USHER SYNDROME - A TEMPORAL BONE REPORT

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