The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls

The purpose of this paper is to review the current diagnostic work-up for patients with suspected Autoimmune Inner Ear Disease (AIED). AIED is a rare disease accounting for less than 1% of all cases of hearing impairment or dizziness, characterized by a rapidly progressive, often fluctuating, bilateral SNHL over a period of weeks to months. While specific tests for autoimmunity to the inner ear would be valuable, at the time of writing, there are none that are both commercially available and proven to be useful. Thus far, most of the identified antigens lack a clear association with localized inner ear pathology and the diagnosis of AIED is based either on clinical criteria and/or on a positive response to steroids. For clinical practice, we recommend an antigen-non-specific test battery including blood test for autoimmune disorders and for conditions that resemble autoimmune disorders. Nevertheless, if financial resources are limited, a very restricted work-up study may have a similar efficiency.