Sensorineural hearing loss as the first manifestation of Sjögren's syndrome.

introduction Dysfunction of inner ear, vestibulocochlear nerve or central brain processing centers leads to sensorineural hearing loss (SNHL). Autoimmune inner ear disease (AIED) is a rare but potentially treatable cause of hearing loss, characterized by progressive evolution over weeks to months.1 In 1958, an antigen-antibody reaction was described in 13 patients with progressive bilateral SNHL,2 and later, in 1979, the term autoimmune SNHL was proposed after an idiopathic bilateral progressive SNHL had markedly improved with steroids therapy.3 Since there are no defined diagnostic criteria for AIED, it remains a diagnosis of exclusion, supported by clinical suspicion and responsiveness to corticotherapy. About one third of the cases of AIED is secondary to a systemic autoimmune disease and may, rarely, be the first manifestation of the latter.4,5