Abstract
A 72-year-old Japanese woman presented to our hospital with progressive hearing loss and dysphagia. Blood tests revealed elevated C-reactive protein and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Contrast-enhanced magnetic resonance imaging of the head showed hypertrophic pachymeningitis of the left middle cranial fossa with compression of the cranial nerves, including the trigeminal (V), facial (VII), glossopharyngeal (IX), and vagal (X) nerves, resulting in cranial nerve palsy. She was diagnosed with Garcin syndrome associated with otitis media with ANCA-associated vasculitis (OMAAV) and treated with high-dose glucocorticoid therapy followed by intravenous cyclophosphamide and rituximab. Therefore, OMAAV should be considered in the differential diagnosis of refractory otitis media with unilateral cranial nerve involvement.