TY - JOUR
T1 - Enlarged vestibular aqueduct
T2 - Hearing progression and cochlear implant candidacy in pediatric patients
AU - Hodge, Sarah E.
AU - Thompson, Nicholas J.
AU - Park, Lisa R.
AU - Brown, Kevin D.
N1 - Funding Information:
Address correspondence and reprint requests to Kevin D. Brown, M.D., Ph.D., Associate Professor, Vice Chair of Outpatient Services, Chief of Division of Otology/Neurotology, Skull Base Surgery, Medical Director of Children’s Cochlear Implant Center at UNC, 170 Manning Drive, CB#7070, Houpt Building, Rm G190A, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7070; E-mail: kevin_d_brown@med.unc.edu L.R.P. is supported by a research grant from MED-EL; K.D.B. serves on the Surgical Advisory Board for MED-EL.
Funding Information:
The project described was supported by the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant Award Number UL1TR002489. NCTraCs assistance was utilized for data acquisition only. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.
Publisher Copyright:
© 2020, Otology & Neurotology, Inc.
PY - 2021/1
Y1 - 2021/1
N2 - 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.
AB - 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.
KW - Cochlear implant
KW - EVA
KW - Enlarged vestibular aqueduct
KW - Inner ear malformation
KW - Pediatric hearing loss
KW - Progressive hearing loss
KW - Sensorineural hearing loss
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U2 - 10.1097/MAO.0000000000003034
DO - 10.1097/MAO.0000000000003034
M3 - Article
C2 - 33885268
AN - SCOPUS:85105695459
SN - 1531-7129
VL - 42
SP - 203
EP - 206
JO - American Journal of Otology
JF - American Journal of Otology
IS - 1
ER -