Autoimmune inner ear disease

Current Opinion in Otolaryngology & Head and Neck Surgery
Michael J Ruckenstein

Abstract

The role of the immune system in mediating inner ear pathology has received considerable attention over the past two decades. The purpose of this paper is to summarize recent basic science research into the pathogenesis of autoimmune inner ear disease (AIED), review the current diagnostic work-up for patients with suspected AIED, and delineate treatment strategies. Basic science and clinical studies have been performed to delineate the mechanisms by which autoimmune processes may affect the ear and to develop treatment strategies to reverse this pathology. AIED refers to a rapidly progressive (over a course of weeks to months) sensorineural hearing loss that responds to the administration of corticosteroids. In addition, systemic autoimmune diseases (eg, vasculitides, lupus, Wegener granulomatosis) can secondarily affect the inner ear. Although a number of diagnostic tests for AIED have been advocated, the diagnosis of this entity is still predicated on a positive therapeutic response to corticosteroid administration. Alternate immunosuppressive regimens, designed to be used in patients who cannot be weaned off corticosteroids or whose disease becomes refractory to treatment, have proven to be difficult to develop. Methotrexate...Continue Reading

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