Sensory hearing loss may be associated with a wide array of systemic autoimmune disorders, such as systemic lupus erythematosus, granulomatosis with polyangiitis (formerly Wegener granulomatosis), and Cogan syndrome (hearing loss, keratitis, aortitis) The loss is most often bilateral and progressive The hearing level often fluctuates, with periods of deterioration alternating with partial or even complete remission Usually, there is the gradual evolution of permanent hearing loss, which often stabilizes with some remaining auditory function but occasionally proceeds to complete deafness Vestibular dysfunction, particularly dysequilibrium and postural instability, may accompany the auditory symptoms A syndrome resembling Ménière disease may also occur with intermittent attacks of severe vertigo In many cases, the autoimmune pattern of audioves- tibular dysfunction presents in the absence of recognized systemic autoimmune disease Responsiveness to oral corticosteroid treatment is helpful in making the diagnosis and constitutes first-line therapy If stabilization of hearing becomes dependent on long-term corticosteroid use, steroid-sparing immunosuppressive regimens may become necessary