Immune-mediated inner ear disease is a nonspecific term used to describe cochleovestibular dysfunction that results from immune-mediated injury. The ear may be involved directly or secondarily by the immune reaction, and evidence exists to implicate all four of the Gell and Coombs mechanisms of immune injury. The clinical presentation may be variable, with the diagnosis based on presentation, laboratory studies, and response to therapy. Current research is directed toward determining antigen-specific laboratory tests to improve diagnostic capabilities, examining human temporal bones to elucidate the pathophysiologic mechanisms involved, and developing a clinical laboratory and pathologic correlation.
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