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.