1. EFFICACY of OAE/ABR PROTOCOL in IDENTIFYING HEARING LOSS National Early Hearing Detection and Intervention Meeting Washington, D.C. February 20, 2004

2. Funded by the Centers for Disease Control and Prevention under a Cooperative Agreement with: The Association of Teachers of Preventive Medicine with a sub-agreement to: The University of Hawai`i

3. CORE STAFF Jean Johnson DrPH - Principal Investigator Karl White, PhD - Research Coordinator Judith E. Widen, PhD - Diagnostic Evaluation Coordinator

4. SITE CO-PRINCIPAL INVESTIGATORS Judith Gravel, PhD:Jacobi Medical Center (Bronx, New York) Michele James-Trychel, MEd:Arnold Palmer Hospital (Florida) Antonia B. Maxon, PhD:Lawrence & Memorial (Connecticut) Teresa Kennalley, MA:Via Christi Regional Medical Center (Kansas) 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)

5. CDC CONSULTANTS Krista Biernath, MD Technical Advisor Lee Ann Ramsey, BBA, GCPH Program Advisor

6. Background: Conclusion of the National Institutes of Health (NIH) Consensus Panel: (3) 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. NIH Consensus Statement March 3, 1993

7. RESEARCH QUESTION Does this two-stage procedure miss a significant number of babies with a congenital hearing loss?

8. CRITERIA for SELECTION of BIRTHING 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

9. PROJECTED SAMPLE SIZE 53,889 Annual Births 1,616 Eligible (Refer on OAE, Pass ABR) 1,500Consent to participate; and family speaks either English or Spanish 1,000 Babies return for complete diagnostic testing

10. BIRTHING CENTERS 2001 - 2003 BIRTH CENSUS Name of HospitalEnrollment PeriodNumber of Births Arnold Palmer 06/01/2001 – 12/31/2002 16,608 Good Samaritan 06/01/2001 – 01/31/2003 9,393 Jacobi Medical 09/20/2001 – 01/31/2003 4,747 Lawrence & Memorial 06/27/2001 – 03/31/2003 1,380 Long Island Jewish 05/01/2001 – 01/31/2003 10,424 Kapi`olani Medical 05/15/2001 – 01/31/2003 9,252 Via Christi 05/01/2001 – 01/31/2003 6,217 Women & Infants 05/01/2001 – 01/31/2003 16,623 Huntington 05/01/2001 – 01/31/2003 3,384 Northshore 05/01/2001 – 01/31/2003 10,224 Total Birth Census for Enrollment Period 88,252

11. PROCESS Eligible babies 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. Baby seen for audiological diagnostic evaluation between 7-9 months of adjusted age.

12. DATA BEING COLLECTED BirthdateBronchio-pulmonary Dsplasia 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

13. CURRENT ENROLLMENT STATUS 1,572 Infants Enrolled as of September 2003 Exceeded Target Enrollment

14. PERCENTAGE of CURRENT SAMPLE at EACH PARTICIPATING HOSPITAL

15. CUMULATIVE ENROLLMENT As of September 2003 Goal: 1,500 infants

16. CHILD ETHNICITY (Percentage of Current Sample)

17. HEALTH INSURANCE STATUS (percentage of current sample)

18. Annual Income in Babys Household

19. AUDIOLOGICAL DIAGNOSTIC EVALUATION Visual reinforcement audiometry Tympanometry EOAE – either TOAE or DPOAE

20. VRA PROTOCOL Well-defined, detailed protocol Well-defined, detailed protocol Responses at 500, 1K, 2K, 4K Hz Responses at 500, 1K, 2K, 4K Hz Response levels of 15 dB HL Response levels of 15 dB HL

21. 885 Diagnostic Evaluations Completed DIAGNOSTIC EVALUATIONS

22. Behavioral Evaluations as of January 29, 2004

23. DIAGNOSTIC EVALUATIONS (percentage completed at each participating hospital)

24. EXPANDED STUDY Comparison Group: Refer/Refer Babies who failed both OAE and ABR and were referred for a diagnostic evaluation Same enrollment data Results of audiological diagnostic evaluations

25. VALUE of EXPANDED STUDY Enables study to determine what proportion that babies with a hearing loss from the refer/pass group represent of all babies identified with hearing loss in the sample cohort. Enables study to determine what proportion that babies with a hearing loss from the refer/pass group represent of all babies identified with hearing loss in the sample cohort. Additional data provides an accurate estimate of the proportion of all babies with congenital hearing loss who are being missed by the two- stage OAE/ABR protocol. Additional data provides an accurate estimate of the proportion of all babies with congenital hearing loss who are being missed by the two- stage OAE/ABR protocol.

26. ATPM EXPANDED STUDY: Cumulative Enrollment As of January 2004

27. Number of Inactivated Infants by Month

28. REVISED TIME LINE Enrolled babies through January 2003. Enrolled babies through January 2003. Complete evaluations by January 2004. Complete evaluations by January 2004. Investigators analyze data in March 2004. Investigators analyze data in March 2004. Present results in May 2004. Present results in May 2004.

29. Are we missing babies with existing screening equipment? If so how many and of what type?

30. What is the significance of variable pass-refer rates associated with different screening devices (AOAE and AABR)? What is the significance of variable pass-refer rates associated with different screening devices (AOAE and AABR)?

31. What Kind of Babies will be found Congenital hearing loss Late-onset loss Study Design Improvements Diagnostic ABR for all babies who failed OAE regardless of AABR result Larger sample size

32. Would the follow-up of high- risk babies improve the sensitivity of the screening tests in detecting mild forms of hearing loss?

33. What is the cost effectiveness of different screening protocols?

34. Does ASSR have a role in the screening and assessment of infants?

35. Auditory Neuropathy How many of the babies in our sample who passed AOAE would have been identified as at risk for AN if we had tested all with AABR? Should we use different screening protocols in the NICU than WBN?