This paper reports on a 55-year-old female who had undergone middle ear surgery 12 years previously and was admitted with a 6-months history of unilateral hearing loss and facial weakness. MRI and CT demonstrated a space-occupying lesion arising from the temporal bone and extending into the posterior fossa. Treatment consisted in complete tumour removal. Temporal and mastoid bone destruction associated with typical histological features led to the diagnosis of neoplasm of endolymphatic sac origin. Clinical, histological, radiological and intra-operative features of these rare tumours are described and discussed. The pertinent literature is reviewed.
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