Although the pathogenesis of sudden sensorineural hearing loss (SNHL) in patients with systemic lupus erythematosus (SLE) is not clear, several reports suggest an association with the antiphospholipid antibody (aPL). We describe 6 patients with SLE or a lupus-like syndrome, who had sudden SNHL and had elevated levels of anticardiolipin antibodies (aCL) or the lupus anticoagulant. In a literature search, of 5 additional reported cases, 2 were not tested for aPL; the remaining 3 had elevated aCL levels. Thus, acute SNHL in patients with SLE who have aPL may be a manifestation of the antiphospholipid syndrome. We recommend anticoagulation treatment of these patients.