Endolymphatic sac tumors: experience of three cases

Eur Arch Otorhinolaryngol. 2013 Mar;270(4):1551-7. doi: 10.1007/s00405-012-2298-7. Epub 2012 Dec 5.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Audiometry, Pure-Tone
  • Ear Neoplasms / diagnosis*
  • Ear Neoplasms / pathology
  • Ear Neoplasms / surgery*
  • Endolymphatic Sac / pathology
  • Endolymphatic Sac / surgery*
  • Endoscopy
  • Facial Paralysis / etiology
  • Female
  • Hearing Loss, Mixed Conductive-Sensorineural / etiology
  • Hearing Loss, Sudden / etiology
  • Humans
  • Labyrinth Diseases / diagnosis*
  • Labyrinth Diseases / pathology
  • Labyrinth Diseases / surgery*
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neoplasm Grading
  • Neoplasm Invasiveness / pathology
  • Neoplasm Recurrence, Local / diagnosis
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / surgery
  • Neoplasms, Multiple Primary / diagnosis
  • Neoplasms, Multiple Primary / pathology
  • Neoplasms, Multiple Primary / surgery
  • Reoperation
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Vertigo / etiology
  • Young Adult
  • von Hippel-Lindau Disease / diagnosis
  • von Hippel-Lindau Disease / pathology
  • von Hippel-Lindau Disease / surgery