ABSTRACT
Responsiveness to steroid or immunosuppressant treatment is a considered characteristic of autoimmune inner ear disease (AIED). AIED is likely to represent only 1% of cases of hearing loss, although this may be an underestimate. AIED should also be considered in patients with Meniere’s disease affecting one ear who subsequently develops fluctuating hearing thresholds in the contralateral ear. Arriving at a diagnosis of AIED can be challenging, particularly when there is no suggestion of systemic autoimmune disease. In primary AIED, antibodies to Heat shock proteins 70 have also been identified in up to 47% of Meniere’s disease patients, suggesting a possible immunological role for the disease. In systemic cases of secondary AIED with hearing loss, the patient is best managed in a multidisciplinary manner with rheumatologists, otolaryngologists and audiologists. Immunosuppression is the mainstay of treatment for primary single organ AIED. Clinicians need a high index of suspicion in order to diagnose AIED.
