%0 Journal Article
%T Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct - clinical challenges, surgical results, and complications.
%A Mey K
%A Bille M
%A Cayé-Thomasen P
%J Acta Otolaryngol
%V 136
%N 10
%D Oct 2016
%M 27241825
%F 1.698
%R 10.1080/00016489.2016.1185538
%X <B>OBJECTIVE: </B>To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA).<BR><B>BACKGROUND: </B>Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications.<BR><B>METHODS: </B>Retrospective review of patients' medical records and CT/MRI.<BR><B>METHODS: </B>Tertiary referral center.<BR><B>METHODS: </B>The medical records and CT/MRI images of 55 PS/NSEVA patients receiving 80 cochlear implantations from 1982-2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved.<BR><B>RESULTS: </B>Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo.<BR><B>CONCLUSIONS: </B>Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.