Hypertrophic cranial pachymeningitis associated with middle ear inflammation

Objective: To describe a series of patients with hypertrophic cranial pachymeningitis (HCP) associated with chronic middle ear inflammation. Study Design: Retrospective case review. Setting: University Hospital. Patients: Six consecutive patients between the years 2000 and 2004 who had both middle ear disease and HCP (three men and three women; age range, 36-72 yr, mean age, 58.2 yr). The diagnosis of HCP was made by the use of contrast-enhanced magnetic resonance imaging scans of the brain in all patients. Interventions: Contrast-enhanced magnetic resonance imaging (MRI) of the brain, dural biopsy, and otomicroscopic examination. Main Outcome Measure: Thickening and abnormal enhancement of the dura mater on the MRI of the brain. Results: All of the six patients had chronic inflammatory middle ear diseases. Three patients had otitis media with effusion, two had chronic otitis media, and one had postoperative otorrhea. The most common presentations of HCP were headache and cranial nerve palsies. Cranial Nerves V and VIII were the most frequently involved. On contrast-enhanced MRI examination of the brain, an enhancement of the dura mater was observed on the same side as the middle ear diseases in five of the six patients, whereas bilateral enhancement was observed in one patient. Corticosteroid therapy was effective for alleviating the symptoms in all six patients. Conclusion: Chronic middle ear inflammation might be involved in the pathogenesis of HCP, probably mediated by the venous return.