Autoimmune Inner Ear Disease (AIED) A Tutorial Short Course
Short Course  |   March 01, 1997
Autoimmune Inner Ear Disease (AIED)
 
Author Affiliations & Notes
  • Raymond M. Hurley, PhD
    Louisiana State University Medical Center, New Orleans
    Department of Communication Disorders, LSU Medical Center, 1900 Gravier Street, New Orleans, LA 70112-2234
  • Janet P. Sells
    Naval Hospital, Newport, RI
  • Corresponding author: e-mail: rhurle@lsumc.edu
Article Information
Hearing Disorders / Balance & Balance Disorders / Short Course
Short Course   |   March 01, 1997
Autoimmune Inner Ear Disease (AIED)
American Journal of Audiology, March 1997, Vol. 6, 22-30. doi:10.1044/1059-0889.0601.22
History: Received December 29, 1995 , Accepted May 26, 1996
 
American Journal of Audiology, March 1997, Vol. 6, 22-30. doi:10.1044/1059-0889.0601.22
History: Received December 29, 1995; Accepted May 26, 1996

A portion of idiopathic rapidly progressive sensorineural hearing loss (SNHL) cases can be attributed to an immunological process in the cochlea that has been designated "autoimmune inner ear disease" (AIED). AIED is characterized by asymmetric bilateral SNHL that progresses over weeks or months, is often accompanied by vestibular symptoms, and responds positively to immunosuppressant medication. Historically, the inner ear was thought to be protected from autoimmune pathology; however, recent evidence suggests that the perisacular tissue surrounding the endolymphatic sac contains the necessary components for an immunological reaction. In addition, the inner ear is capable of producing an autoimmune response to sensitized cells that can enter the cochlea from the circulatory system through the spiral modiolar vein. Thus, AIED can be organ-specific, originating within the inner ear, or non-organ-specific, originating outside the inner ear. Although AIED is a recognized form of SNHL that may be stabilized or even reversed by appropriate medication, it remains the subject of controversy and debate. This paper discusses the pathogenesis of AIED, diagnostic strategy and treatment procedures for AIED, and the role of the audiologist in patient management.

Acknowledgment
Thanks are extended to Christopher D. Bauch for his editorial assistance and guidance and to two anonymous reviewers for their valuable comments. Thanks also to Peter L. Rigby for his comments on the first version of this paper.
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