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Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study

Clinical evidence of the effectiveness of cochlear implantation for hearing loss with mitochondrial DNA mutation is limited. Most reports have only described short-term postoperative speech perception, which may not reflect the limitations of cochlear implantation caused by progressive retrocochlear...

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Autores principales: Kanemoto, Kai, Kashio, Akinori, Ogata, Erika, Akamatsu, Yusuke, Koyama, Hajime, Uranaka, Tsukasa, Hoshi, Yujiro, Iwasaki, Shinichi, Yamasoba, Tatsuya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9029697/
https://www.ncbi.nlm.nih.gov/pubmed/35454973
http://dx.doi.org/10.3390/life12040482
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author Kanemoto, Kai
Kashio, Akinori
Ogata, Erika
Akamatsu, Yusuke
Koyama, Hajime
Uranaka, Tsukasa
Hoshi, Yujiro
Iwasaki, Shinichi
Yamasoba, Tatsuya
author_facet Kanemoto, Kai
Kashio, Akinori
Ogata, Erika
Akamatsu, Yusuke
Koyama, Hajime
Uranaka, Tsukasa
Hoshi, Yujiro
Iwasaki, Shinichi
Yamasoba, Tatsuya
author_sort Kanemoto, Kai
collection PubMed
description Clinical evidence of the effectiveness of cochlear implantation for hearing loss with mitochondrial DNA mutation is limited. Most reports have only described short-term postoperative speech perception, which may not reflect the limitations of cochlear implantation caused by progressive retrocochlear dysfunction. The present study aimed to investigate long-term speech perception after cochlear implantation in patients with severe to profound hearing loss associated with mitochondrial DNA mutation. A retrospective chart review was performed on patients with mitochondrial DNA mutation who had undergone cochlear implantation at the Department of Otolaryngology and Head and Neck Surgery at the University of Tokyo Hospital. We extracted data on causative mutations, clinical types, clinical course, perioperative complications, and short-term and long-term postoperative speech perception. Nine patients with mitochondrial DNA mutation underwent cochlear implantation. The mean observation period was 5.5 ± 4.2 years (range, 1–13 years), and seven patients were followed for more than 3 years. Two of the seven patients who initially showed good speech perception exhibited deterioration during long-term follow-up. The absence of an acute progression of cognitive decline in patients, showing a gradual decrease in speech perception, suggests that the deterioration of speech perception was caused by progressive retrocochlear degeneration. Although most patients with mitochondrial DNA mutation maintained good speech perception for more than 3 years after cochlear implantation, retrocochlear degeneration could cause the deterioration of speech perception during long-term follow-up.
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spelling pubmed-90296972022-04-23 Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study Kanemoto, Kai Kashio, Akinori Ogata, Erika Akamatsu, Yusuke Koyama, Hajime Uranaka, Tsukasa Hoshi, Yujiro Iwasaki, Shinichi Yamasoba, Tatsuya Life (Basel) Communication Clinical evidence of the effectiveness of cochlear implantation for hearing loss with mitochondrial DNA mutation is limited. Most reports have only described short-term postoperative speech perception, which may not reflect the limitations of cochlear implantation caused by progressive retrocochlear dysfunction. The present study aimed to investigate long-term speech perception after cochlear implantation in patients with severe to profound hearing loss associated with mitochondrial DNA mutation. A retrospective chart review was performed on patients with mitochondrial DNA mutation who had undergone cochlear implantation at the Department of Otolaryngology and Head and Neck Surgery at the University of Tokyo Hospital. We extracted data on causative mutations, clinical types, clinical course, perioperative complications, and short-term and long-term postoperative speech perception. Nine patients with mitochondrial DNA mutation underwent cochlear implantation. The mean observation period was 5.5 ± 4.2 years (range, 1–13 years), and seven patients were followed for more than 3 years. Two of the seven patients who initially showed good speech perception exhibited deterioration during long-term follow-up. The absence of an acute progression of cognitive decline in patients, showing a gradual decrease in speech perception, suggests that the deterioration of speech perception was caused by progressive retrocochlear degeneration. Although most patients with mitochondrial DNA mutation maintained good speech perception for more than 3 years after cochlear implantation, retrocochlear degeneration could cause the deterioration of speech perception during long-term follow-up. MDPI 2022-03-26 /pmc/articles/PMC9029697/ /pubmed/35454973 http://dx.doi.org/10.3390/life12040482 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Communication
Kanemoto, Kai
Kashio, Akinori
Ogata, Erika
Akamatsu, Yusuke
Koyama, Hajime
Uranaka, Tsukasa
Hoshi, Yujiro
Iwasaki, Shinichi
Yamasoba, Tatsuya
Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title_full Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title_fullStr Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title_full_unstemmed Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title_short Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study
title_sort cochlear implantation in patients with mitochondrial gene mutation: decline in speech perception in retrospective long-term follow-up study
topic Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9029697/
https://www.ncbi.nlm.nih.gov/pubmed/35454973
http://dx.doi.org/10.3390/life12040482
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