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

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

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

Audiological findings

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

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

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.

Etiology of Hearing Loss

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

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

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.

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

“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)

“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).

% 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)

Reported Etiology of HL by onset, for the estimated population of Deaf and HH students in The US, 1992-1993 (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% 1992-1993 Annual survey of hearing impaired children and youth, Center for Assessment and Demographic Studies, Gallaudet University.

Gallaudet Research Institute Reported Etiology of HL, Jan 2003 (N= 42,361) Genetic / Hereditary / Familial 20.8 % Cause can not be determined / DNA 52.4 % Pregnancy related Maternal rubella 0.8 % CMV 1.8 % Prematurity 4.2 % Trauma at birth / complications 5.3 % Other causes 1.3 % Post Birth Disease / injury OM 5.6 % Meningitis 4.7 % Other infections 1.7 % Medications 1.3 % Trauma 0.9 % Other 3.3 % Regional and National Summary Report of Data from 2001-2002 Annual survey of Deaf and Hard of Hearing Children and Youth. Washington, DC: GRI, Gallaudet University

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.

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.

Child C: DOB: 03-09-1998 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

Child C

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

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.

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

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

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

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

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

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

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

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

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

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

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

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

Hawaii Data (continued)

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, ????)