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Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

Published online by Cambridge University Press:  07 December 2020

K Pollaers ,
A Thompson  and
J Kuthubutheen
K Pollaers*
Affiliation:
Department of ENT, Perth Children's Hospital, Nedlands, Australia
A Thompson
Affiliation:
Department of Medical Imaging, Perth Children's Hospital, Nedlands, Australia
J Kuthubutheen
Affiliation:
Department of ENT, Perth Children's Hospital, Nedlands, Australia Division of Surgery, Faculty of Health and Medical Sciences, University of Western Australia, Crawley, Australia
*
Author for correspondence: Dr Katherine Pollaers, ENT Department, Perth Children's Hospital, 15 Hospital Ave, NedlandsWA6009, Australia E-mail: Katherine.pollaers@health.wa.gov.au Fax: +61 (0)8 6457 4899
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Abstract

Objective

To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss.

Methods

A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia.

Results

Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases).

Conclusion

Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

Keywords

Hearing Loss, SensorineuralPediatricsMagnetic Resonance ImagingCochlear Nerve Disorders
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 134 , Issue 11 , November 2020 , pp. 1014 - 1017
Copyright
Copyright © The Author(s), 2020. Published by Cambridge University Press

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Footnotes

Dr K Pollaers takes responsibility for the integrity of the content of the paper

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