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Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?

Published online by Cambridge University Press:  08 March 2017

J H Spiegel  and
A K Lalwani
J H Spiegel
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Boston University School of Medicine, Massachusetts, USA
A K Lalwani*
Affiliation:
Department of Otolaryngology, New York University, USA
*
Address for correspondence: Professor Anil K Lalwani, Mendik Foundation Professor and Chair, Department of Otolaryngology, New York University, 550 First Avenue, NBV 5 East 5, New York, NY 10016, USA. Fax: +1 212 263 8257 E-mail: [email protected]
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Abstract

Objective:

To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis.

Method:

Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops.

Results:

We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic Ménière's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis.

Conclusion:

To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.

Keywords

Meniere's DiseaseInner EarVertigoVestibular Aqueduct
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 8 , August 2009 , pp. 919 - 921
Copyright
Copyright © JLO (1984) Limited 2008

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