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Because our recent clinical experiences suggested a possible association between hearing loss and systemic lupus erythematosus (SLE), we prospectively studied the hearing of 30 patients hospitalized because of exacerbation of SLE. Twenty-nine of the 30 patients were receiving immunosuppressive therapy at the time of testing. We found an 8% incidence of substantial, previously undetected hearing loss without attributable cause. Hearing loss could not be correlated to age, sex, disease activity, organ-system involvement, laboratory test abnormalities, or duration of symptoms of SLE. Otolaryngologists treating patients with unexplainable hearing loss--particularly if it is sudden, fluctuating, or rapidly progressive--are alerted to the possibility of underlying systemic autoimmunity. We advise physicians who frequently treat patients with SLE to include questions on hearing in the review of systems and to refer the patient with hearing complaints for thorough otologic evaluation.

Susceptibility to many autoimmune diseases is associated with inheritance of certain Human Leukocytic Antigens (HLA), determinants which have become diagnostic or prognostic markers. In this study of HLA antigen frequencies among patients with autoimmune sensorineural hearing loss, 39 patients with this otologic disorder were typed for HLA-A, B, C, and DR antigens. A significant increase of Cw7 occurred in 51% of patients compared to 21% of 627 matched controls (relative risk, 3.95). Trends towards increased frequencies of Cw4, B35 and reduced frequency of DR4 were also observed. These data suggest a possible immunogenetic predisposition to this otologic disorder. The presence of Cw7 may facilitate the diagnosis of autoimmune sensorineural hearing loss.