Early diagnosis of otologic Wegener's granulomatosis using the serologic marker C-ANCA

Abstract

Wegener's granulomatosis is a systemic vasculitis that may involve any organ system. Otologic manifestations are common, and can be the presenting complaint. In the past, diagnosis often necessitated the development of characteristic pulmonary or renal disease. The identification of a new serologic marker, cytoplasmic pattern antineutrophil cytoplasmic autoantibody (C-ANCA), allows for the early diagnosis of Wegener's granulomatosis and gives the patient the best chance for remission with cytotoxic therapy. We report two patients with Wegener's granulomatosis who presented with refractory otitis media, one of whom subsequently developed facial nerve paralysis, in which an early diagnosis was facilitated by the use of the C-ANCA test. Otologic manifestations of Wegener's granulomatosis and the basis of the C-ANCA test are discussed.