1/1. Autoimmune sensorineural hearing loss: a human temporal bone study.PURPOSE: To describe histopathologic findings in temporal bones of a patient whose clinical history suggests a sensorineural hearing loss (SNHL) of autoimmune origin. MATERIALS AND methods: Temporal bones from a patient with a history of ulcerative colitis, leukemia, and SNHL were examined by light microscopy. RESULTS: Histopathologic findings included: (1) organs of Corti missing or absent in all cochlear turns; (2) cells decreased in spiral ganglia, and lymphocytic infiltration; (3) absence of portions of the spiral prominence; (4) endolymphatic hydrops in basal, middle, and apical cochlear turns and in the saccule and utricle; (5) fibrosis and osteoneogenesis of a scala tympani of the basal turn of the cochlea, the posterior semicircular canal, and the canal of Cotugno; (6) fibrosis of the vestibular aqueduct and endolymphatic sac; and (7) lymphocytes in the endolymphatic sac, perisaccular area, inferior cochlear vein, and Rosenthal's canal. CONCLUSION: Histopathologic findings in the temporal bones of this patient with ulcerative colitis, sensorineural hearing loss, and vestibular symptoms closely parallel those in a previously reported animal study of autoimmunity and suggest the possibility of a SNHL of autoimmune origin.
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