The primary goal of the Center for Hearing Loss in Children is to improve the future prospects of children with educationally significant hearing loss through a tightly integrated program of research, research training, continuing education and information dissemination. The Center will concern itself with children having all degrees of hearing loss, ranging from mild to profound, recognizing that each point along the continuum of impairment poses unique diagnostic and rehabilitative challenges. To achieve the goal of the Center, research efforts will be expanded in three areas: 1) development of improved assessment and remediation strategies for children with hearing loss, 2) the genetics of hearing loss, and 3) animal models of auditory system development. Two research projects are described in each of these areas. Core units will provide administrative, technical, data analysis, and media support, genetics support, and clinical support for identification and management of the hearing-impaired children who participate in research studies. All educational programs will stress the three key aspects of hearing loss in children summarized above. The research training program will encompass training of undergraduate, predoctoral, postdoctoral, and more senior investigators in the above areas, with a unique multilevel program designed to increase the number of gifted deaf students who pursue careers in the communication sciences. The continuing education program will provide training in the three areas to practicing the information dissemination program will draw on our extensive experience in this area to provide a combination of newsletters, video tapes, and other materials to the general public, parents of children with hearing loss, and to the children themselves.

National Institute of Health (NIH)
National Institute on Deafness and Other Communication Disorders (NIDCD)
Comprehensive Center (P60)
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Special Emphasis Panel (SRC (01))
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Father Flanagan's Boys' Home
Boys Town
United States
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Song, Lei; McGee, Joann; Walsh, Edward J (2008) The influence of thyroid hormone deficiency on the development of cochlear nonlinearities. J Assoc Res Otolaryngol 9:464-76
Song, Lei; McGee, JoAnn; Walsh, Edward J (2008) Development of cochlear amplification, frequency tuning, and two-tone suppression in the mouse. J Neurophysiol 99:344-55
Varga, R; Avenarius, M R; Kelley, P M et al. (2006) OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele. J Med Genet 43:576-81
Song, Lei; McGee, Jo Ann; Walsh, Edward J (2006) Consequences of combined maternal, fetal and persistent postnatal hypothyroidism on the development of auditory function in Tshrhyt mutant mice. Brain Res 1101:59-72
Song, Lei; McGee, Joann; Walsh, Edward J (2006) Frequency- and level-dependent changes in auditory brainstem responses (ABRS) in developing mice. J Acoust Soc Am 119:2242-57
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Pennings, Ronald J E; Topsakal, Vedat; Astuto, Lisa et al. (2004) Variable clinical features in patients with CDH23 mutations (USH1D-DFNB12). Otol Neurotol 25:699-706
Varga, R; Kelley, P M; Keats, B J et al. (2003) Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet 40:45-50
Higgins, Maureen B; McCleary, Elizabeth A; Carney, Arlene Earley et al. (2003) Longitudinal changes in children's speech and voice physiology after cochlear implantation. Ear Hear 24:48-70
Bhattacharya, Gautam; Miller, Caroline; Kimberling, William J et al. (2002) Localization and expression of usherin: a novel basement membrane protein defective in people with Usher's syndrome type IIa. Hear Res 163:1-11

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