Acute or progressive sensorineural hearing loss, with or without vertigo and tinnitus, has a considerable impact upon an individual's quality of life. Sensorineural hearing disorders are more commonly observed in patients with rheumatic disease than in the general population, are more likely to be accompanied by the presence of serum autoantibodies, and might respond to steroid therapy. A subgroup of these disorders seem to be immune mediated, but audiologic presentation and serologic testing do not enable this subgroup to be further defined. Rheumatologists should be prepared to manage patients with known rheumatic disease who develop acute hearing loss and to evaluate referred patients without known rheumatic illness experiencing progressive or refractory sensorineural hearing loss. In this article, we summarize the literature and outline a rational approach for the evaluation and treatment of such patients.