
70 Infant death 10% 0 Hearing loss 60% 7 15% Mental retardation 45% 2 10% Cerebral palsy 35% <1% Chorioretinitis 15% 1 2%
71 4 Million 1 Percent 40,000 4,000 36,000 4,292 3/1, Annual Birth Rate - Average CMV Infection Rate - Children Infected -Symptomatic CMV (40.7% with HI) -Asymptomatic CMV( 7.4 % with HI) -Children born annually with/develop HI from CMV - Hearing loss in newborn population - % of hearing loss due to CMV Adapted from Dahle et al,
72 4 Million 1 Percent 40,000 4,000 36,000 4,292 with/develop CMV 3/1,000 population Annual Birth Rate - Average CMV Infection Rate - Children Infected -Symptomatic CMV (40.7% with HI) -Asymptomatic CMV( 7.4 % with HI) -Children born annually HI from - Hearing loss in newborn - % of hearing loss due to CMV Adapted from Dahle et al,
73 1960s - CID/symptomatic CMV infection and hearing loss first reported. Medearis, l964; McCracken, et al s - inapparent / asymptomatic CMV infection and HL was first reported. Reynolds, et al. 1974; Dahle, et al. 1974; Hanshaw, et al. 1976; Stagno, et al s & 1980s, progression and delayed onset HL first described. Dahle, et al, 1979; Williamson et al % 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
74 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%)
75 United States Primary Sero + N=176 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
76 Characteristics of CMV-Related HL Fluctuating HL, High Frequency HL and Improvement of HL all occur in CMV-Related HL more often in asymptomatic infants Asymptomatic Symptomatic Total Number of Children Fluctuating Loss 26 (54.1%) 25 (29.4%) Improvement of Loss 23 (47.9%) 18 (21.2%) High-Frequency Only 18 (37.5%) 11 (12.9%) ( Hz) Dahle, et. al., 2000
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78 SUBJECTS ASYMPTOMATIC SYMPTOMATIC SUBJECTS 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 Months Months Progression 26(54.2%) 46(54.1%) Age Range Months Months
79 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
80 11% developed some degree of HL in their good ear
81 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
82 15% of congenital hearing loss Associated with acquired Progression Fluctuation Frequently only children with CI candidacy have imaging
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