Genetic Causes of Hearing Loss in a Large Cohort of Cochlear Implant Recipients.

Authors :: Seligman KL
Shearer AE
Frees K
Nishimura C
Kolbe D
Dunn C
Hansen MR
Gantz BJ
Smith RJH
Source :: Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery [Otolaryngol Head Neck Surg] 2022 Apr; Vol. 166 (4), pp. 734-737. Date of Electronic Publication: 2021 Jun 22.
Publication Year :: 2022
Abstract: Understanding genetic causes of hearing loss can determine the pattern and course of a patient's hearing loss and may also predict outcomes after cochlear implantation. Our goal in this study was to evaluate genetic causes of hearing loss in a large cohort of adults and children with cochlear implants. We performed comprehensive genetic testing on all patients undergoing cochlear implantation. Of the 459 patients included in the study, 128 (28%) had positive genetic testing. In total, 44 genes were identified as causative. The top 5 genes implicated were GJB2 (20, 16%), TMPRSS3 (13, 10%), SLC26A4 (10, 8%), MYO7A (9, 7%), and MT-RNR1 (7, 5%). Pediatric patients had a higher diagnostic rate. This study lays the groundwork for future studies evaluating the relationship between genetic variation and cochlear implant performance.

Subjects :: Adult
Child
Cohort Studies
Humans
Membrane Proteins genetics
Neoplasm Proteins
Serine Endopeptidases genetics
Cochlear Implantation
Cochlear Implants
Deafness genetics
Hearing Loss genetics
Hearing Loss surgery

Language :: English
ISSN :: 1097-6817
Volume :: 166
Issue :: 4
Database :: MEDLINE
Journal :: Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
Publication Type :: Academic Journal
Accession number :: 34154485
Full Text :: https://doi.org/10.1177/01945998211021308

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