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New Treatments for Autoimmune Inner Ear Disease

 The patient's own immune system may become reactive to self antigens resulting in autoimmune disease. Autoimmune inner ear disease may be associated with multisystemic autoimmune diseases (e.g., systemic lupus erythematosus) or it may occur as an organ-specific process (primary autoimmune inner ear disease). autoimmune inner ear disorder.

Incidence and Epidemiology

Inner ear dysfunction is a rare complication of multisystemic autoimmune diseases (e.g., polyarteritis nodosa, systemic lupus erythematosus). Similarly, primary autoimmune inner ear disease represents a rare cause of labyrinthine pathology. These processes represent, however, some of the few reversible causes of sensorineural hearing loss.


Target sites within the inner ear for autoimmune reactions may include its cellular constituents and the vascular supply. A variety of disorders characterized by the presence of vasculitis are known to result in labyrinthine pathology. In addition, animal models have demonstrated the inner ear's ability to mount an immune response, manifested by inflammatory cellular infiltrates.

Clinical Presentation

Organ Specific Autoimmune Inner Ear Disease

Rapidly progressive (over a period of weeks to months), bilateral, asymmetric sensorineural hearing loss represents the classic clinical symptomatology associated with this disorder. Hearing levels may fluctuate during the acute phases of the disease. Vestibular complaints, most commonly vertigo, are present in 25% to 50% of these patients. During its initial presentation, this disorder may be difficult to distinguish from Meniere's disease. Ultimately, the more aggressive course of this disorder, together with the high incidence.

Cogan's Syndrome

Cogan's syndrome represents the classic autoimmune disorder associated with inner ear dysfunction. Patients present with nonsyphylitic interstitial keratitis together with vertigo, hearing loss, and tinnitus. Ocular and labyrinthine pathology may occur simultaneously or within 6 months of each other. Atypical Cogan's syndrome is characterized by the presence.

Several tests have been advocated as being useful in the diagnosis of organ specific autoimmune inner ear disease. Tests of cellular immunity, including the lymphocyte migration inhibition assay and the lymphocyte transformation test, have been advocated by several authors.


Patients in whom a diagnostic evaluation suggests an autoimmune etiology for the progressive inner ear dysfunction are treated initially with prednisone (1 to 2 mg/kg/day) for 1 month. If during taper a recurrence of symptoms occurs, consideration is given to adding a prednisone sparing immunosuppressant such as methotrexate or cyclophosphamide. Cyclophosphamide may be used as a primary agent in patients.


Autoimmune disorders show a significant response to immunosuppressive therapy, but can prove challenging to maintain a state of remission. A multidisciplinary approach combining the expertise of an otolaryngologist with a rheumatologist or immunologist is necessary.

Incidence and Epidemiology

The reported incidence of Meniere's disease varies, partly because of the manner in which different clinicians derive a diagnosis of this disorder, ranging from approximately 10 to 150 cases/100,000 people. Males and females are affected equally, with onset typically occurring during the fifth decade of life, with the first presentation rarely occurring before 20 years of age.