Sensorineural hearing loss from suspected Wegener's granulomatosis; report of 2 cases

2 cases of suspected Wegener's granulomatosis showing inflammation in the middle ear and progressive sensorineural hearing loss (SNHL) are reported. The first patient was a 73-year-old female who visited our hospital with a complaint of sudden onset of hearing loss. She had SNHL and administration of steroid hormone improved her hearing acuity temporarily, but then her hearing deteriorated. Subsequently she showed subglottic stenosis, pneumonia and saddle nose. The second patient was a 55-year-old male with otalgia and hearing loss. He had progressive SNHL and ear polyp, and then showed saddle nose. His hearing loss was almost fully recovered after treatment with cyclophosphamide and predonisolone. It was considered in these cases that sensorineural hearing loss had an autoimmune origin. Otitis media is well known as an otological manifestation of Wegener's granulomatosis, but SNHL is comparatively rare.Diagnosis of Wegener's granulomatosis was made by clinical symptoms because c-ANCA titers of our cases were negative, and histopathological studies were not typical. In these cases, they took predonisolone before diagnosis. We suggest that steroid hormones may suppress disease activity and make diagnosis difficult.It is important to look for this disease among SNHL patients and perform general examination in the early stage, because early therapy can lead to an improvement in the morbidity and survival of this disease.