Brian McCabe, MD, first described autoimmune sensorineural hearing loss in his landmark 1979 paper. McCabe described a series of 18 patients whose "clinical pattern did not fit with known entities and thus seemed to merit distinctive categorization." Since that time, autoimmune inner ear disease (AIED) or immune-mediated sensorineural hearing loss, has taken its place as a cause of sensorineural hearing loss and vestibular symptoms.
AIED occurs when the body's immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occuring in less than 1% of the 28 million Americans with a hearing loss. It can happen in isolation (as just labyrinthine disease) or as part of other systemic autoimmune disorders. Twenty percent of patients have other autoimmune diseases, such as rheumatoid arthritis or lupus.
The exact clinical description can vary somewhat; however, most agree that the syndrome follows a fairly typical course. AIED will manifest as rapidly progressing, sensorineural hearing loss that is most commonly bilateral. Typically, it begins unilaterally and ultimately affects the other ear, and it can occur suddenly. Word recognition is usually disproportionately poor. Vestibular symptoms can have a fluctuating course during the active phase of disease. Additionally, tinnitus, as a ringing, roaring, or hissing noise, may be present. Diagnosis can be difficult because AIED can mimic other ear disorders. In any patient with presumed AIED, it is essential to rule out other causes of progressive sensorineural hearing loss, such as acoustic neuroma, neurosyphilis, and Meniere's, as well as other metabolic conditions.
Because of the difficulty in the differential diagnosis of AIED, many have proposed the use of lab tests to assist in the medical diagnosis. McCabe originally recommended an "immune screen" to include
Later, Campbell and Klemens (2000) listed other medical tests they commonly use to detect AIED:
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