Immunosuppressive Therapy for Autoimmune Inner Ear Disease

Abstract

Autoimmune inner ear disease (AIED) is a rare disease that is diagnosed after clinical suspicion and response to corticosteroids. AIED manifests as progressive, bilateral, although often asynchronous, sensorineural hearing loss and can be associated with vestibular symptoms. Since its description as a defined disease entity in 1979, the initial mainstay of treatment remains high-dose corticosteroids. Several animal models have been developed to assist in determining efficacy of immunosuppression in AIED, and several clinical studies have also investigated the role of both steroid and steroid-sparing treatments. Here we discuss the basic science and clinical research surrounding the history of immunosuppressive therapy in AIED. Keywords autoimmunity; corticosteroid; hearing loss; immunomodulation; immunosuppression Autoimmune inner ear disease (AIED) was first described by McCabe in 1979 [1]. AIED is one of the few treatable forms of sensorineural hearing loss and is diagnosed on clinical suspicion. Autoimmune sensorineural hearing loss is characterized by bilateral disease, often with the severity of hearing loss being asymmetric. In McCabe's initial report, hearing loss was slowly progressive and nonfluctuating, worsening over the course of weeks to months. Some cases were associated with temporary facial paralysis and tissue destruction. However, vertiginous episodes were rarely observed. A lymphocyte-migration inhibition assay was the only laboratory test available and assessed the ability of inner ear homogenate to inhibit migration of the patient's peripheral blood mononuclear cells (PBMCs). Most importantly, the auditory symptoms responded to immunosuppressive therapy. McCabe's report summarized data from 18 patients seen and treated over the course of 10 years.In 1984, Hughes and colleagues published a clinical profile for autoimmune hearing loss developed from 15 patients with laboratory-suggested AIED [2]. In addition to McCabe's observations, Hughes concluded that AIED may present as a localized primary disease or be present in association with a systemic autoimmune disorder, being referred to as secondary. Approximately 30% of patients with AIED have a systemic autoimmune disease [3]. In contrast to McCabe's initial report, Hughes concluded that hearing loss could begin abruptly, fluctuate over time, and occur with or without vertigo.As reviewed by Hughes and colleagues, treatment at the time was guided by both the experience of the clinician and theoretical management of active autoimmune response [2,4]. Glucocorticoids were, and still remain to be, the first-line therapy for AIED. Those who failed glucocorticoid therapy were then offered other immunosuppressive agents, with plasmaphoresis being reserved for the most resistant cases. In the three decades since the first report of autoimmune hearing loss, research on the molecular mechanisms underlying this disease and investigation of treatment efficacy have advanced clinical understanding and practice. Our goal here is to provide an overview...

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