Usher syndrome. A temporal bone report

The bilateral temporal bones of a deceased 84-year-old man who had been suffering from Usher syndrome were examined using light microscopy. Histopathologic examination disclosed degeneration of the organ of Corti that was most profound in the basal turn, degeneration of cochlear neurons in all of the turns, and severe loss of spiral ganglia in both cochleas. Endolymphatic hydrops of unknown cause and a functionally unimportant pit malformation in the macular utricle were observed in the right cochlea. We compared the aforementioned findings with temporal bone reports cited in the literature.