Otologic Symptoms as Initial Manifestation of Wegener Granulomatosis: Diagnostic Dilemma

Objective: To show 7 cases of Wegener granulomatosis (WG) with early aural symptoms and to discuss the problems of otologic manifestation in WG. Study Design: Retrospective case review. Setting: Tertiary care university hospital. Patients: All patients were administered to the ENT University Department in Poznan in years 2002-2008 because of otitis media with effusion, facial palsy, sensorineural profound hypoacusis, hypoacusis combined with purulent discharge, and facial nerve palsy or progression of mixed type hypoacusis. Interventions: Diagnostics and treatment. Main Outcome Measures: Otologic symptoms as initial manifestation of WG diagnostic dilemma. Results: The authors want to underline the young age of the patients, ranging from 32 to 46 years. The outcome of initially otologic cases, which developed generalized form of WG, was poor (the first patient died after 2 months, the second patient died after 7 days, the third had the pulmonary insufficiency in 2 months of observation, and the fourth had severe renal failure in 1 month), whereas the patients with localized disease have been successfully under control for 1 to 5 years. Conclusion: As WG often presents otologic symptoms, as an initial sign in some cases, it is important to take WG into consideration in atypical inflammatory states of the ear. The otologic onset of WG is very insidious, and prompt diagnosis in early stage of disease is a challenge. Focal and localized disease in the aural region might possibly require less aggressive therapy than acute-onset multi-organ disease and is connected with better prognosis.