Autoimmune Inner Ear Disease (AIED)

A Tutorial

¹ Louisiana State University Medical Center, New Orleans
² Naval Hospital, Newport, RI

rhurle{at}lsumc.edu

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.

Key Words: autoimmune inner ear disease, autoimmune sensorineural hearing loss, immune inner ear disease, rapidly progressive sensorineural hearing loss, sudden hearing loss

Submitted on December 29, 1995
Accepted on May 26, 1996