Hostname: page-component-8448b6f56d-wq2xx Total loading time: 0 Render date: 2024-04-24T12:04:41.678Z Has data issue: false hasContentIssue false
  • English
  • Français

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
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

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

References

World Health Organization. Newborn and Infant Hearing Screening: Current Issues and Guiding Principles for Action. Geneva: World Health Organization, 2010Google Scholar
Ghogomu, N, Umansky, A, Lieu, JEC. Epidemiology of unilateral sensorineural hearing loss with universal newborn hearing screening. Laryngoscope 2014;124:295–300CrossRefGoogle ScholarPubMed
Usami, SI, Kitoh, R, Moteki, H, Nishio, SY, Kitano, T, Kobayashi, M et al. Etiology of single-sided deafness and asymmetrical hearing loss. Acta Otolaryngol 2017;137:S2–7CrossRefGoogle ScholarPubMed
Clemmens, CS, Guidi, J, Caroff, A, Cohn, SJ, Brant, JA, Laury, AM et al. Unilateral cochlear nerve deficiency in children. Otolaryngol Head Neck Surg 2013;149:318–25CrossRefGoogle ScholarPubMed
Nakano, A, Arimoto, Y, Matsunaga, T. Cochlear nerve deficiency and associated clinical features in patients with bilateral and unilateral hearing loss. Otol Neurotol 2013;34:554–8CrossRefGoogle ScholarPubMed
Lieu, JEC, Tye-Murray, N, Karzon, RK, Piccirillo, JF. Unilateral hearing loss is associated with worse speech-language scores in children. Pediatrics 2010;125:1348–55CrossRefGoogle ScholarPubMed
D'Alessandro, HD, Sennaroğlu, G, Yücel, E, Belgin, E, Mancini, P. Binaural squelch and head shadow effects in children with unilateral cochlear implants and contralateral hearing aids. Acta Otorhinolaryngol Ital 2015;35:343–9Google Scholar
Purcell, PL, Shinn, JR, Davis, GE, Kathleen, CY, Children, S. Children with unilateral hearing loss may have lower intelligence quotient scores: a meta-analysis. Laryngoscope 2017;126:746–54CrossRefGoogle Scholar
Lieu, JEC. Unilateral hearing loss in children: speech-language and school performance. B-ENT Suppl 2013;21:107–15Google ScholarPubMed
Fischer, C, Lieu, J. Unilateral hearing loss is associated with a negative effect on language scores in adolescents. Int J Pediatr Otorhinolaryngol 2014;78:1611–17CrossRefGoogle ScholarPubMed
Bess, F, Tharpe, A. Unilateral hearing impairment in children. Pediatrics 1984;74:206–16Google ScholarPubMed
Gordon, KA, Wong, DDE, Papsin, BC. Bilateral input protects the cortex from unilaterally-driven reorganization in children who are deaf. Brain 2013;136:1609–25CrossRefGoogle ScholarPubMed
Gilley, PM, Sharma, A, Dorman, MF. Cortical reorganization in children with cochlear implants. Brain Res 2008;1239:56–65CrossRefGoogle ScholarPubMed
Van Zon, A, Peters, JPM, Stegeman, I, Smit, AL, Grolman, W. Cochlear implantation for patients with single-sided deafness or asymmetrical hearing loss: a systematic review of the evidence. Otol Neurotol 2015;36:209–19CrossRefGoogle ScholarPubMed
Peters, JPM, Smit, AL, Stegeman, I, Grolman, W. Review: bone conduction devices and contralateral routing of sound systems in single-sided deafness. Laryngoscope 2015;125:218–26CrossRefGoogle ScholarPubMed
Polonenko, MJ, Gordon, KA, Cushing, SL, Papsin, BC. Cortical organization restored by cochlear implantation in young children with single sided deafness. Sci Rep 2017;7:1–8CrossRefGoogle ScholarPubMed
Thomas, J, Neumann, K, Dazert, S. Cochlear implantation in children with congenital single-sided deafness. Otol Neurotol 2017;38:496–503CrossRefGoogle ScholarPubMed
Birman, CS, Powell, HRF, Gibson, WPR, Elliott, EJ. Cochlear implant outcomes in cochlea nerve aplasia and hypoplasia. Otol Neurotol 2016;37:438–45CrossRefGoogle ScholarPubMed