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Sensorineural hearing loss may be due to an autoimmune mechanism. The mechanisms that could induce autoimmune inner ear damage are now better understood, but are not exclusive. Moreover, there is no specific immunologic test available for the diagnosis of autoimmune sensorineural hearing loss, which could also complicate the disease course of other autoimmune systemic diseases. Thus, the incidence of sensorineural autoimmune hearing loss is probably underestimated. The aim of this study was to review the experimental immunologic data in favour of an autoimmune mechanism in this subgroup of sensorineural hearing loss: humoral specific response against inner ear (autoantibodies against a transmembrane transporter) and also cellular response (against cochlin: one of the major proteins expressed in the inner ear). The aim of this review was also to focus on clinical and epidemiological human data that provide evidence for an autoimmune etiopathogeny of some sensorineural hearing loss. Therapeutic options such as immunosuppressive treatments (oral corticosteroids and other immunosuppressive drugs, such as methotrexate and anti-TNFalpha) are also discussed., (2009 Elsevier Masson SAS. All rights reserved.)

Intravenous Immunoglobulin (IVIg) are therapeutic preparations of normal human IgG that are obtained from pools of plasma from healthy blood donors. IVIg can be used at high dose as an immunomodulatory agent in a large number of autoimmune and/or systemic inflammatory diseases, particularly in hematologic or neurologic diseases. Mechanisms of action of IVIg are multiple and intricate. The development of new therapeutic trials in association with analyses of mechanisms of action should help to define new indications of IVIg therapy.