Otological symptoms as initial manifestations of Wegener's granulomatosis have been observed in 3 patients. In one of them, symptoms consisted of a bilateral sensorineural hearing loss, improved by corticoid therapy. No other organ system was involved and laboratory tests remained within normal limits for 2 years after the onset of otological signs. In the other 2 patients, Wegener's granulomatosis manifested mainly as serous otitis media. Otologic involvement underscores the role of the otolaryngologist in the early diagnosis and treatment of this disease.