Study of systemic lupus erythematosus in temporal bones

Despite some reports of sensorineural hearing loss with systemic lupus erythematosus (SLE), its pathologic correlate has remained unidentified due to the scarcity of human temporal bone studies. We here present findings in 14 temporal bones from 7 patients with SLE, examined histologically and immunohistochemically for pathologic conditions in the cochlea that might relate to their otologic histories. Blue-staining concretions were seen in the stria vascularis of 6 ears. Most of the cases showed a loss of spiral ganglion cells, with various degrees of hair cell loss and atrophy of the stria vascularis. One ear demonstrated formation of fibrous tissue and bone throughout the cochlea, with complete loss of the membranous labyrinth. Cochlear hydrops was found in only 1 ear. These findings in temporal bones from patients with SLE are discussed in relation to autoimmune disease of the inner ear.