Efficacy of the OAE/ABR Protocol in Identifying Permanent Hearing Loss in Newborns

1. Efficacy of the OAE/ABR Protocol in Identifying Permanent Hearing Loss in Newborns 12th Annual Maternal-Child Health Epidemiology Conference Jean L. Johnson, DrPH Center for Disability Studies University of Hawai`i Atlanta, Georgia December 8, 2006

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 Michele James, AuD Teresa Kennalley, MA Antonia B. Maxon, PhD Lynn Spivak, PhD Maureen Sullivan-Mahoney, MA Betty Vohr, MD Yusnita Weirather, MA

3. Funded by the Centers for Disease Control and Prevention CDC Consultants: June Holstrum, PhD Roy Ing, MD, MPH 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. Why is early identification of hearing loss so important? • Hearing loss occurs more frequently than any other birth defect. • Undetected hearing loss has serious negative consequences. • Dramatic benefits are associated with early identification of hearing loss. It is true for severe to profound loss. Is it also true for milder hearing loss?

5. 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 1990’s that the two-stage OAE/AABR protocol was not identifying some infants with permanent hearing loss. • The CDC issued a competitive Request for Proposals in 2000 to investigate whether the OAE/AABR screening protocol was not identifying babies with hearing loss.

6. Oto-Acoustic Emissions (OAE) Screening

7. Auditory Brain Stem (ABR)Screening

8. Research Question Are infants with permanent hearing loss not being identified when newborn hearing screening is done with a two-stage OAE/A-ABR protocol in which infants who fail OAE and pass A-ABR are not followed? OAE Screening Prior to Hospital Discharge AABR Screening Comprehensive Hearing Evaluation Before 3 Months of Age Fail Fail Pass Pass Discharge Discharge Study Sample Comprehensive Audiological Assessment at 8-12 months of age Comparison Group

9. 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 85% or more of referrals • Ethnic and socio-economic distribution similar to US population

10. Participating Sites Name of Hospital Location Arnold Palmer Hospital Orlando, Florida Good Samaritan Hospital Columbus, Ohio Jacobi Medical Center New York, New York Kapi`olani Medical Center Honolulu, Hawaii Long Island Jewish Medical System New York, New York Via Christi Regional Medical Center Kansas City, Kansas Women & Infants Hospital Providence, Rhode Island

11. Enrollment Process • Eligible babies (Failed OAE and Passed A-ABR) were identified following newborn hearing screening. • Parents were contacted and research study explained. • Consent was obtained from families. • Enrollment data was collected. • Contact was maintained with family at 2, 4, & 6 months of age via post cards. • Babies were seen for audiological diagnostic evaluation at 8-12 months of adjusted age.

12. Date Collected for Each Participating Baby Birthdate Bronchio-pulmonary Dsplasia Gender Mechanical Ventilation >7 Days Birth Weight ECMO Gestational Age Number of Children in Home APGAR Scores Number of Adults in Home Days in NICU Total Household Income Malformations of the Head and Neck Child’s Race/Ethnicity Syndrome Associated with Hearing Loss Health Insurance In-utero Infections Family History of Hearing Loss

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

14. Enrollment of Study Participants

15. Enrollment of Study Participants(continued)

16. Audiological Diagnostic Evaluation • Visual reinforcement audiometry (VRA) • Tympanometry • OAE - Either TOAE or DPOAE

17. VRA Protocol • Protocol patterned after National institutes of Health Study (Norton, Univ. of Washington) • 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

21. Criteria for Categorizing Hearing Loss * OAEs within normal limits were defined as > 3-6dB at 1K and > 6dB at 2K and 4K.

23. PHL in Comparison Group Sites (Fail OAE/Fail A-ABR)

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

25. Degree of Hearing Loss in Study and Comparison Group Babies 28.6% 80.3% As measured in the worse ear

26. Comparability of Study and Comparison Groups • 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 child 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

27. Represents 12% of all babies with PHL in birth cohort How Many Additional Babies with Permanent Hearing Loss (PHL) were Identified? *Adjusted for proportion of OAE fails that enrolled

28. Is it important that 21 Babies (30 ears) with PHL 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 babies would you have to follow to find 21 babies PHL? • Is this congenital or late-onset hearing loss?

29. How many babies 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 babies to return for outpatient screening must be considered

30. Screening Failures True Positives False Positives

31. Cost of Screening • Direct cost • Indirect cost • Follow-up cost

32. Cost-Benefit of Screening BenefitCOST Good follow-up BenefitCOST Mediocre follow-up Benefit COST Poor follow-up

33. 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, 9 of 21 babies would still have been missed • Little is known about the incidence or what predicts late-onset hearing loss • Most (>3/4ths) of the hearing losses “missed” were mild as was expected

34. Different Criteria for Determining Permanent Hearing Loss

35. Different Criteria for Determining Permanent Hearing Loss X X X X

36. Variation Among Sites • The study design assumed that sites are all equally well implemented • To the degree that this isn’t true, data from some sites may be a better estimate of the number of babies being missed

37. Indicators of Implementation Quality at Each Site PHL=permanent hearing loss

38. Presumed Curve of Health Care

39. Bell-Shaped Curve of Health Care

40. Best Estimate of Amount of PHL Missed by OAE/AABR protocol 12% of children with PHL in birth cohort 23% of children with PHL in birth cohort 17% of children with PHL in birth cohort 23% of children with PHL in birth cohort

41. What’s the Best Estimate of the Number of Babies Missed 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

42. Conclusions • The OAE/AABR protocol, as implemented at these sites, failed to identify a substantial number of infants with PHL. • Best estimate is .55 per thousand or 24% of all infants with PHL. • Most were mild sensorineural hearing loss • Impossible from this study to determine how many are late-onset losses • About 41% might be identified if all infants with risk factors or opposite refer ears were followed, but this is not likely.

43. Recommendations • Screening for permanent hearing loss should extend into early childhood (e.g. physician’s 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/A-ABR) protocol need to be carefully considered for individual programs.

44. Further Research Recommendations • Prevalence and methods of identifying late-onset hearing loss • Identification and monitoring of progressive 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 or additional “continuous” screening and surveillance techniques, especially in early childhood

45. Questions to Ponder • What degree of hearing loss do you want to identify? • What cost will be required for that identification? • Can you assure that follow-up will occur? • What interventions can you provide? • How can you provide continuing surveillance of late onset or progressive loss? • How comfortable are you with the quality of services being provided?

46. Key Large-Scale Newborn Hearing Screening Studies in the United States

47. Previous Presentations4thInternational Conference on Newborn Hearing Screening, Diagnosis and Intervention Cernobbio, Italy - May 20042nd Conference of the CDC Centers on Birth Defects and Developmental Disabilities Washington, DC – July 2004Asia Regional Neonatal Screening ConferenceShanghai, China - September 2004UH College of Education Research SymposiumHonolulu, HI - October 2004 American Speech-Language and Hearing AssociationPhiladelphia, PA – November 2004CDC Ad Hoc Group – TeleconferenceJanuary 11, 2005EDHI ConferenceAtlanta, GA – March 2005Council on Exceptional Children, Baltimore, MD - April 20052nd International Conference on Birth Defects and Disabilities in the Developing WorldBeijing, China - September 2005

48. Publications • Johnson, JL, et al (2005). A multicenter evaluation of how many infants with permanent hearing loss pass a two-stage otoacoustic emissions/automated auditory bainstem response newborn hearing screening. Pediatrics, 116:3, 663-72. • Johnson JL, et al (2005). A multisite study to examine the efficacy of the otoacoutstic emission/automated auditory brainstem response newborn hearing screening protocol: Introduction and overview of the study. American Journal of Audiology, 14, S178-185. • White, KR, et al (2005). A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: Research design and results of the study. American Journal of Audiology, 14, S186-199. • Widen, JE, et al (2005). A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: Results of visual reinforcement audiometry. American Journal of Audiology, 14, S200-216. • Gravel, JS, et al (2005). A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: Recommendations for policy, practice, and research. American Journal of Audiology, 14, S217-228.

49. Additional Information on Newborn Hearing Screening www.infanthearing.org www.babyhearing.org jeanj@hawaii.edu

50. Deafness separates people from people. --- Helen Keller