1. THE EFFICACY OF AN OAE/AABR PROTOCOL FOR IDENTIFYING HEARING LOSS IN NEWBORNS: Are Infants with Hearing Loss Not Being Identified? presented at NHS 2004 The International Conference on Newborn Hearing Screening, Diagnosis and Intervention Cernobbio, Italy May 29, 2004

2. Research Team Principal Investigator: Jean Johnson, DrPH Research Coordinator: Karl R. White, PhD Diagnostic Evaluation Coordinator: Judith E. Widen, PhD Site Co-Principal Investigators: Judith Gravel, PhD:Jacobi Medical Center (Bronx, New York) Michele James-Trychel, MEd:Arnold Palmer Hospital (Florida) Teresa Kennalley, MA:Via Christi Regional Medical Center (Kansas) Antonia B. Maxon, PhD:Lawrence & Memorial (Connecticut) Lynn Spivak, PhD:Long Island Jewish Health System (New York) Maureen Sullivan-Mahoney, MA:Good Samaritan Hospital (Ohio) Betty Vohr, MD:Women & Infants Hospital (Rhode Island) Yusnita Weirather, MA:Kapi`olani Medical Center (Hawai`i)

3. Funded by the Centers for Disease Control and Prevention CDC Consultants: June Holstrum, PhD Brandt Culpepper, PhD Krista Biernath, MD Lee Ann Ramsey, BBA, GCPH under a Cooperative Agreement with: The Association of Teachers of Preventive Medicine with a sub-agreement to: The University of Hawai`i

4. Background National Institutes of Health (NIH) Consensus Panel recommended in March 1993 that: the preferred model for screening should begin with an evoked otoacoustic emissions test and should be followed by an auditory brainstem response test for all infants who fail the evoked otoacoustic emissions test. Continuing improvement of ABR technology led to a number of hospitals in the US implementing a variation of the NIH recommendation that was based on automated ABR (AABR) Anecdotal reports to the Centers for Disease Control and Prevention (CDC) in the mid to late 1990s that the two-stage OAE/AABR protocol was not identifying infants with mild hearing loss. The CDC issued a competitive Request for Proposals in late 2000 to investigate whether the OAE/AABR screening protocol was not identifying babies with hearing loss

5. AABR Screening Comprehensive Hearing Evaluation Before 6 Months of Age Fail Pass Discharge OAE Screening Prior to Hospital Discharge RESEARCH QUESTION Are infants with permanent hearing loss not being identified when newborn hearing screening is done with a two-stage OAE/AABR protocol in which infants who fail OAE and pass AABR are not followed? Study Sample Comprehensive Audiological Assessment at 8-12 months of age Comparison Group

6. CRITERIA for SELECTING SITES 2,000 or more births per year Established newborn hearing screening program with at least six month history of success Historical refer rates of less than 10% for OAE and 4% for ABR Success in obtaining follow-up on 90% or more of referrals Ethnic and socio-economic distribution similar to US population

7. Participating Sites Name of Hospital Location Arnold Palmer HospitalTampa, Florida Good Samaritan HospitalColumbus, Ohio Jacobi Medical Center and North Central Bronx HospitalNew York, New York Kapi`olani Medical CenterHonolulu, Hawaii Long Island Jewish Medical SystemNew York, New York (included North Shore University, Hunter and Long Island Jewish Hospitals) Via Christi Regional Medical CenterKansas City, Kansas Women & Infants HospitalProvidence, Rhode Island

8. Data Collection Process Eligible infants (Failed OAE and Passed AABR) identified following newborn hearing screening. Parents contacted and research study explained. Consent obtained from families. Enrollment data collected. Contact maintained with family at 2, 4, & 6 months of age via post cards. Infants seen for audiological diagnostic evaluation at 8-12 months of adjusted age.

9. Data Collected for Each Participating Infant BirthdateBronchio-pulmonary Dysplasia GenderMechanical Ventilation >7 Days Birth WeightECMO Gestational AgeNumber of Children in Home APGAR ScoresNumber of Adults in Home Days in NICU Total Household Income Malformations of the Head and NeckChilds Race/Ethnicity Syndrome Associated with Hearing LossHealth Insurance In-utero InfectionsFamily History of Hearing Loss

10. Study Sample 1,524 Infants Enrolled 973 (63.8%) Returned for Evaluation 1,432 Ears Evaluated

11. Enrollment of Study Participants

12. Number of Infants: Births During Enrollment Eligible for Enrollment Total EnrolledNot Recruited# of Refusals Site # 1 16,6081,044191418435 6.3%18.3%40.0%41.7% Site # 2 9,3934213701833 4.5%87.9%4.3%7.8% Site # 3 4,5092858418615 6.3%29.5%65.3%5.3% Site # 4 9,2522091473032 2.3%70.3%14.4%15.3% Site # 5 24,03245617011275 1.9%37.3%2.4%60.3% Site # 6 16,62361429671247 3.7%48.2%11.6%40.2% Site # 7 6,21743326650117 7.0%61.4%11.5%27.0% Total 86,6343,4621,5247841,154 4.0%44.0%22.6%33.3% Enrollment of Study Participants (continued)

13. AUDIOLOGICAL DIAGNOSTIC EVALUATION » Visual reinforcement audiometry » Tympanometry » Either TEOAE or DPOAE

14. VRA PROTOCOL Protocol based on University of Washington (2000) study Responses at 500, 1K, 2K, 4K Hz –Order of testing 2K,.5K, 4K, 1K –Aiming for minimal response level of 15 dB HL Multiple visits often necessary to complete testing –68% completed in 1 visit –24 % required 2 visits –8% required 3 or more visits

15. CategoryDescription Not Permanent Hearing Loss Using the best results from all assessments, MRL thresholds of < 20dB at 1K, 2K, and 4K. Probable Not Permanent Hearing Loss MRL data not available at 1K, 2K, and 4K, BUT *All frequencies had MRLs < 20dB OR OAEs within normal limits* OR Tone burst ABR data < 25dB. Permanent Hearing Loss (PHL) Sensorineural MRLs > 25dB at 1K, 2K, or 4K (tested with good confidence) OR ABR threshold > 30dB; AND if tested, OAEs below normal limits at the frequencies with elevated MRLs; AND normal middle ear functioning based on tympanometry or bone conduction. Permanent Conductive MRLs > 25dB at 1K, 2K, or 4K (tested with good confidence); AND if tested, OAEs below normal limits; AND bone conduction thresholds 15dB at frequencies with MRLs > 25dB. Increased Suspicion of PHL High Suspicion MRLs > 25dB at 1K, 2K, or 4K, BUT OAEs within normal limits for those frequencies OR only fair confidence in VRA testing. Some Suspicion * MRLs > 30dB at 1 frequency or > 25dB at more than one frequency, BUT abnormal tympanometry AND no bone conduction. * Sound field thresholds > 25dB (with fair confidence) AND normal tympanometry AND OAEs below normal limits. Not Sufficient Data to Rule Out PHL * No MRLs or OAEs within normal limits for 1K, 2K, or 4K and none of the above criteria for permanent hearing loss are met. * OAEs within normal limits were defined as > 3-6dB at 1K and > 6dB at 2K and 4K. Criteria for Categorizing Hearing Loss

16. Examples of How Hearing Status was Categorized

18. Is It Important that 21 Infants (30 ears) with Permanent Hearing Loss Were Found? How many does it add to what would have been identified otherwise? How many ears with hearing loss were found among those that passed the initial screen? How many infants would you have to follow to find 21 infants with PHL? Is this congenital or late-onset hearing loss?

19. PHL in Comparison Group Sites (Fail OAE/Fail AABR)

21. How Many Additional Infants with Permanent Hearing Loss were Identified? Comparison Group (Fail OAE/ Fail AABR) Study Group (Fail OAE/ Pass AABR) Total Number of Infants 15821179 Prevalence per 1,000 1.820.242.06 Represents 11.7% of all infants with PHL in birth cohort

22. Degree of Hearing Loss* in Study and Comparison Group Infants 79.9% 28.6%

23. PHL in Ears of Study Infants that Passed Initial OAE

24. How Many Infants Must Be Screened to Find 21 with PHL? The obvious answer is 973, but…. –This ignores that most screening programs that use OAE also do second stage OAE screen (usually following hospital discharge) –Such outpatient screening is less expensive than the diagnostic protocol used in this study –Difficulty of getting infants to return for outpatient screening must be considered

25. Were any of these ears late-onset losses? This study was not designed to answer that question. We do know that IF all of the ears with risk factors had been followed and identified, 10 of 21 babies would still not have been identified Little is know about the incidence or what predicts Late-onset hearing loss Most of the hearing losses not identified were mild which is what we would expect if ears are being missed

26. Whats the Best Estimate of the Number of Infants Not Identified by the OAE/AABR Screening Protocol? Depends on the criteria used for determining PHL Variation among sites Adjustments for Differences Between Study and Comparison Groups

28. Different Criteria for Determining Permanent Hearing Loss Comparison Group Study Group Total Based only on those meeting criteria for PHL 1.82.24 (21 babies) 2.06 Including those categorized as high suspicion of PHL 1.82.43 (37 babies) 2.25 XX X X

29. Variation Among Sites The study design assumed that sites are all equally well implemented To the degree that this isnt true, data from some sites may be a better estimate of the number of infants not being identified

30. Indicators of Implementation Quality FirstBest Second Third FourthGood

32. Comparability of Study and Comparison Groups What Percent of Referred Infants Did Sites Try to Follow? What Percent of Followed Infants Were Diagnosed? Study Group (Fail OAE/ Pass AABR 44%63.8% Comparison Group (Fail OAE/ Fail AABR) 100%85.8% Reasonable to adjust prevalence rates for those who were not recruited Adjusting prevalence rates for differences in the percent of diagnostics completed is problematic Families who think their infant has a hearing loss are more likely to return Families that are poor, single heads of household, transient, etc. are less likely to return and these variables may be correlated with the incidence of hearing loss

33. Prevalence of PHL per 1,000 Best Estimate of Amount of PHL not Identified by OAE/AABR protocol Infants Who Failed OAE / Failed AABR Adding Infants who failed OAE / Passed AABR Based on 44% that participated Adjusted for those who did not participate All Sites 1.822.06 (0.24 increase) 2.37 (0.55 increase) Sites with Best Implementation 2.272.73 (0.46 Increase) 2.99 (0.72 increase) 11.7% of infants with PHL in birth cohort 24% of infants with PHL in birth cohort 23% of infants with PHL in birth cohort 16.8% of infants with PHL in birth cohort

34. Conclusions The OAE/AABR protocol implemented in this study does not identify a substantial number of infants with permanent hearing loss Best estimate is.55 per thousand or 23% of all infants with permanent hearing loss Mostly mild sensorineural hearing loss Impossible to determine whether this is congenital or late-onset About 45% would be identified if all infants with risk factors or contralateral refer ears were followed, but this may not be practical

35. Screening for permanent hearing loss should extend into early childhood (e.g. physicians offices, early childhood programs) Emphasize to families and physicians that passing hospital-based hearing screening does not eliminate the need to vigilantly monitor language development. Screening program administrators should ensure that the stimulus levels of equipment used are consistent with the degree of hearing loss they want to identify The relative advantages and disadvantages of the two- stage (OAE/AABR) protocol need to be carefully considered for individual circumstances Recommendations

36. Prevalence and methods of identifying late-onset hearing loss Ongoing investigation of sensitivity of various screening protocols and equipment (including what level of hearing loss is targeted) Practicality and cost-efficiency of alternative continuous screening and surveillance techniques Recommendations for Further Research