Cochlear Implantation in Isolated Large Vestibular Aqueduct Syndrome: Report of Three Cases and Literature Review

Abstract

Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes. Resumed Report One girl and two boys with LVAS were assessed and cochlear implantation was performed for each. Various ways of intraoperative management of cerebrospinal fluid gusher and postoperative care and outcomes are reported. Conclusion Cochlear implantation in the deaf children with LVAS is feasible and effective.

Clinical, Audiological and Radiological Features of Bilateral Enlarged Vestibular Aqueducts Syndrome: Case Series

“…Similarly, Rabindra Pradhananga et al reported three cases of children aged 2-4 years who were diagnosed with EVA and subsequently received cochlear implants (CI) to manage their hearing loss. 4,8 In our case report, the three patients experienced a progressive decline in their hearing abilities over a period of 3-7 years. Notably, the second patient demonstrated a significant worsening of hearing loss from a moderate to a severe degree within a span of just 2 years.…”

Cochlear Implantation in Children with Enlarged Vestibular Aqueduct: A Systematic Review of Surgical Implications and Outcomes

“…Of these, 108 were excluded (Fig. 1), and ultimately 55 studies were included for analysis (Dahm et al 1995; Tucci et al 1995; Slattery & Luxford 1995; Munro et al 1996; Aschendorff et al 1997; Hoffman et al 1997; Woolley et al 1998; Au & Gibson 1999; Bent et al 1999; Harker et al 1999; Temple et al 1999; Fahy et al 2001; Miyamoto et al 2002; Daneshi et al 2003; Arnoldner et al 2004; Buchman et al 2004; Mylanus et al 2004; Loundon et al 2005; Papsin 2005; Sennaroglu et al 2006; Wootten et al 2006; Vassoler et al 2008; Lee et al 2010; Dettman et al 2011; Kontorinis et al 2011; Kuthubutheen et al 2012; Lai et al 2012; Broomfield et al 2013; Ko et al 2013; Tzifa & Hanvey 2013; Eftekharian & Amizadeh 2014; Bille et al 2015; Busi et al 2015; Jeong & Kim 2015; Pradhananga et al 2015; Pritchett et al 2015; Bianchin et al 2016; Manzoor et al 2016; Mey et al 2016; van Nierop et al 2016; Bostic et al 2017; Clarós et al 2017; Grover et al 2017; Isaiah et al 2017; Melo et al 2017; Roh et al 2017; Kim et al 2018; Hall et al 2019; Mikkelsen et al 2019; Shi et al 2019; Hura et al 2020; Ozkan et al 2020; Kumari et al 2021; Patterson et al 2021; Skrivan et al 2021). In total, 1572 children with cochlear implants were included, 698 were male and 539 female.…”