Otologic Wegener's granulomatosis with facial nerve palsy

Wegener's granulomatosis, characterized by necrotizing granulomas and vasculitis of the respiratory tract and kidney, frequently first presents with otologic symptoms. We report a case of primary otologic Wegener's granulomatosis in a patient who presented with symptoms of acute otomastoiditis and associated facial nerve palsy. The patient subsequently developed neuropathies of various cranial nerves. The patient underwent urgent mastoidectomy with facial nerve decompression. Nonspecific inflammatory disease of the mastoid mucosa delayed the correct diagnosis of Wegener's granulomatosis, which was confirmed by an elevated level of cytoplasmic-pattern antineutrophil cytoplasmic antibody (cANCA). We contrast the specificity of middle ear mucosal disease and cANCA serum levels in the diagnosis of Wegener's granulomatosis.