Histopathology of the inner ear in Usher's syndrome as observed by light and electron microscopy

A case of Usher's syndrome, type 3, is described. Histologic study of the inner ear of the patient demonstrated hair cell loss in the basal turn, severe loss of spiral ganglion cells, widespread neural degeneration in the cochlea, and discrete collections of degenerating supporting cells in the organ of Corti. The pattern of neural degeneration bore some similarity to abnormalities in the retina in retinitis pigmentosa. However, the findings in the supporting cells had no obvious parallel to the findings in the retina in this disorder. The histopathology of Usher's syndrome has been equated with the Scheibe, or cochleosaccular, pattern of degeneration. However, the findings in the present case and a critical analysis of published data did not support this concept.