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Updated: Jul 15, 2026
11:49
Surgical Induction of Endolymphatic Hydrops by Obliteration of the Endolymphatic Duct
Published on: January 22, 2010
Familial unilateral deafness and delayed endolymphatic hydrops
Kelley M Dodson1, Tamio Kamei, Aristides Sismanis
1Department of Human Genetics, Virginia Commonwealth University, Richmond, Virginia, USA. kelleydodson@comcast.net
American Journal of Medical Genetics. Part A
|May 15, 2007
Summary
Delayed endolymphatic hydrops (DEH) can present with fluctuating ear symptoms after prior deafness. This study suggests genetic factors may contribute to DEH, as familial cases were observed.
Area of Science:
- Otolaryngology
- Genetics
- Neurology
Background:
- Delayed endolymphatic hydrops (DEH) is a rare condition causing fluctuating otologic symptoms in individuals with prior unilateral deafness.
- Symptoms mimic Meniere disease, including aural fullness, hearing fluctuations, and vertigo, occurring ipsilaterally or contralaterally to the deaf ear.
- Previous causes of unilateral deafness in DEH include labyrinthitis, trauma, otosclerosis, and congenital CMV.
Observation:
- This report details two families with possible instances of delayed endolymphatic hydrops.
- Familial occurrence of DEH has not been previously documented in medical literature.
Findings:
- The observed familial cases suggest a potential genetic predisposition in some individuals with DEH.
- This challenges the traditional understanding of DEH etiology, which primarily focuses on acquired causes.
Implications:
- Genetic factors may play a role in the pathogenesis of delayed endolymphatic hydrops.
- Further research into the genetic basis of DEH could lead to new diagnostic and therapeutic strategies.
- Understanding genetic links may help identify at-risk individuals and inform genetic counseling.
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