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Distinguishing between neuroepithelial and cochleosaccular dysfunction in hereditary hearing impairment may provide prognostic and therapeutic information.
It appears that many forms of syndromic and nonsyndromic hereditary hearing impairment are secondary to either neuroepithelial or cochleosaccular dysfunction. Making this distinction can be difficult in human temporal bone specimens; however, this added knowledge may ultimately provide prognostic and therapeutic information in hearing habilitation. Fundamental studies using animal models of different types of hereditary deafness may also prove useful in this respect.
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Smith et al. (1992) studied this question.
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