Auditory Characteristics in Children With Enlarged Vestibular Aqueduct.

OBJECTIVE

Enlarged vestibular aqueduct (EVA) is the most common radiographic finding in children diagnosed with congenital sensorineural hearing loss (SNHL). Many institutions use the Cincinnati criteria for diagnosis: width ≥2.0 mm at the operculum and/or ≥1.0 mm at the midpoint. Our goals are to expand our understanding of EVA by examining the audiometric and auditory brainstem response (ABR) characteristics of a large population of children with EVA and hearing loss.

STUDY DESIGN

Retrospective chart review.

SETTING

Tertiary-care children's hospital.

PATIENTS

All children diagnosed with EVA from 2006 to 2016.

INTERVENTIONS

Diagnostic.

MAIN OUTCOME MEASURES

Vestibular aqueduct measurements were taken at the operculum.

RESULTS

One hundred six patients were included (63 females; 60 bilateral EVA). The age of hearing loss diagnosis was significantly younger in patients with bilateral EVA compared with unilateral (0.0 [0-3] yr versus 5.0 [0.9-7.0] yr, p = 0.001). The most common pattern seen on ABR was SNHL-like (57%), followed by large wave I pattern (28.6%), followed by auditory neuropathy spectrum disorder (14.3%). Patients with bilateral EVA were more likely to have progressive hearing loss compared with patients with unilateral EVA ( p = 0.001). There was no correlation between EVA size and hearing stability or between EVA size and pure-tone average at the time of diagnosis.

CONCLUSION

There is a wide range of clinical manifestations of EVA, though we found no significant correlation between size and progressiveness or severity. The clinical significance of a large wave I tracing on ABR is not fully understood and warrants further research.