Histopathologic features of the temporal bone in Usher syndrome type I

Temporal bones of 2 patients with Usher syndrome type I were examined using light microscopy. In both patients, findings from histopathologic examinat ion of the cochlea were characterized by degeneration of the organ of Corti , which was most marked in the basal turn, atrophy of the stria vascularis, and a decrease in the number of spiral ganglion cells. The cochlear nerve appeared to be diminished. The sensory epithelium of the saccular and utric ular maculae of patient 1 was normal for age. The left temporal bone of pat ient 2, classified as Usher syndrome genetic subtype USH1D or USH1F, demons trated the typical signs of severe cochleosaccular degeneration. Present ca ses and cases from the literature were reviewed in search of an explanation for the above-described differences in histologic findings.