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Immune-mediated inner ear disease may occur as an organ-specific pathology - autoimmune inner ear disease - or in the context of a systemic autoimmune disease. This is because the inner ear can create a local immune response with vestibulocochlear damage that may occur due to autoantibody damage, immune complex deposition or vasculitis with microcirculation ischemia. The etiology of autoimmune inner ear disease is not yet clearly defined but the damage is presumed to be due to autoantibodies directed to inner ear proteins, the most studied being the anti-68 kDa, anti-Hsp70 and anti-cochlin proteins. The challenge in diagnosing this type of pathology arises not only because of the absence of specific analytical markers, but also because of the wide variety of systemic pathologies that may manifest with audiovestibular symptoms. However, early treatment is preponderant since hearing loss may be reversible.