Sensorineural hearing loss of immune-mediated origin may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without other organ involvement (auto-immune inner ear disease). The diagnosis of auto-immune inner ear disease is still predicated on clinical features and to date specific diagnostic tests are not available. We report a case of right-sided sudden hearing loss in a female patient in which the clinical manifestations, in addition to ANA positivity and hypocomplementaemia allowed us to hypothesize an auto-immune inner ear disease. The immunosuppressive treatment with cyclosporine-A was capable of a recovery of the hearing that, however, occurred progressively with normalization of the hearing function after 1 year of treatment. cyclosporine-A could be proposed as a therapeutic option in case of auto-immune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events
