[Endolymphatic sac tumour as an infrequent cause of Ménière's syndrome].

Endolymphatic sac tumours are uncommon. They have been classified as adenocarcinomas with a low degree of malignancy and no metastases have yet been documented. We report on a female patient with Von Hippel-Lindau disease and Ménière's syndrome suffering from an endolymphatic sac tumour. Diagnosis and early treatment are essential to preserve hearing, so long-term monitoring is recommended when this clinical combination is encountered.