Enlarged vestibular aqueduct: review of controversial aspects

Laryngoscope. 2011 Sep;121(9):1971-8. doi: 10.1002/lary.22083. Epub 2011 Aug 16.

Abstract

Objectives: To review the controversial aspects of the enlarged vestibular aqueduct syndrome.

Study design: Contemporary review.

Methods: A literature search using the terms "enlarged vestibular aqueduct and large vestibular aqueduct" were used to generate the articles for review in this article.

Results: The enlarged vestibular aqueduct is a condition causing variable auditory and vestibular dysfunction. Although it has been 32 years since Valvasorri and Clemis recognized the clinical importance of the enlarged vestibular aqueduct, many controversial aspects of the diagnosis remain. The topics reviewed in this discussion are as follows: size criteria for radiographic diagnosis, precipitating factors for hearing loss, corticosteroid treatment and sac surgery, conductive component to hearing loss, natural progression of hearing loss, correlations between aqueduct size and hearing loss, genetics, vestibular symptoms, and theories regarding mechanisms behind the symptoms.

Conclusion: The enlarged vestibular aqueduct remains a controversial entity with variable presentation, progression, and prognosis.

Publication types

  • Review

MeSH terms

  • Disease Progression
  • Genotype
  • Hearing Loss / etiology*
  • Hearing Loss / genetics
  • Hearing Loss / physiopathology*
  • Hearing Loss / therapy
  • Humans
  • Membrane Transport Proteins / genetics
  • Radiography
  • Sulfate Transporters
  • Vestibular Aqueduct / diagnostic imaging
  • Vestibular Aqueduct / physiopathology*

Substances

  • Membrane Transport Proteins
  • SLC26A4 protein, human
  • Sulfate Transporters