NIDCD Grantees Investigating Long Term Outcomes of EHDI Programs

1. NIDCD Grantees Investigating Long Term Outcomes of EHDI Programs Amy M. Donahue, Ph.D. NIDCD

2. Faculty Disclosure Information In the past 12 months, I have not had a significant financial interest or other relationship with the manufacturer(s) of the product(s) or provider(s) of the service(s) that will be discussed in my presentation This presentation will (not) include discussion of pharmaceuticals or devices that have not been approved by the FDA or if you will be discussing unapproved or "off-label" uses of pharmaceuticals or devices.

3. National Institute on Deafness and Other Communication Disorders (NIDCD) and EHDI Research 2006 National Early Hearing Detection and Intervention Conference Amy Donahue, Ph.D. NIDCD

4. Much of the biomedical research in the United States is supported by the Federal Government, primarily the National Institutes of Health (NIH)

5. NIDCD Mission and Program Activities • Biomedical and Behavioral Research • Hearing, Balance, Smell, Taste, Speech, Voice and Language • Research Grants and Contracts to Universities and Small Businesses • Basic, Translational, and Clinical • Technology Development

6. NIDCD Budget & Success Rate FY2000 2001 2002 2003 2004 2005 2006 NIH budget doubling $(M) 264 300 341 370 382 394 397 % 15.0 14.0 13.6 8.4 3.2 3.2 -0.5 Increase R01 40 43 41 42 36 27 ? Success Rate (Awarded/Reviewed)

7. NIDCD FY 2005 Budget Distribution by Program Hearing 51 % Balance 7 Smell 12 Taste 5 Voice 6 Speech 10 Language 9

8. NIDCD 2005 Success Rates Success Mechanism Reviewed Awarded Rate (%) R01 514 141 27.4 R03 (Small) 95 29 30.5 R15 (AREA) 18 6 33.3 R21 (Exp/dev) 121 19 15.7 New Inv R01 88 24 27.3

9. NIDCD and EHDI • Active NIDCD investigator-initiated research grants in many areas relating to HL in infants and children • Set-aside money to fund special initiatives focusing on urgently needed information (over $6 M in recent years) • Audiology, speech pathology, early intervention and medical communities are actively engaged • An adequate pool of both funding dollars and trained researchers is required to address future needs • Often takes years to get answers, requiring patience and perseverance…

10. EHDI-Related Research(Investigator-Initiated) • Cochlear Implants • Hearing Aids • Normal Auditory Development • Auditory, Speech and Language Development after Hearing Loss • Device Development- Diagnostic and Rehabilitation

11. EHDI-Related Research(Investigator-Initiated) • Behavioral Assessment and Management Test Development • Speech and Lang Development in Infants and Children • Acquisition of American Sign Lang • Genetics and Hearing Loss

12. Special EDHI Initiatives • Value and seek dialogue with scientific community and federal partners for priority setting • Set aside money to develop and support special initiatives to obtain urgently needed information • Seeking answers to the changing issues and questions before us

13. Intervention Strategies Following Early Identification of Hearing Impairment • $1.5M and funded four applications • Auditory Development in Early Amplified Children (UCLA) • Optimizing Amplification for Infants and Young Children with Hearing Loss (Boys Town) • Effect of Very Early Cochlear Implantation (Washington University) • Improving Speech Intervention for Deaf Children (University Minnesota)

14. Genetic Testing and the Clinical Management of Infants with Nonsyndromic Hearing Impairment • $1.5M and funded four applications • How will genetic testing be integrated into the clinical management of the individual and utilized by the family • What are the attitudes about genetic testing or the level of interest • Unique challenges for a condition not considered to be disabling or undesirable by everyone

15. Genetic Testing and the Clinical Management of Infants with Nonsyndromic Hearing Impairment • Genetics and Pediatric Nonsyndromic Hearing Loss (BU/Harvard) • Outcomes of Genetic Testing for Hearing Impairment (U PN) • Connexin 26 Testing in Infants (UCLA) • Genetics and Mild Hearing Loss in Schoolage Children (Univ Australia)

16. Auditory/Perceptual Processing in Infants with Hearing Loss: Issues in Assessment and Management • 2M dollars and funded 6 applications • Multidisciplinary research • Understand how early hearing loss affects the whole child: sign and/or spoken language, cognition, learning, psychosocial development • Develop new tools and methodologies for assessment and management

17. Auditory/Perceptual Processing in Infants with Hearing Loss: Issues in Assessment and Management • Word Learning in Infants with HL (Boystown) • Early Development of Children with HL Loss (Ohio State) • Assessing Auditory Capacity of HL Children (HEI) • Central Auditory Development in HL Infants (U TX) • Speech Perception by Infants after Cochlear Implantation (Indiana U)

18. Congenital Cytomegalovirus Approx 40,000 infants year born with CMV 10- 15% will develop HL, many will have delayed onset 7-year study to evaluate presence of CMV and long-term audiologic/otologic outcome and address validity and utility of CMV screening in the detection of HI in the newborn period and prediction of late-onset HL after newborn period

19. Congenital Cytomegalovirus University of Alabama, Birmingham Seven sites- MS, NC, NJ, PA, OH, TX, AL Three Phases: • Protocol development • Determination of best screening methodology (saliva or blood drop) • Testing of 100,000 newborns for CMV and follow-up of positive cases

20. Long Term Outcomes: Childhood Outcomes After Cochlear Implantation (JHU) “Whole” Child: cognitive, social and behavioral development. Systematically evaluating the predictive value of variables as they relate to outcomes of oral language acquisition, speech recognition skills, selective attention and problem-solving skills, behavioral and social development, parent-child interactions, and quality-of-life measures in children implanted in six U.S. implant centers.

21. Long Term Outcomes: Childhood Outcomes After Cochlear Implantation (JHU) Data will contribute to understanding of the factors predicting implant-associated language use, communication competence in early childhood, psychosocial development, and the perceived value of early cochlear implantation in light of associated costs. Conclusions will enable a refined approach to implant candidacy when considering (re)habilitative strategies designed to optimize the development of children with severe-to-profound SNHL.

22. Long Term Outcomes: Iowa Cochlear Implant Center Clinical outcomes research in 20th year. Goals include: studying the selection criteria for implantation; developing a reliable evaluation strategy including accurate assessment of auditory thresholds; developing signal processing for speech perception and music appreciation; assessing communication outcomes (speech production, language, reading, writing, and music appreciation) of children using hearing aids and/or implants; and determining the factors that affect the long term benefit of cochlear implants in adults and children.

23. Long Term Outcomes:Word Learning in Infants with Hearing Loss (Boys Town) Goal is to gain an understanding of the early word-learning processes of children with hearing loss, and to delineate factors that influence language development. Many hard-of-hearing (HH) children are slower than normal-hearing (NH) children to develop similar lexicons, even with early intervention. Sources of early delays and individual differences in long-term outcomes in this population are not well understood. Is early lexical development influenced by complex interactions of maternal, child, and environmental factors.? This study systematically examines the factors that influence word learning in HH infants.

24. Long Term Outcomes:Word Learning in Infants with Hearing Loss (Boys Town) Characterize the rate and course of word learning in HH children in the context of mother-child interaction, providing baseline data on word learning in HH children, as well as guidelines for measuring progress and adjusting interventions in clinical settings. Word-learning experiments and parent surveys are conducted to determine if HH children are able to learn words from incidental exposure. Explore the role of children's social understanding in vocabulary development, and determine how conversations about thoughts and feelings influence children's knowledge. Outcome to provide direction for early interventionists in their efforts to promote nurturing caregiver-child interactions.

25. Long Term Outcomes: Assessing Auditory Capacity of HL Children (HEI) There are few clinical measures to assess speech perception abilities in infants as they mature. Better measures are needed for decision-making regarding early intervention. This study focuses on auditory-perceptual development and the emergence of spoken language in the young hearing-impaired child. Goal: develop and refine new behavioral assessment tools to measure speech perception in children between the ages of 6 months and 5 years. Longitudinal and cross-sectional studies.

26. Long Term Outcomes: Assessing Auditory Capacity of HL Children (HEI) Aims include: 1) ascertain the extent to which assessment task contributes to variability on measures of speech perception, 2) measure changes in performance over time as a function of chronological age, assessment task, auditory status, and sensory assistance; 3) investigate the relationship between speech recognition and linguistic / cognitive variables in young developing children with hearing loss; 4) to describe and measure the contributions of maternal involvement to performance on tasks of language and speech perception in hearing-impaired young children, and to validate parent report as a tool for assessing language development.

27. Health Communication • Research to investigate methods to inform the public and to be used by public health and professional audiences in information dissemination. • Resources for infants with newly identified HL (Boystown, Univ Arkansas, Children’s Hospital Philadelphia)

28. NIDCD Office of Health Communication and Public Liaison • Communicate NIDCD-supported research to the public, health professionals • Publications, Web site, Information Clearinghouse • Media Relations • Outreach, Exhibits, Conferences

30. Fact Sheets for Parents and Medical Professionals • Newborn Hearing Screening: Has Your Baby’s Hearing Been Screened? 3200 • What to Do if Your Baby’s Screening Reveals a Possible Hearing Problem 2250 • When a Newborn Doesn’t Pass the Hearing Screening: How Health Professionals Can Encourage Follow-up Hearing Evaluation for Newborns 300 • Communication Considerations for Parents of Deaf and Hard-of-Hearing Children 1900 Annual distribution through the NIDCD Information Clearinghouse:

33. Outreach • Respond to inquiries at NIDCD Information Clearinghouse • Disseminate at medical, health, and prevention conferences • Participation in EHDI activities, conferences • Media opportunities to promote awareness of newborn screening, follow-up care

34. Healthy Hearing 2010 Progress Review • In 2001, 66% of infants received a hearing screening before they were one month old. 2010 target: 90% • Of infants who needed follow-up evaluation, 56% received this service before 3 months of age. 2010 target: 70% • Of infants who had hearing loss during the follow-up evaluation, 57% were enrolled in appropriate intervention before the age of 6 months. 2010 target: 85% • NIDCD continues to develop new tools to evaluate and modify intervention programs in newborns.

35. Resources & Contact Information • Visit NIDCD’s Web site: www.nidcd.nih.gov • Research funding opportunities: Dr. Amy Donahue or Dr. Lynn Luethke (301) 402-3458 donahuea@nidcd.nih.gov luethkel@nidcd.nih.gov • Subscribe to NIDCD: updates on research findings, biannual newsletter

36. Resources & Contact Information • Visit NIDCD booth in the Exhibit Hall Contact: Patricia Blessing Chief, Office of Health Communication and Public Liaison, NIDCD 301-496-7243 blessinp@mail.nih.gov