Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges, surgical results, and complications

Abstract

Objective: 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).

Background: 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.

Study design: Retrospective review of patients’ medical records and CT/MRI.

Setting: Tertiary referral center.

Materials and methods: 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.

Results: 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.

Conclusion: 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.

Keywords:

• Cochlear implantation
• enlarged vestibular aqueduct
• gusher
• malformations
• Pendred syndrome
• surgical complications

Acknowledgements

The authors wish to thank radiologist Ali Muhamad for his revision and descriptions of CT/MRI-scans, in order to comply with the new classifications in the primary diagnostics of PS/NSEVA, and genetics professor Lisbeth Tranebjærg for her assistance in identifying patients with SLC26A4 mutations, and professor emeritus (of linguistics) Jacob L. Mey for his support in revising this manuscript with regards to the English language.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Funding information

KM has received a PhD grant from the Oticon foundation.