Semin Hear 2001; 22(4): 339-346
DOI: 10.1055/s-2001-19109
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Defining Auditory Characteristics in Hereditary Hearing Loss

Linda J. Hood
  • Department of Otolaryngology and Biocommunication, Louisiana State University Health Sciences Center, New Orleans, Louisiana
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Publication History

Publication Date:
18 December 2001 (online)

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ABSTRACT

Genetic factors contribute to more than half of all congenital hearing losses and also are responsible for later onset hearing losses. Hereditary hearing losses may be part of a syndrome, although the majority are nonsyndromic in nature. Broad variability exists in auditory characteristics of hereditary hearing losses even when the underlying genetic factors do not differ. Sensitive measures, such as otoacoustic emissions, are necessary to characterize hereditary hearing loss adequately. Individuals with nonsyndromic and syndromic hearing losses as well as unaffected carriers of genes related to recessive hearing losses have been studied. These studies suggest that otoacoustic emissions may provide a sensitive measure of auditory function and may play a part in understanding the roles of various genes in hearing.

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