European Archives of Oto-Rhino-Laryngology

, Volume 270, Issue 4, pp 1551–1557

Endolymphatic sac tumors: experience of three cases

  • Pierre-Louis Bastier
  • Erwan de Mones
  • Magali Marro
  • Wael Elkhatib
  • Valérie Franco-Vidal
  • Dominique Liguoro
  • Vincent Darrouzet
Case Report

DOI: 10.1007/s00405-012-2298-7

Cite this article as:
Bastier, PL., de Mones, E., Marro, M. et al. Eur Arch Otorhinolaryngol (2013) 270: 1551. doi:10.1007/s00405-012-2298-7

Abstract

Objectives

To describe the clinical features, radiological findings, treatment and outcomes of three cases of endolymphatic sac tumors (ELST).

Methods

Retrospective analysis of three cases of ELST.

Results

The first patient had a large ELST invading the labyrinth after a long history of vertigo. He was recurrence-free 1 year after retrolabyrinthine surgical removal. In the second case, an acute peripheral facial nerve paralysis associated with ipsilateral sensorineural hearing loss led to the diagnosis. A translabyrinthine approach was used to remove the tumor, which recurred three times over 10 years. The third patient was a young woman suffering from von Hippel–Lindau (VHL) disease and referred for a sudden sensorineural hearing loss due to an intralabyrinthine hemorrhage secondary to a 2 mm-large endolymphatic sac-confined ELST. Her hearing was totally lost after the deafness recurred 1 month after this first episode. MRI demonstrated a small bilateral ELST. The patient refused surgery on the deaf side.

Conclusion

ELST are difficult to diagnose due to the wide variety of their presentations. Patients with ELST should be screened for VHL disease. Dural invasion and tumor hypervascularization increase the risk of local recurrences after surgery. Early surgical resection may lead to complete tumor removal and inner ear preservation.

Keywords

Endolymphatic sac tumorVon Hippel–Lindau diseaseIntralabyrinthine hemorrhageEndolymphatic hydrops

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Pierre-Louis Bastier
    • 1
    • 3
  • Erwan de Mones
    • 1
  • Magali Marro
    • 1
  • Wael Elkhatib
    • 1
  • Valérie Franco-Vidal
    • 1
  • Dominique Liguoro
    • 2
  • Vincent Darrouzet
    • 1
  1. 1.Otolaryngology and Skull Base Surgery DepartmentUniversity Bordeaux SegalenBordeauxFrance
  2. 2.Department of NeurosurgeryUniversity Bordeaux SegalenBordeauxFrance
  3. 3.Service ORLHôpital PellegrinBordeaux CedexFrance