Wegener's granulomatosis (WG) is characterized by granulomatous inflammation involving the upper and lower respiratory tracts and necrotizing vasculitis affecting small to medium-sized vessels. Here, we report the cases of three patients presenting with intractable otitis media with progressive hearing loss as an initial sign. In two patients, a definite diagnosis of WG was made serologically and histopathologically. Although one patient was negative for any test for WG, he was administered predonisolone and cyclophosphamide as a diagnositic medication. All other patients were treated with satisfactory outcomes including hearing improve-ment. In patients with a limited form of WG in the ear and nose who had been previously reported, it iwas difficult to obtain a definite diagnosis by serological or histlogical tests, and a positeve C (PR-3)-ANCA seemed to be a risk facter of poor hearing recovery. However, the hearing could be improved even in patients with a positive C (PR-3)-ANCA if the immunosuppressive medication is administered as quickly as possible. Therefore, precise diagnosis including the diagnositic medication for WG and quick therapeutic intervention are required to interrupt the spread of necrotizing vasculitis to other organs and to improve hearing
