Abstract
In recent years, association between P-ANCA positive hypertrophic pachymeningitis and Wegener's granulomatosis has been suggested, including several reports showing that intractable otitis media is an initial sign of P-ANCA positive hypertrophic pachymeningitis.
A 69-year-old male was referred from a local clinic to our facility because of bilateral progressive hearing loss and dizziness. The tentative diagnosis of exudative otitis media was made, and the patient was treated with antibiotics, steroid and insertion of a ventilation tube into the tympanic cavity. His ear symptoms became alleviated by the treatment. However, as the steroid tapering, the symptoms exacerbated, accompanied by intense headache. After admission to hospital, he remained resistant to antibiotics. Contrast-enhanced MRI was carried out and revealed hypertrophic pachymeningitis. It seemed probable that inflammation had spread from the middle ear to the dura mater. Mastoidectomy with radical middle ear operation was performed, but headache did not alleviate. The involvement of ANCA-associated disease was suspected, steroid was then re-administered resulting in remarkable alleviation of ear symptoms and headache. Although headache relapsed following steroid tapering, the symptoms subsided in response to combined use of an immunosuppressor.
