We described a 67-year-old man who had a right jugular foramen tumour expanding extracranially to the level of C2 cervical body. Paralysis of the glossopharyngeal, vagal, accessory, hypoglossal nerves and sensorineural hearing disturbance were found. The tumour originated from the accessory nerve and the histological examination revealed it was schwannoma. Accessory nerve as an origin of the jugular foramen neurinoma and its pre-operative neurological signs are reviewed.
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