Introduction: Characterized by sensorineural hearing loss, with fast progress, the autoimmune disease of the inner ear can involve symptoms such as disequilibrium, vertigo, tinnitus, and ear fullness. In 15 to 30% of the patients, there is a concomitant systemic autoimmune disease. The physical examination is mostly normal and laboratory tests may help screening for systemic immunologic dysfunction. The treatment consists in immunosuppressive therapy and, recently, intratympanic therapy has been used.