The hearing section has recently published the results of a detailed clinical characterization of hearing and balance dysfunction in a large series of patients with Stickler syndrome and Marshall syndrome. These syndromes are phenotypically similar disorders of connective tissue that are caused by mutations in genes encoding fibrillar collagens. Hearing loss is a common feature of these disorders, and our work provided a detailed description of the hearing loss associated with these disorders, as well as a demonstration of a correlation between the degree and progression of sensorineural hearing loss with the underlying fibrillar collagen genotype. Another recent accomplishment was the publication of audiologic monitoring guidelines for ototoxicity associated with the use of cancer chemoprevention agents. A major focus of the hearing section has continued to be the characterization of hereditary disorders primarily affecting hearing and balance. The hearing section has now evaluated over 2 dozen individuals with hearing loss associated with enlargement of the endolymphatic system of the inner ear. A manuscript has been written describing the auditory manifestations of observed in patients treated for Lyme disease. Such patients often experience nonspecific auditory symptoms, and a variety of nonspecific audiologic findings, the most prominent of which appears to be a reduction in loudness discomfort level. The hearing section continues to evaluate patients with Smith-Magenis syndrome, Von Hippel-Landau disease, Pallister-Hall syndrome, and Greig Cephalopolysyndactyly syndrome as part of ongoing collaborative studies of the otolaryngologic and audiologic manifestations of these heritable disorders. Finally, Ms. Mastroianni has accrued several dozen patients and volunteers for her study of the efferent auditory system in children with attention deficit disorders. This study utilizes suppression of otoacoustic emissions to examine efferent auditory function in these subjects. The data are currently being analyzed to determine if efferent auditory activity is abnormal in the experimental group.
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