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Auditory Characteristics in Children With Enlarged Vestibular Aqueduct
Punam Patel1, William Parkes2, Cedric Pritchett3
1Department of Otolaryngology, Phoenix Children's Hospital, Phoenix, Arizona.
Summary
Enlarged vestibular aqueduct (EVA) is common in children with hearing loss. This study found no correlation between EVA size and hearing loss severity or progression, but bilateral EVA was linked to earlier diagnosis and progressive loss.
Area of Science:
- Audiology
- Pediatric Otolaryngology
- Radiology
Background:
- Enlarged vestibular aqueduct (EVA) is the most frequent radiographic finding in pediatric congenital sensorineural hearing loss (SNHL).
- The Cincinnati criteria (width ≥2.0 mm at operculum and/or ≥1.0 mm at midpoint) are commonly used for EVA diagnosis.
- Understanding the audiometric and auditory brainstem response (ABR) characteristics in children with EVA is crucial.
Purpose of the Study:
- To examine the audiometric and ABR characteristics of a large pediatric population with EVA and hearing loss.
- To expand the understanding of the clinical manifestations associated with enlarged vestibular aqueduct.
- To investigate potential correlations between EVA size and hearing loss patterns.
Main Methods:
- Retrospective chart review of children diagnosed with EVA between 2006 and 2016 at a tertiary-care children's hospital.
- Analysis of vestibular aqueduct measurements at the operculum.
- Evaluation of audiometric data and auditory brainstem response (ABR) patterns.
Main Results:
- 106 children were included; 60 had bilateral EVA.
- Children with bilateral EVA were diagnosed with hearing loss significantly younger than those with unilateral EVA (0.0 vs. 5.0 years).
- The most common ABR patterns were SNHL-like (57%), large wave I (28.6%), and auditory neuropathy spectrum disorder (14.3%). Bilateral EVA was associated with a higher likelihood of progressive hearing loss.
- No correlation was found between EVA size and hearing stability or pure-tone average at diagnosis.
Conclusions:
- Enlarged vestibular aqueduct presents with diverse clinical manifestations, but size did not correlate with hearing loss progressiveness or severity in this cohort.
- The clinical significance of a large wave I tracing on ABR in EVA patients requires further investigation.
- Bilateral EVA is associated with earlier diagnosis and a greater likelihood of progressive hearing loss.
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