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...

Full description

Bibliographic Details
Main Authors: Camron Davies, Jenna Bergman, Carly Misztal, Renuka Ramchandran, Jeenu Mittal, Erdogan Bulut, Viraj Shah, Rahul Mittal, Adrien A. Eshraghi
Format: Article
Language:English
Published: MDPI AG 2021-06-01
Series:Journal of Clinical Medicine
Subjects:
Online Access:https://www.mdpi.com/2077-0383/10/13/2915
id doaj-952d84cde3a5496a80532b5007dad1f6
record_format Article
spelling 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
work_keys_str_mv AT camrondavies theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT jennabergman theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT carlymisztal theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT renukaramchandran theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT jeenumittal theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT erdoganbulut theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT virajshah theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT rahulmittal theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT adrienaeshraghi theoutcomesofcochlearimplantationinushersyndromeasystematicreview
AT camrondavies outcomesofcochlearimplantationinushersyndromeasystematicreview
AT jennabergman outcomesofcochlearimplantationinushersyndromeasystematicreview
AT carlymisztal outcomesofcochlearimplantationinushersyndromeasystematicreview
AT renukaramchandran outcomesofcochlearimplantationinushersyndromeasystematicreview
AT jeenumittal outcomesofcochlearimplantationinushersyndromeasystematicreview
AT erdoganbulut outcomesofcochlearimplantationinushersyndromeasystematicreview
AT virajshah outcomesofcochlearimplantationinushersyndromeasystematicreview
AT rahulmittal outcomesofcochlearimplantationinushersyndromeasystematicreview
AT adrienaeshraghi outcomesofcochlearimplantationinushersyndromeasystematicreview
_version_ 1721299120738533376