Cochlear Implantation in Pediatric Patients With Enlarged Vestibular Aqueduct: A Systematic Review

2021 ◽  
Author(s):  
Liliya Benchetrit ◽  
Nicolette Jabbour ◽  
Swathi Appachi ◽  
Yi‐Chun Liu ◽  
Michael S. Cohen ◽  
...  
2020 ◽  
Vol 134 ◽  
pp. 110065
Author(s):  
William J. Riggs ◽  
Meghan M. Hiss ◽  
Varun V. Varadarajan ◽  
Jameson K. Mattingly ◽  
Oliver F. Adunka

2016 ◽  
Vol 37 (2) ◽  
pp. e96-e103 ◽  
Author(s):  
Nauman F. Manzoor ◽  
Cameron C. Wick ◽  
Marian Wahba ◽  
Amit Gupta ◽  
Robin Piper ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-9 ◽  
Author(s):  
Giovanni Bianchin ◽  
Valeria Polizzi ◽  
Patrizia Formigoni ◽  
Carmela Russo ◽  
Lorenzo Tribi

Objective. To share our experience of cerebrospinal fluid gusher in cochlear implantation in patients with enlarged cochlear or vestibular aqueduct.Study Design. Case series with comparison and a review of the literature.Methods. A retrospective study was performed.Demographic and radiological results of patients with enlarged cochlear aqueduct or enlarged vestibular aqueduct in 278 consecutive cochlear implant recipients, including children and adults, were evaluated between January 2000 and December 2015.Results. Six patients with enlarged cochlear aqueduct and eight patients with enlarged vestibular aqueduct were identified. Cerebrospinal fluid gusher occurs in five subjects with enlarged cochlear aqueduct and in only one case of enlarged vestibular aqueduct.Conclusion. Based on these findings, enlarged cochlear aqueduct may be the best risk predictor of cerebrospinal fluid gusher at cochleostomy during cochlear implant surgery despite enlarged vestibular aqueduct.


2009 ◽  
Vol 124 (1) ◽  
pp. 86-89 ◽  
Author(s):  
M J F de Wolf ◽  
J Honings ◽  
F B M Joosten ◽  
L Hoefsloot ◽  
E A M Mylanus ◽  
...  

AbstractObjective:Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.Design:Case report.Subjects:Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.Results:These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.Conclusion:Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.


2012 ◽  
Vol 126 (4) ◽  
pp. 349-355 ◽  
Author(s):  
R Lai ◽  
P Hu ◽  
F Zhu ◽  
G Zhu ◽  
R Vivero ◽  
...  

AbstractObjective:To review the genotype and cochlear implantation outcome of patients with nonsyndromic hearing loss and enlarged vestibular aqueduct.Methods:Twenty-one Chinese children with nonsyndromic hearing loss and enlarged vestibular aqueduct underwent genetic examination. A DNA microarray was used to screen for the IVS7-2A>G and H723R mutations. Any DNA samples with one or none of the two mutant alleles were sequenced to detect other mutations in the SLC26A4 and FOXI1 genes.Results:Twelve SLC26A4 mutations were detected, including three novel mutations. The most common mutations detected were IVS7-2A>G and H723R. Twelve patients received cochlear implants, and subsequently demonstrated excellent speech perception.Conclusion:Three novel mutations were detected in Chinese patients with nonsyndromic hearing loss and enlarged vestibular aqueduct. The SLC26A4 mutation spectrum in the Chinese population is similar to that in other East Asian populations. Cochlear implantation is a safe and effective treatment in patients with enlarged vestibular aqueduct.


2010 ◽  
Vol 120 (8) ◽  
pp. 1675-1681 ◽  
Author(s):  
Kenneth H. Lee ◽  
James Lee ◽  
Brandon Isaacson ◽  
J. Walter Kutz ◽  
Peter S. Roland

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