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Delineating the hearing loss in children with enlarged vestibular aqueduct

Guangwei Zhou1, Quinton Gopen, Margaret A Kenna

  • 1Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts 02115, USA. guangwei.zhou@childrens.harvard.edu

The Laryngoscope
|July 31, 2008
PubMed

Insights

Children with enlarged vestibular aqueduct (EVA) often have air-bone gaps due to inner ear anomalies, not middle ear issues. Proper bone conduction testing is crucial for accurate hearing loss diagnosis in EVA.

Area of Science:

  • Pediatric Otolaryngology
  • Audiology
  • Neurotology

Background:

  • Enlarged vestibular aqueduct (EVA) is a common inner ear malformation in children.
  • EVA is associated with hearing loss, but its precise audiologic characteristics require further elucidation.

Purpose of the Study:

  • To investigate the clinical features and audiologic outcomes in pediatric patients diagnosed with EVA.
  • To identify potential causes of hearing loss in children with EVA.

Main Methods:

  • Retrospective analysis of 54 pediatric cases (82 ears) with confirmed EVA via imaging.
  • Audiologic assessments included behavioral testing, auditory brainstem response (ABR), tympanometry, acoustic reflex, and vestibular evoked myogenic potential (VEMP).

Main Results:

  • Bilateral EVA occurred in 52% of cases; 43% had cochlear malformations.
  • Conductive or mixed hearing loss (HL) was found in 80% of ears, often with missed air-bone gaps due to inadequate bone conduction testing.
  • Normal middle ear function and low-threshold VEMP responses were observed despite air-bone gaps.

Conclusions:

  • Air-bone gaps are common in EVA and may be overlooked without proper bone conduction assessment.
  • The audiologic profile in EVA suggests an inner ear anomaly, potentially akin to the "third" labyrinthine window syndrome.
Abstract

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