Large vestibular aqueduct syndrome

Large vestibular aqueduct syndrome (LVAS) refers to the presence of congenital sensorineural hearing loss with an enlarged vestibular aqueduct. It is thought to be one of the most common congenital causes of sensorineural hearing loss (SNHL). 

SNHL starts in childhood and is progressive 4. Often SNHL is sudden onset, and occurs after minor head trauma 6.

Associations

Associated anomalies are the norm, occurring in ~85% of patients 6:

CT/MRI

A measurement of the vestibular aqueduct of 1.5 mm is considered the upper limit of normal 3-4. This measurement is ideally made half way between the crus and the aperture on an axial view (i.e. AP dimension). Some studies suggest consideration of upper limit measurements of the midpoint of the aqueduct and operculum being at 1 mm and 2 mm, respectively 5.  

The bony modiolus is not visible in up to 90% of cases.


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Article Information

rID: 8567
Section: Syndromes
Synonyms or Alternate Spellings:
  • Large vestibular aqueduct syndrome
  • Large vestibular aquaduct syndrome (LVAS)
  • Enlarged endolymphatic duct syndrome (EEDS)
  • Enlarged endolymphatic sac syndrome (EESS)
  • Large vestibular aquaeduct syndrome
  • Enlarged vestibular aqueduct syndrome
  • Enlarged endolymphatic duct syndrome
  • Enlarged endolymphatic sac syndrome
  • EVAS
  • LVAS
  • EEDS
  • EESS

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    Case 4: bilateral
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    Case 6: bilateral
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