Abstract
A 47-year old man presented with a five-year history of fluctuating hearing impairment in the left ear. There was no tinnitus or vertigo. Imaging studies demonstrated a contrast-enhancing cerebellopontine angle mass in the left internal auditory canal. Surgically the lesion was attached to the cochlear nerve. Pathological evaluation revealed what is best described as an angiolipomatous hamartoma of the cochlear nerve. Similar lesions have only rarely been described.