Hereditary progressive sensorineural deafness

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Abstract
Progressive sensorineural hearing losses found in 7 members of 3 families are presented. Genetic transmission patterns in the study appeared to be autosomal dominant in 2 families and recessive in one. The common audiological features of these cases include bilaterally symmetrical audiometric configuration and fairly good speech discrimination. The hearing losses in most of the cases appeared to begin at high frequencies progressing later to involve lower frequencies. The main histopathological changes in the temporal bones of 1 of the cases were degeneration of the organ of Corti and of the spiral ganglion in the lower cochlear coils and cystic degeneration of the stria vascularis in the upper coils.

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