Abstract
A 74-year-old woman complained of right aural fullness. She was diagnosed as secretory otitis media in her right ear and received a transtympanic ventilation tube insertion accompanied with antibiotic treatment. However, ear discharge through ventilation tube had been persisting and her symptoms were deteriorated. She was referred to our hospital because of mixed conductive-sensorineural hearing loss and dizziness. We suspected of bacterial labyrinthitis and treated with antibiotics and steroids (hydrocortisone). Although ear discharge and dizziness were improved after treatment, she had right facial nerve palsy and sever headache. Gadolinium-enhanced MRI revealed a thickening of dura mater in the right middle cranial fossa. Serum concentration of myeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was elevated. But proteinase 3 (PR3)-ANCA was negative. Histopathological examination of the specimen taken by right exploratory tympanomastoidectomy revealed non-specific inflammatory granulation tissue. Pulmonary and renal involvements were not detected. Thus, we diagnosed her disease as hypertrophic pachymeningitis associated with ANCA-associated vasculitis including a localized type of Wegener's granulomatosis (WG). After the administration of methylprednisolone and cyclophosphamide, her symptoms were sufficiently improved and the serum level of MPO-ANCA became normalized.
The treatment experience demonstrated that we have to make an early diagnosis of ANCA- associated vasculitis including WG and started steroids therapy along with immunosuppressant, even if the results of histopathological examination were not specific and MPO-ANCA was only positive.
