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August 1995

Usher Syndrome: A Temporal Bone Report

Author Affiliations

From the Department of Otorhinolaryngology, University Hospital Nijmegen (the Netherlands) (Drs van Aarem and Cremers); and Institute for the Deaf and the Blind 'Kalorama,' Beek/Ubbergen, the Netherlands (Dr Benraad-van Rens).

Arch Otolaryngol Head Neck Surg. 1995;121(8):916-921. doi:10.1001/archotol.1995.01890080082016

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.

(Arch Otolaryngol Head Neck Surg. 1995;121:916-921)

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