Acute bilateral deafness with nephritis: a human temporal bone study

Temporal bone pathology is described in a 37-year-old man who had acute, bilateral, profound sensorineural hearing loss without improvement 4 months before death. The patient had suffered from low complement nephritis, for which he had received prednisolone therapy. Autopsy revealed malignant lymphoma with non-Hodgkin's type, membranous proliferative glomerulonephritis and necrotizing vasculitis of middle and small arteries. In the temporal bone study, pathological changes were limited to the cochlear region. The vestibular structure showed no detectable pathological changes. The changes included total absence of the organ of Corti, atrophy and/or disappearance of the stria vascularis in the upper turns, collapse of Reissner's membrane in the middle turn, and new bone formation in the apical turn.