The authors present a case report of a 38-year-old female patient admitted at the Department of Otorhinolaryngology and Head and Neck Surgery in Ružomberok with an 18-month history of vertigo, right-sided tinnitus, sensorineural hearing loss and facial nerve paresis. The patient was dia gnosed with an 8 mm temporal bone tumor, located in the area of the lateral posterior semicircular canal and vestibule, which was intraoperatively identified as an extremely rare aggressive low-grade adenocarcinoma of the endolymphatic sac. In the case report, the authors discuss the anatomy, dia gnosis, CT and MRI images, the chosen surgical procedure and the subsequent dispensarization of the patient as well as an overview of the relevant literature. Keywords papillary adenocarcinoma – Heff ner’s tumor – saccus endolymphaticus
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