1. Identification of Late-Onset Hearing Loss
As a supplement to Universal Newborn Hearing Screening Programs.

2. PRESENTERS Yusnita Weirather – Kapiolani Medical Center, Hawaii
Karl White – Utah State University.

3. Child A: DOB: 05-22-1998 Age of identification: 5 years and 4 months
Failed behavioral hearing screening at pediatrician’s office during routine physical
Passed newborn hearing screening with OAE
Hospitalized for 3 days within the 1st year of life due to fever with unknown diagnosis.
No reported history of otitis media.
Right ear: normal hearing thresholds
Left ear: moderate sloping to mild sensorineural hearing loss

4. Audiological findings

5. Late onset vs Progressive Hearing Loss
PROGRESSIVE HL
Onset time: at birth or after birth
LATE ONSET HL
Onset time: after birth
Indicators of progressive hearing loss :
Delayed or abnormality in motoric development
unilateral and asymmetric hearing loss
Children with dizziness or vestibular problem
Changes in auditory behaviors

6. Late onset hearing loss
Defining feature: Normal hearing at birth
Onset time: several days or months after birth or later in life
Screening for late onset loss particularly important before age 5

7. Lack of clarity of infant’s hearing at birth
Approximately 5-20 % of infants screened do not complete the screening process every year.
Some NICU children screen weeks after birth.
Some parents or physicians do not have access to the results if the infant passes screening.
An electronic statewide newborn hearing screening database is not available in all states.

8. Etiology of Hearing Loss

9. Child B Age of identification: 1 year chronological age
Failed hearing screening at one year chronological age
Delayed screening was because of her medical condition and life support equipment
Mother reported that her child gradually became unresponsive to her voice.
24 weeks premature, 923 grams

10. Audiological and otological findings
Normal CT scan findings of temporal bone structure
Normal middle ear functions
Absent OAEs and ABRs to click and tone stimuli.
Vibrotactile bone conduction responses to 250 and 500 Hz at 60 dBHL

11. Pre-lingual vs post-lingual hearing loss
Easier detection by parents or caregivers if post-lingual
Less effect in post-lingual children
Easier to fit hearing aids for post-lingual children.

12. Late Onset Hearing Loss
What do we know about late-onset hearing loss (LOHL) ?
How are states implementing surveillance?
What are obstacles / challenges in implementing surveillance?
Considerations for implementing surveillance

13. “JCIH 2000” indicators for late onset or progressive HL
Family history
Parental concern
In utero and postnatal infections
Physical findings: syndromal stigmata, craniofacial anomalies
Neurodegenerative disorders
Temporal bone trauma
Neonatal indicators (continued)

14. “JCIH 2000” indicators for late onset or progressive HL continued ………….
Neonatal indicators—hyperbilirubinemia at a serum level requiring exchange transfusion, persistent pulmonary hypertension associated with mechanical ventilation, and conditions requiring the use of extracorporeal membrane oxygenation (ECMO).

15. % of children with hearing loss who have the following risk factors
Cone-Wesson et al. (2000) taken from Fortnum & Davis, 1997 and the NIH multicenter study (Norton et al., 2000)

16. Reported Etiology of HL by onset, for the estimated population of Deaf and HH students in The US, (N= 48,300)
CMV
Rubella
Hereditary
Birth complica-tion
Other causes
Un-
known
At birth
1.3
2.1 13
8.7
4.5
17.8 OM
Trauma
Infection
Meningitis
After birth
3.7
0.6 4
8.1
1.5
5.3
Onset not known: 29.4%
Annual survey of hearing impaired children and youth, Center for Assessment and Demographic Studies, Gallaudet University.

17. Gallaudet Research Institute Reported Etiology of HL, Jan 2003 (N= 42,361)
Genetic / Hereditary / Familial %
Cause can not be determined / DNA %
Pregnancy related
Maternal rubella %
CMV %
Prematurity %
Trauma at birth / complications %
Other causes %
Post Birth Disease / injury
OM %
Meningitis %
Other infections %
Medications %
Trauma %
Other %
Regional and National Summary Report of Data from Annual survey of Deaf and Hard of Hearing Children and Youth. Washington, DC: GRI, Gallaudet University

18. Examples how states are implementing surveillance for LOHL
Provide parents with a list of risk indicators
Send NHS results to the child’s physician
“Tickler file” to call back children with risk indicators
Hearing screening for children in Head Start, Part C, and Preschool programs.
Hearing screenings by physicians as part of children’s annual physicals.

19. Obstacles / challenges in implementing surveillance for LOHL
NHS is still voluntary in some states / hospitals.
Some risk indicators are difficult to assess (hypoxia).
Some etiologies are unknown or difficult to access.
Lack of familiarity with auditory development and auditory behaviors
Comprehensive electronic population data management is unavailable.
Inconsistency exists in the schedule and protocol for audiological follow up across the country.

20. Child C: DOB:
Age of identification: 7 months at first follow up requested by hospital hearing screening program due to PPHN
Passed newborn hearing screening with AABR
Medical history: 37 ½ weeks gestational age, meconium aspiration, maternal history of substance abuse, amp/gent/lasix, PPHN, hydronephrosis, mild chronic lung disease.
Audiological findings: Bilateral moderate sensorineural hearing loss

21. Child C

22. Child D: DOB: 08-19-2000 Age of identification: 3 years of age.
Passed newborn hearing screening with AABR.
Medical history: Meconium aspiration and PPHN requiring treatment with ECMO

23. Audiological findings
Normal middle ear functions
Absent DPOAE in both ears
No response to click and tone air conduction ABR at 90 dBnHL and no response to bone conduction ABR at 65 dBnHL.

24. Possible referral sources for detecting LOHL
NHS program
Parents
Physician
School
Other health professional

25. Considerations for implementing surveillance
Parental education through NHS
Educate medical home providers about risk indicators
Education in auditory development and auditory behaviors to health care providers, day care providers, and preschool teachers
Complete recording of medical history
Appropriate protocol for audiological follow up based on the disorder
Create a LOHL Registry

26. Parental education during NHS
More than just leaving a brochure in the infant’s crib
Include newborn hearing screening results in the immunization record
Developmental examples on the importance of good hearing
The effect of hearing loss can be minimized or managed

27. Include in the overall developmental milestones
Education for health care providers, day care providers, and preschool teachers
Include in the overall developmental milestones
Include in the activities to stimulate developmental skill
Perform hearing screening as part of physical check up

28. Complete recording of medical history
Pre and postnatal history
Primary and secondary diagnosis
Medical management
Screening results
Family history

29. Obstacles to follow up after NHS
Medical chart review - Finding risk indicators
Contacting the family
Performing audiological evaluations for children under 3 years of age
Financial consequences
Family compliance

30. JCIH Recommendation for Detecting LOHL
Any infant with [the following] risk indicators for progressive or delayed-onset hearing loss who has passed the birth screen should, receive audiologic monitoring every 6 months until 3 years.
(a) Parental or caregiver concern.
(b) Family history of permanent childhood hearing loss
(c) Stigmata associated with a syndrome known to include a SNHL or conductive hearing loss
(d) Postnatal infections (e.g., bacterial meningitis)
(e) In-utero infections (e.g., CMV, herpes, rubella, syphilis, and toxoplasmosis.
(f) Neonatal indicators -- especially hyperbilirubinemia, PPHN, ECMO
(g) Syndromes associated with progressive hearing loss
(h) Neurodegenerative disorders sensory motor neuropathies (e.g., Hunter syndrome, Friedreich’s ataxia, Charcot-Marie-Tooth syndrome)
(i) Head trauma
(j) Recurrent or persistent otitis media with effusion for at least 3 months
(See Section E of the JCIH Year 2000 Statement

31. Logistical Considerations for Implementing JCIH Recommendation
Assume 8% of all children passing newborn hearing screening have a risk indicator
4 million births per year x .08 = 320,000 babies with risk indicators born each year
Assessing each baby with a risk indicator every 6 months until 3 years of age would require:
320,000 babies x 6 assessments;
or 1,920,000 assessments each year
Currently, about 1% of all births (40,000) are referred from NBHS programs for audiological assessment each year
The most serious concern of State EDHI coordinators is the lack of experienced pediatric audiologists
Audiologic monitoring may not require a full diagnostic assessment

32. Expected “Yield” of Regular Monitoring of 0-3 Year- old Children for LOHL
Not enough data for a definitive estimate
University of Washington Study
4,911 children (mostly NICU) were screened at birth and asked to return for VRA at 8-12 months of age regardless of newborn hearing screening result (~65% had useable VRA results)
56 children with permanent hearing loss were identified
Investigators concluded only 1 of these children had late onset loss
Anecdotal evidence
States with long-established EHDI programs (CO and RI) report about 5% of children with hearing loss are late onset

33. Late onset or Progressive Hearing Loss?
Although the terms are often used interchangeably, they should not be
Congenital Late-onset
Progressive
Stable
From University of Washington study
1 of 56 children (1.79%) was late onset
7 of 56 children (12.50%) were progressive

34. Hawaii Data
1998
1999
2000
2001
# of birth
11,997
15,009
14,989
14,979
# need f/u
154
326
159
124
# received f/u 59 71 51 44
# confirmed 7 5 2 4
# confirmed loss by NHS 62 65 64

35. Risk indicators among LOHL children in Hawaii
2 with Family History of hearing loss
2 with Parental concern
2 with in utero and postnatal infections
1 with syndromal stigmata
9 with Neonatal indicators ( ECMO=1, PPHN=3, hyperbilirubinemia=3, mechanical ventilation=2)
1 with Ototoxic medication: 1

36. Hawaii Data (continued)

37. Take-Home Messages
Late-onset and progressive hearing loss should not be used interchangeably
More data is needed about the incidence of both late-onset and progressive hearing loss
Following all babies with risk-indicators who pass the newborn hearing screening test would be very expensive and logistically difficult
Evaluations of alternative approaches needed (e.g., screening in the medical home and/or early childhood programs, parent education, ????)