Acute febrile neutrophilic dermatosis, or Sweet’s syndrome, was first described in 1964 and consists of a triad of erythematous cutaneous plaques infiltrated by neutrophils in association with fever and leukocytosis. Sweet’s syndrome has been reported to be associated with conditions ranging from upper respiratory tract infections to inflammatory bowel disease to rheumatoid arthritis. We report a patient with a 2-year history of Sweet’s syndrome in whom idiopathic progressive bilateral sensory neural hearing loss (IPBSNHL) developed. IPBSNHL is a suspected immunologically mediated hearing loss first described by McCabe in 1979. On the basis of this association, hearing evaluation should be considered in the initial and subsequent examinations of the patient with Sweet’s syndrome. (J Am Acad Dermatol 2000;42:932-5.)