1. Severe hearing loss in children: background information Nan Bernstein Ratner, F-AAAS Department of Hearing and Speech Sciences University of Maryland, College Park 20742 nratner@umd.edu Speakers sponsored by the American Speech-Language-Hearing Association

2. How many children are impacted? ~ 1 to 6 per 1,000 newborns show hearing impairment at birth and are potentially identifiable by newborn and infant hearing screening. However, some congenital hearing loss may not become evident until later in childhood (see Bernstein Ratner, 2012). Approximately 738,000 individuals in the U.S. have severe to profound hearing loss. Of these, almost 8% (~ 60,000) are under the age of 18. The majority of severe hearing loss is pre-linguistic (before learning to talk – the most detrimental to educational outcomes) http://www.asha.org/public/hearing/Prevalence-and-Incidence-of- Hearing-Loss-in-Children/ http://www.asha.org/public/hearing/Prevalence-and-Incidence-of- Hearing-Loss-in-Children/

3. Things we now know that make this research important Children without some form of useful implant or amplification/hearing aid (which these children cannot use) have very poor educational outcomes Historically, with only amplification (hearing aids) and special education services, many students graduated school with less than functional literacy skills. For historical impacts, see Bernstein Ratner (2012)

4. What are the typical impacts of severe HL on children’s development WITHOUT adequate amplification/access to speech signal?

5. Prior to implantation, Deaf children show significant delays in interpersonal socialization Such as joint attention with parents during play (Cejas, et al., 2014)

6. The earlier we implant children, the better their language outcomes Significant advantages seen, even after years of use/experience is accounted for (e.g., Geers & Nicholas, 2013; Tobey et al., 2013)

7. We must help children for whom CIs are not appropriate As the speakers in this session will illustrate, many children are not eligible to receive cochlear implants, And others do not show benefit from them, suggesting a need for alternative approaches

8. We must educationally support children even after implantation Need for auditory habilitation E.g. Dettman et al. (2013); Beers et al. (2014) Address memory and attention, as well as speech/language skills Dr. Eisenberg’s work suggests that ABI children may require more support than CI children Need to address other risk factors for educational failure E.g., many profoundly deaf children (~30-40%) have additional impairments (see Bernstein Ratner, 2012; Hayward, et al., 2013) Many children with HL live in low SES homes: parental language stimulation is a large predictor of child language growth in such cases (Szagun, et al., 2012) – children require enriched language to HEAR in order to benefit most from implantation

9. Take-home messages: Despite advances in technology, we have yet to “cure” childhood deafness in many cases, and must continually seek new options Technology, in and of itself, does not remedy the impacts of childhood deafness Children will still require additional support from educators, speech-language pathologists, audiologists and family members

10. References, further resources Beer, J., Kronenberger, W. G., Castellanos, I., Colson, B. G., Henning, S. C., & Pisoni, D. B. (2014). Executive functioning skills in preschool-age children with cochlear implants. Journal of Speech, Language & Hearing Research, 57(4), 1521-1534. doi:10.1044/2014 Bernstein Ratner, Nan (2012). Atypical speech and language development. In J. Berko Gleason & N. Bernstein Ratner (eds.) The development of language (8th ed.). Boston: Pearson. (pp 266-328) Cejas, I., Barker, D. H., Quittner, A. L., & Niparko, J. K. (2014). Development of joint engagement in young deaf and hearing children: Effects of chronological age and language skills. Journal of Speech, Language & Hearing Research, 57(5), 1831-1841. doi:10.1044/2014 Dettman, S., Wall, E., Constantinescu, G., & Dowell, R. (2013). Communication outcomes for groups of children using cochlear implants enrolled in auditory-verbal, aural-oral, and bilingual-bicultural early intervention programs. Otology & Neurotology, 34(3), 451-459. doi:10.1097/MAO.0b013e3182839650

11. Eisenberg LS, Johnson KC, Martinez AS, Visser-DumontL, Ganguly DH, Still JF. Studies in pediatric hearing loss at the House Research Institute. J Am Acad Audiol. 2012;23:412-421. Eisenberg LS, Johnson KC, Martinez AS, et al. Comprehensive evaluation of a child with an auditory brainstem implant. Otol Neurotol. 2008;29:251- 257. Geers, A. E., & Nicholas, J. G. (2013). Enduring advantages of early cochlear implantation for spoken language development. Journal of Speech, Language & Hearing Research, 56(2), 643-653. doi:10.1044/1092- 4388(2012/11-0347) Hayward, D. V., Ritter, K., Grueber, J., & Howarth, T. (2013). Outcomes that matter for children with severe multiple disabilities who use cochlear implants: The first step in an instrument development process. Canadian Journal of Speech-Language Pathology & Audiology, 37(1), 58-69.

12. Szagun, G., Stumper, B., Oetting, J., & Tobey, E. (2012). Age or Experience? The Influence of Age at Implantation and Social and Linguistic Environment on Language Development in Children With Cochlear Implants. Journal of Speech, Language & Hearing Research, 55(6), 1640-1654. doi:10.1044/1092-4388(2012/11-0119) Tobey, E. A., Thal, D., Niparko, J. K., Eisenberg, L. S., Quittner, A. L., & Wang, N. (2013). Influence of implantation age on school-age language performance in pediatric cochlear implant users. International Journal of Audiology, 52(4), 219-229. doi:10.3109/14992027.2012.759666

13. See also: http://www.asha.org/aud/Facts-about-Pediatric-Hearing-Loss/ ASHA Practice Portal page on Permanent Childhood Hearing Loss http://www.asha.org/Practice-Portal/Clinical-Topics/Permanent-Childhood- Hearing-Loss/ http://www.asha.org/Practice-Portal/Clinical-Topics/Permanent-Childhood- Hearing-Loss/ Prevalence of early hearing loss (US): http://www.cdc.gov/ncbddd/hearingloss/2009- data/2009_ehdi_hsfs_summary_508_ok.pdf http://www.cdc.gov/ncbddd/hearingloss/2009- data/2009_ehdi_hsfs_summary_508_ok.pdf Prevalence of early hearing loss (International): http://www.cdc.gov/ncbddd/hearingloss/documents/international-hearing-loss- data-table.pdf http://www.cdc.gov/ncbddd/hearingloss/documents/international-hearing-loss- data-table.pdf Even in America, deaf children can go undetected for years: http://www.nytimes.com/2014/02/23/opinion/sunday/kristof-when-even-the- starting-line-is-out-of-reach.html?_r=0 http://www.nytimes.com/2014/02/23/opinion/sunday/kristof-when-even-the- starting-line-is-out-of-reach.html?_r=0