To systematically evaluate the evidence for treatment of autoimmune inner ear disease (AIED).
Medline/EMBASE, Cochrane Review.
Studies reviewed were English language original articles reporting outcome data for subjects with AIED undergoing any treatment other than oral steroids alone with particular emphasis on prospectively collected data.
The full text of articles meeting selection criteria was reviewed for subject inclusion criteria, number of subjects, treatment type and duration, audiologic and vestibular evaluations, blinding, randomization, and follow-up.
Twelve studies representing 272 subjects met selection criteria and presented prospective data, three of which were randomized and placebo controlled. Low numbers and heterogeneity between studies precluded meta analysis.
Clear evidence of an effective treatment for AIED from high-quality prospective trials remains lacking.