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We report a case of Wegener's granulomatosis with hypertrophic pachymeningitis. A 36-year-old man presented with bilateral hearing loss and middle ear effusion. CT and exploratory mastoidectomy findings showed a granulomatous lesion with inflammatory cell infiltration in the mastoid cavity. The laboratory result was positive for C-ANCA. On the basis of these findings, the patient was diagnosed as Wegener's granulomatosis. Middle ear symptoms and signs were reduced by treatment with prednisolone and cyclophosphamide. However, severe headache and diplopia developed. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed thickening of the dura mater in the tentorium cerebella caused by hypertrophic pachymeningitis. Further treatment with prednisolone and cyclophosphamide improved these symptoms. The thickening of the dura mater in the tentorium cerebella showed a dramatic improvement in MRI at 5 months after starting treatment.

A Case Report of Wegener's Granulomatosis with Hypertrophic Pachymeningitis.