title: Deafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome.
abstract: A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histological appearance, immunocytochemical findings, and the clinical context gave evidence that the tumours had to be reclassified as endolymphatic sac tumours--extremely rare entities. The report supports the hypothesis, suggested by the few earlier case reports, that endolymphatic sac tumours could be one of the inherent tumour manifestations in von Hippel-Lindau syndrome.
authors.affiliation: Albert-Ludwigs- Universität, Universitätsklinik, Freiburg i Br, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Kempermann
authors.forename: H P
authors.initials: HP
authors.lastname: Neumann
authors.forename: R
authors.initials: R
authors.lastname: Scheremet
authors.forename: B
authors.initials: B
authors.lastname: Volk
authors.forename: W
authors.initials: W
authors.lastname: Mann
authors.forename: J
authors.initials: J
authors.lastname: Gilsbach
authors.forename: R
authors.initials: R
authors.lastname: Laszig
mesh_terms: D002277:Carcinoma / Q000473:pathology; D002528:Cerebellar Neoplasms / Q000556:secondary / Q000601:surgery; D002530:Cerebellopontine Angle / Q000473:pathology / Q000601:surgery; D003638:Deafness / Q000175:diagnosis / Q000209:etiology; D004428:Ear Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D004712:Endolymphatic Sac / Q000473:pathology*; D007839:Functional Laterality; D006801:Humans; D009362:Neoplasm Metastasis; D013961:Thyroid Gland / Q000473:pathology; D013964:Thyroid Neoplasms / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis / Q000473:pathology