Abstract
A 62–year–old man suddenly developed right ocular pain and was diagnosed with scleritis. Serum immunological examination revealed elevated levels of myeloperoxidase anti–neutrophil cytoplasmic antibody (ANCA) and C–reactive protein. Thus, the patient was diagnosed with ANCA–related vasculitis. Although administration of prednisolone (PSL ; 30mg/day) resolved his symptoms, serum examination still revealed elevated levels of myeloperoxidase ANCA and C–reactive protein. His condition also deteriorated, including headache, left–side mixed hearing loss, left facial sensory loss, and interstitial pneumoniae. Increasing the PSL dose to 50mg/day resolved his symptoms, and PSL was then tapered stepwise. However, at 30mg/day PSL, the patient complained of deterioration of hearing loss and otalgia, and was diagnosed with bacterial otitis media. Further, after reduction of PSL to 27.5mg/day, the patient showed left–side facial nerve palsy and hoarseness. Administration of valaciclovir did not improve his symptoms, and he was consulted to our neurological service. Neurological examinations revealed otitis media and isolated left–side poly cranial neuropathy, involving cranial nerves V (1st and 2nd branches), VII, VIII, IX, X, and XI. Cranial gadolinium–enhanced T1–weighted magnetic resonance imaging revealed a massive granuloma located at the petrous portion of the carotid artery, cavernous sinus, and mastoid cells. Computed tomography showed left side otitis media with osteoclasia of mastoid cells. He was diagnosed with Garcin syndrome due to otitis media with ANCA–associated vasculitis. After intravenous gamma–globulin and methylprednisolone pulse therapies, his facial sensory disturbance and palsy resolved, and cranial magnetic resonance imaging revealed reduced granuloma size. Overall, neurologist should be aware that Garcin syndrome can be associated with OMAAV.
