The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review
Objective: To systematically appraise the implementation of cochlear implantation (CI) in Usher Syndrome (USH) Types 1, 2, and 3 patients, and analyze who would benefit from CI. Data Sources: A comprehensive search of PubMed, Embase, CINAHL, and Cochrane Library electronic databases from inception t...
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doaj-952d84cde3a5496a80532b5007dad1f62021-07-15T15:39:22ZengMDPI AGJournal of Clinical Medicine2077-03832021-06-01102915291510.3390/jcm10132915The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic ReviewCamron Davies0Jenna Bergman1Carly Misztal2Renuka Ramchandran3Jeenu Mittal4Erdogan Bulut5Viraj Shah6Rahul Mittal7Adrien A. Eshraghi8Cochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USACochlear Implant and Hearing Research Laboratory, Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, FL 33136, USAObjective: To systematically appraise the implementation of cochlear implantation (CI) in Usher Syndrome (USH) Types 1, 2, and 3 patients, and analyze who would benefit from CI. Data Sources: A comprehensive search of PubMed, Embase, CINAHL, and Cochrane Library electronic databases from inception through June 2020 was performed. There were no language restrictions. Study Selection: The PRISMA strategy was followed. Included studies discuss USH patients who underwent CI regardless of age, nationality, or clinical subtype. All included studies report post-implantation functional, cognitive, or quality of life outcomes. Only reviews were excluded. Results: Fifteen studies met the inclusion criteria. USH patients experienced improvements in PTA and speech perception and expression outcomes after CI, as well as improvements in phonological memory and quality of life measures. Overall, patients implanted at younger ages outperformed older patients in audiological testing. Similarly, patients with prolonged auditory deprivation had relatively poor performance outcomes in sentence recognition and speech detection following CI. Conclusions: Most USH patients benefit from CI. USH patients who undergo CI at younger ages generally achieve better hearing, speech, and cognitive outcomes. CI at older ages can still prove beneficial if appropriate auditory amplification is started at the right time. Further research is warranted to fill the gap in understanding regarding the gene mutations underlying the pathophysiology of USH that have favorable CI outcomes as well as the optimal time to perform CI.https://www.mdpi.com/2077-0383/10/13/2915Usher Syndromecochlear implantsystematic reviewspeech perceptionaudiologic outcomes |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Camron Davies Jenna Bergman Carly Misztal Renuka Ramchandran Jeenu Mittal Erdogan Bulut Viraj Shah Rahul Mittal Adrien A. Eshraghi |
spellingShingle |
Camron Davies Jenna Bergman Carly Misztal Renuka Ramchandran Jeenu Mittal Erdogan Bulut Viraj Shah Rahul Mittal Adrien A. Eshraghi The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review Journal of Clinical Medicine Usher Syndrome cochlear implant systematic review speech perception audiologic outcomes |
author_facet |
Camron Davies Jenna Bergman Carly Misztal Renuka Ramchandran Jeenu Mittal Erdogan Bulut Viraj Shah Rahul Mittal Adrien A. Eshraghi |
author_sort |
Camron Davies |
title |
The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review |
title_short |
The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review |
title_full |
The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review |
title_fullStr |
The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review |
title_full_unstemmed |
The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review |
title_sort |
outcomes of cochlear implantation in usher syndrome: a systematic review |
publisher |
MDPI AG |
series |
Journal of Clinical Medicine |
issn |
2077-0383 |
publishDate |
2021-06-01 |
description |
Objective: To systematically appraise the implementation of cochlear implantation (CI) in Usher Syndrome (USH) Types 1, 2, and 3 patients, and analyze who would benefit from CI. Data Sources: A comprehensive search of PubMed, Embase, CINAHL, and Cochrane Library electronic databases from inception through June 2020 was performed. There were no language restrictions. Study Selection: The PRISMA strategy was followed. Included studies discuss USH patients who underwent CI regardless of age, nationality, or clinical subtype. All included studies report post-implantation functional, cognitive, or quality of life outcomes. Only reviews were excluded. Results: Fifteen studies met the inclusion criteria. USH patients experienced improvements in PTA and speech perception and expression outcomes after CI, as well as improvements in phonological memory and quality of life measures. Overall, patients implanted at younger ages outperformed older patients in audiological testing. Similarly, patients with prolonged auditory deprivation had relatively poor performance outcomes in sentence recognition and speech detection following CI. Conclusions: Most USH patients benefit from CI. USH patients who undergo CI at younger ages generally achieve better hearing, speech, and cognitive outcomes. CI at older ages can still prove beneficial if appropriate auditory amplification is started at the right time. Further research is warranted to fill the gap in understanding regarding the gene mutations underlying the pathophysiology of USH that have favorable CI outcomes as well as the optimal time to perform CI. |
topic |
Usher Syndrome cochlear implant systematic review speech perception audiologic outcomes |
url |
https://www.mdpi.com/2077-0383/10/13/2915 |
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