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Identification of Late-Onset Hearing Loss

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

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Identification of Late-Onset Hearing Loss

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  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 HL Onset time: after birth PROGRESSIVE HL Onset time: at birth or after birth Late onset vs Progressive Hearing Loss 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 HLcontinued …………. 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, 1992-1993 (N= 48,300) 1992-1993 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 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

  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: 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

  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. 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

  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, ????)

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