Autoimmune inner ear disease.

Department of Otorhinolaryngology, Head and Neck Surgery, University of Pennsylvania, 3400 Spruce Street, 5 Ravdin, Philadelphia, PA 19104, USA.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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, which initially showed promise, has recently been shown to be ineffective in preventing progression of hearing loss. Further research is required to better delineate the pathophysiologic mechanisms involved in AIED and to establish more effective and better tolerated treatment regimens. SUMMARY: The original enthusiasm that was generated by the concept that immune-mediated mechanisms may mediate reversible forms of inner ear pathology has been tempered by the realities conveyed by scientific research. The pathophysiology of AIED is still not well understood. Multiple potential mechanisms have been identified that can result in immune-mediated inner ear pathology. The diagnosis of AIED is based on clinical presentation and response to the administration of corticosteroids. Diagnostic testing may support the diagnosis, and the results of a recent multicenter study should clarify the role of immunologic testing in the diagnosis of this entity. Treatment options are limited, with corticosteroids being the only validated treatment option, and methotrexate offering no significant benefit to patients. The role of cyclophosphamide is restricted to patients willing to endure the attendant risks. With the advent of cochlear implants, the administration of toxic medications to preserve hearing at all costs is a less desirable

Immune-mediated inner ear disease: 10-year experience.

Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA.

BACKGROUND: Autoimmune inner ear disease (AIED) was first described in 1979 and the disease has become more widely recognized over the last decade. Limited information is available regarding clinical features of the disease, disease course, and response to treatment. OBJECTIVE: To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome. METHODS: A retrospective chart review was conducted on all patients considered to have AIED by the Otolaryngology division physicians and on all patients positive for antibody testing to inner ear antigens from 1990 to 1999. Patients who were antibody positive with a clinical diagnosis of AIED were included in this review. RESULTS: Patients with AIED presented with rapidly progressive, frequently bilateral (79%), often fluctuating sensironeural hearing loss. Mean age at presentation was 50 years (22-80) with no gender predilection. Tinnitus (83%), vestibular complaints (79%), and Menieres (50%) were common concomitant symptoms along with hearing loss. Seven of 42 (17%) of the patients had evidence for other systemic autoimmune disorders. In 4 of the patients the onset of vestibuloauditory complaints preceded the diagnosis of autoimmune disorder. Thirty-three of 42 demonstrated antibodies to inner ear antigens but other autoantibodies were infrequent except in patients with systemic autoimmune disorders. Twenty-three of 33 (70%) of patients treated with corticosteroids improved clinically, often short-term. Sixteen patients received treatment with other immunosuppressive drugs including methotrexate, cyclophosphamide, azathioprine, mycophenolic mofetil, and intravenous immunoglobulin (IVIg) generally with a limited modest response. Using clinical trial criteria for response, only 5/35 (14%) demonstrated improvement over a mean 34.4 months follow-up. CONCLUSIONS: Immune-mediated inner ear disease is not a uniform disease with simple diagnosis or treatment. The course of the disease often results in significant long-term disability due to hearing loss and response to aggressive immunosuppression including corticosteroids is poor.

PMID: 15505770 [PubMed - indexed for MEDLINE]