Hypothesis/Objective: Investigate the rate of hearing loss progression and incidence of cochlear implant candidacy in children with enlarged vestibular aqueduct (EVA). Background: EVA is the most common congenital malformation of the inner ear, is responsible for a large percentage of children with hearing loss, and is associated with hearing loss progression. Rates and degree of progression of hearing loss to cochlear implantation candidacy have not been well described. Methods: Review of children with EVA who presented to a single academic medical center. Audiometric data were reviewed to determine subjects who met criteria for cochlear implantation (≥75 dB pure-tone average) at presentation. For those not meeting criteria, serial audiometric data were reviewed for progression to candidacy. Results: A total of 257 ears met inclusion criteria. One hundred ninety-two (74.7%) met cochlear implant candidacy criteria by age 12, yet only 117 ears (60.9%) actually received implants before turning 13. One hundred fifty-three ears (59.5%) met implant candidacy criteria at presentation. Nearly 50% of those not initially meeting implantation criteria had a ≥15 dB shift in pure-tone average by age 12, with 37.5% of this subgroup meeting implant candidacy criteria before their teen years at an average age of 7.10 years. Conclusion: The majority of children with EVA reach cochlear implant candidacy before reaching adulthood, yet implantation rates for candidate ears remain at 60% and delay in implantation persist. Parents of children with EVA should be counseled on the likelihood of progression and closely monitored for cochlear implant candidacy.
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