Presentation on theme: "Identification of Late-Onset Hearing Loss"— Presentation transcript:
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 physicalPassed newborn hearing screening with OAEHospitalized 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 thresholdsLeft ear: moderate sloping to mild sensorineural hearing loss
5 Late onset vs Progressive Hearing Loss PROGRESSIVE HLOnset time: at birth or after birthLATE ONSET HLOnset time: after birthIndicators of progressive hearing loss :Delayed or abnormality in motoric developmentunilateral and asymmetric hearing lossChildren with dizziness or vestibular problemChanges in auditory behaviors
6 Late onset hearing loss Defining feature: Normal hearing at birthOnset time: several days or months after birth or later in lifeScreening 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.
9 Child B Age of identification: 1 year chronological age Failed hearing screening at one year chronological ageDelayed screening was because of her medical condition and life support equipmentMother 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 structureNormal middle ear functionsAbsent 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-lingualLess effect in post-lingual childrenEasier 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 historyParental concernIn utero and postnatal infectionsPhysical findings: syndromal stigmata, craniofacial anomaliesNeurodegenerative disordersTemporal bone traumaNeonatal 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)CMVRubellaHereditaryBirth complica-tionOther causesUn-knownAt birth1.32.1138.74.517.8OMTraumaInfectionMeningitisAfter birth3.70.6188.8.131.52Onset 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 relatedMaternal rubella %CMV %Prematurity %Trauma at birth / complications %Other causes %Post Birth Disease / injuryOM %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 indicatorsSend NHS results to the child’s physician“Tickler file” to call back children with risk indicatorsHearing 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 behaviorsComprehensive 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 PPHNPassed newborn hearing screening with AABRMedical 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
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 functionsAbsent DPOAE in both earsNo 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 programParentsPhysicianSchoolOther health professional
25 Considerations for implementing surveillance Parental education through NHSEducate medical home providers about risk indicatorsEducation in auditory development and auditory behaviors to health care providers, day care providers, and preschool teachersComplete recording of medical historyAppropriate protocol for audiological follow up based on the disorderCreate a LOHL Registry
26 Parental education during NHS More than just leaving a brochure in the infant’s cribInclude newborn hearing screening results in the immunization recordDevelopmental examples on the importance of good hearingThe 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 teachersInclude in the overall developmental milestonesInclude in the activities to stimulate developmental skillPerform hearing screening as part of physical check up
28 Complete recording of medical history Pre and postnatal historyPrimary and secondary diagnosisMedical managementScreening resultsFamily history
29 Obstacles to follow up after NHS Medical chart review - Finding risk indicatorsContacting the familyPerforming audiological evaluations for children under 3 years of ageFinancial consequencesFamily 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 indicator4 million births per year x .08 = 320,000 babies with risk indicators born each yearAssessing 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 yearCurrently, about 1% of all births (40,000) are referred from NBHS programs for audiological assessment each yearThe most serious concern of State EDHI coordinators is the lack of experienced pediatric audiologistsAudiologic 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 estimateUniversity of Washington Study4,911 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 identifiedInvestigators concluded only 1 of these children had late onset lossAnecdotal evidenceStates 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 beCongenital Late-onsetProgressiveStableFrom University of Washington study1 of 56 children (1.79%) was late onset7 of 56 children (12.50%) were progressive
34 Hawaii Data1998199920002001# of birth11,99715,00914,98914,979# need f/u154326159124# received f/u59715144# confirmed7524# confirmed loss by NHS626564
35 Risk indicators among LOHL children in Hawaii 2 with Family History of hearing loss2 with Parental concern2 with in utero and postnatal infections1 with syndromal stigmata9 with Neonatal indicators ( ECMO=1, PPHN=3, hyperbilirubinemia=3, mechanical ventilation=2)1 with Ototoxic medication: 1
37 Take-Home MessagesLate-onset and progressive hearing loss should not be used interchangeablyMore data is needed about the incidence of both late-onset and progressive hearing lossFollowing all babies with risk-indicators who pass the newborn hearing screening test would be very expensive and logistically difficultEvaluations of alternative approaches needed (e.g., screening in the medical home and/or early childhood programs, parent education, ????)