Children with EVA undergoing cochlear implantation are at greater risk for CSF gushers, but they do well audiometrically and functionally. Laryngoscope, 2010
from The Laryngoscope
Screening of SLC26A4, FOXI1 and KCNJ10 genes in unilateral hearing impairment with ipsilateral enlarged vestibular aqueduct
Together, these data suggest that SLC26A4, FOXI1 and KCNJ10 are not major determinants in unilateral deafness and enlarged vestibular aqueduct compared with their implication in Pendred syndrome and non-syndromic bilateral enlarged vestibular aqueduct.
The objective of this study is to examine the correlation between enlarged vestibular aqueduct (EVA) anomaly and other inner ear anomalies such as cochlear dysplasia, vestibulocochlear dysplasia and modiolar hypoplasia.
Retrospective chart review, with institutional review board approval, of patients with EVA who received treatment at Primary Children’s Medical Center or University Hospital at the University of Utah, between 1997 and 2006. Review of radiographs was done to evaluate for the presence of EVA and other inner ear anomalies.
Twenty patients (40 ears) were included in the study, 17 patients had bilateral EVA and three patients had unilateral EVA. There were 10 females and 10 males. The average age of all patients at the time of initial diagnosis was 1.7 years (0–6 years). Thirty-seven ears were shown to have EVA (92.5%). Of those ears with EVA, 29 (78.4%) had one or more inner ear anomalies. Twenty-three (62.2%) ears had cochlear dysplasia, six (16.2%) had vestibulocochlear dysplasia, and 18 (48.7%) had modiolar hypoplasia.
Using small field of view, thin section CT and/or MR imaging, cochleovestibular abnormalities are commonly identified in association with EVA.
1. Determine whether corticosteroid therapy improves hearing thresholds of patients with enlarged vestibular aqueduct (EVA) anomaly. 2. Determine sample size for a future prospective study.
Retrospective chart review hearing loss in EVA patients comparing patients treated with corticosteroids and untreated patients.
Eighty percent (n = 5) of patients treated with steroids for hearing loss demonstrated audiometric improvement, compared to 14.3% of patients (n = 7) not treated. The pure tone average improved by 17.7 dB in the steroid treated group. A prospective, placebo-controlled trial would need between 19 and 45 patients in each group, treatment versus no-treatment, to achieve statistical significance.
Patients with EVA who develop hearing loss have a high rate of hearing improvement when treated with corticosteroid therapy. The hearing improvement appears to be better than spontaneous recovery.