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


METHODS
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.


CONCLUSIONS
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.
Spiegel
J H
JH
Department of Otolaryngology - Head and Neck Surgery, Boston University School of Medicine, Massachusetts, USA.
Lalwani
Anil K
AK
D000328:Adult; D018159:Endolymphatic Hydrops / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008575:Meniere Disease / Q000209:etiology / Q000503:physiopathology; D035781:Siblings; D013577:Syndrome; D014723:Vestibular Aqueduct / Q000503:physiopathology*