Autoimmune vestibulo-cochlear disorders

Autoimmune vestibulo-cochlear disorders (AVCD) represent a group of syndromes with overlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertigo, tinnitus, and aural fullness, and believed to be caused by an autoimmune mechanism. Although definitive evidence of a classic "autoimmune process" is still lacking, substantial indirect evidence has accumulated to strongly indicate such a pathogenesis. Rapidly progressing AVCD is analogous to rapidly progressive glomerulonephritis in that inner ear inflammation progresses to severe, irreversible damage within 3 months of onset (and often much more quickly). Thus patients with rapidly progressive AVCD are treated with a sense of urgency. Prompt treatment with corticosteroids and other antirheumatic/immunosuppressive agents can preserve hearing and vestibular functions. We are not aware of any randomized controlled clinical trials evaluating the efficacy of antirheumatic/immunosuppressive agents in AVCD. In this article we review reports of various therapies that have been tried in this condition and our experience of etanercept therapy in AVCD.