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European Archives of Oto-Rhino-Laryngology
Published in: European Archives of Oto-Rhino-Laryngology 4/2013

01-03-2013 | Case Report

Endolymphatic sac tumors: experience of three cases

Authors: Pierre-Louis Bastier, Erwan de Mones, Magali Marro, Wael Elkhatib, Valérie Franco-Vidal, Dominique Liguoro, Vincent Darrouzet

Published in: European Archives of Oto-Rhino-Laryngology | Issue 4/2013

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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.
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Metadata
Title
Endolymphatic sac tumors: experience of three cases
Authors
Pierre-Louis Bastier
Erwan de Mones
Magali Marro
Wael Elkhatib
Valérie Franco-Vidal
Dominique Liguoro
Vincent Darrouzet
Publication date
01-03-2013
Publisher
Springer-Verlag
Published in
European Archives of Oto-Rhino-Laryngology / Issue 4/2013
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-012-2298-7

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