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Review
. 2021 Jun 29;10(13):2915.
doi: 10.3390/jcm10132915.

The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review

Camron Davies  1 , Jenna Bergman  1 , Carly Misztal  1 , Renuka Ramchandran  1 , Jeenu Mittal  1 , Erdogan Bulut  1 , Viraj Shah  1 , Rahul Mittal  1 , Adrien A Eshraghi  1   2   3   4
Affiliations
Review

The Outcomes of Cochlear Implantation in Usher Syndrome: A Systematic Review

Camron Davies et al. J Clin Med. .

Abstract

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.

Keywords: Usher Syndrome; audiologic outcomes; cochlear implant; speech perception; systematic review.

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Conflict of interest statement

A.A.E. is a consultant and received research funding from MED-EL corporation. Other authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Figures

Figure 1
Figure 1
The PRISMA flowchart diagram for literature searching and screening.
See this image and copyright information in PMC

References

    1. Jatana K.R., Thomas D., Weber L., Mets M.B., Silverman J.B., Young N.M. Usher syndrome: Characteristics and outcomes of pediatric cochlear implant recipients. Otol. Neurotol. 2013;34:484–489. doi: 10.1097/MAO.0b013e3182877ef2. - DOI - PubMed
    1. Mathur P.D., Yang J. Usher syndrome and non-syndromic deafness: Functions of different whirlin isoforms in the cochlea, vestibular organs, and retina. Hear. Res. 2019;375:14–24. doi: 10.1016/j.heares.2019.02.007. - DOI - PMC - PubMed
    1. Moller C.G., Kimberling W.J., Davenport S.L., Priluck I., White V., Biscone-Halterman K., Odkvist L.M., Brookhouser P.E., Lund G., Grissom T.J. Usher syndrome: An otoneurologic study. Laryngoscope. 1989;99:73–79. doi: 10.1288/00005537-198901000-00014. - DOI - PubMed
    1. Smith R.J., Berlin C.I., Hejtmancik J.F., Keats B.J., Kimberling W.J., Lewis R.A., Moller C.G., Pelias M.Z., Tranebjaerg L. Clinical diagnosis of the Usher syndromes. Usher Syndrome Consortium. Am. J. Med. Genet. 1994;50:32–38. doi: 10.1002/ajmg.1320500107. - DOI - PubMed
    1. Yan D., Liu X.Z. Genetics and pathological mechanisms of Usher syndrome. J. Hum. Genet. 2010;55:327–335. doi: 10.1038/jhg.2010.29. - DOI - PMC - PubMed

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