Autoimmune sensorineural hearing loss: A human temporal bone study

Dick L. Hoistad, Patricia A. Schachern, Michael M. Paparella

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)33-39
Number of pages7
JournalAmerican Journal of Otolaryngology - Head and Neck Medicine and Surgery
Issue number1
StatePublished - Jan 1 1998

Bibliographical note

Funding Information:
Sensorineural hearing loss (SNHL) can be attributed to many causes and is often irreversible. One type (autoimmune SNHL), if clinically detected and treated, may be partially or fully recoverable. In recent years, this distinct type of hearing loss has become a popular topic for discussion and investigation but remains incompletely understood. In 1958, the first case report of an immune reaction suspected as the etiologic agent for bilateral sudden deafness was presented by Lenhardt.’ Terrayama and Saski3 and BeickerF soon published experimental data concerning autoimmune reaction in the cochlea of guinea pigs. But it was not until McCabe4 described 18 patients with autoimmune SNHL in a 1979 From the Otitis Media Research Center, Department of Otolaryngology, School of Medicine, University of Minnesota; the Minnesota Ear, Head, and Neck Clinic; and the International Hearing Foundation, Minneapolis, MN. Supported in part by NIH NIDCD Grant no. P50-DC-03093 and by the International Hearing Foundation, Minneapolis, MN. Address reprint requests to Patricia A. Schachern, BS, Rm 226, Lions Research Building, 2001 6th St SE, Minneapolis, MN 55455. Copyright o 1998 by W.B. Saunders Company 0196-0709/98/l 901-0007$8.00/O study of diagnosis and treatment that this disorder became widely accepted as an entity.


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