Current Issues With Pediatric Cochlear Implantation

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Abstract

Cochlear implants (CIs) have demonstrated a clear functional benefit in children with severe-to-profound sensorineural hearing loss (SNHL) and thus have gained wide acceptance for treating deafness in the pediatric population. When evaluating young children for cochlear implantation, there are unique considerations beyond the standard issues addressed during surgery in adults. Because of advances in genetic testing, imaging resolution, CI technology, post-implant rehabilitation, and other factors, issues related to CI surgery in children continue to evolve. Such factors have led to changes in candidacy guidelines, vaccine requirements, and lowering of age requirement for surgery. In addition, differences in the anatomy and physiology of infants require special attention to ensure safety when operating on young children. This review summarizes these issues and provides guidance for surgeons treating children with SNHL.

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Most cited references 73

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Severe deafness or hearing impairment is the most prevalent inherited sensory disorder, affecting about 1 in 1,000 children. Most deafness results from peripheral auditory defects that occur as a consequence of either conductive (outer or middle ear) or sensorineuronal (cochlea) abnormalities. Although a number of mutant genes have been identified that are responsible for syndromic (multiple phenotypic disease) deafness such as Waardenburg syndrome and Usher 1B syndrome, little is known about the genetic basis of non-syndromic (single phenotypic disease) deafness. Here we study a pedigree containing cases of autosomal dominant deafness and have identified a mutation in the gene encoding the gap-junction protein connexin 26 (Cx26) that segregates with the profound deafness in the family. Cx26 mutations resulting in premature stop codons were also found in three autosomal recessive non-syndromic sensorineuronal deafness pedigrees, genetically linked to chromosome 13q11-12 (DFNB1), where the Cx26 gene is localized. Immunohistochemical staining of human cochlear cells for Cx26 demonstrated high levels of expression. To our knowledge, this is the first non-syndromic sensorineural autosomal deafness susceptibility gene to be identified, which implicates Cx26 as an important component of the human cochlea.

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Shani Dettman, Richard Dowell, Dawn Choo … (2016)

Examine the influence of age at implant on speech perception, language, and speech production outcomes in a large unselected paediatric cohort.

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Author and article information

Journal

Journal ID (nlm-ta): J Audiol Otol

Journal ID (iso-abbrev): J Audiol Otol

Journal ID (publisher-id): JAO

Title: Journal of Audiology & Otology

Publisher: The Korean Audiological Society and Korean Otological Society

ISSN (Print): 2384-1621

ISSN (Electronic): 2384-1710

Publication date (Print): April 2024

Publication date (Electronic): 10 April 2024

Volume: 28

Issue: 2

Pages: 79-87

Affiliations

[1 ]Department of Otolaryngology-Head & Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA

[2 ]Division of Pediatric Otolaryngology, Children’s Health, Dallas, TX, USA

Author notes

Address for correspondence Kenneth H. Lee, MD, PhD Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, 2001 Inwood Road Dallas, TX 75390-9035, USA Tel +1-972-400-0904 E-mail kenneth.lee@ 123456utsouthwestern.edu

Author information

Donald Tan http://orcid.org/0009-0005-5763-1297

Rance J.T. Fujiwara http://orcid.org/0000-0002-1033-2397

Kenneth H. Lee http://orcid.org/0000-0001-6148-1133

Article

Publisher ID: jao-2024-00073

DOI: 10.7874/jao.2024.00073

PMC ID: 11065545

PubMed ID: 38695052

SO-VID: 25d2dad3-4913-4400-ba12-64e63b2cb7df

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 23 January 2024

Date accepted : 16 March 2024

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