Predictive Value of GJB2 Mutation Status for Hearing Outcomes of Pediatric Cochlear Implantation
Objective
To systematically review and quantify current evidence regarding the association of GJB2 mutation status with outcomes of pediatric cochlear implantation.
Data Sources
PubMed, Embase, and the Cochrane Library were searched for “GJB2,”“pediatric hearing loss,” and “cochlear implantation” and their synonyms, with no language restrictions, until December 2, 2015.
Review Methods
Studies were included that investigated the status of GJB2 mutation and its predictive value for outcomes of pediatric cochlear implantation. Speech recognition scores, Infant-Toddler Meaningful Auditory Integration Scale, Speech Intelligibility Rating, and Categorized Auditory Performance were pooled using weighted mean differences, and a 95% confidence interval.
Results
Eighteen studies met the inclusion criteria. The differences between GJB2-related deafness and non- GJB2-related deafness due to unidentified causes and other types of genetic deafness without additional disabilities were not statistically significant ( P = .15 and P = .30, respectively); however, the difference between GJB2-related deafness and acquired hearing loss due to environmental etiologies was statistically significant and favored GJB2-related deafness ( P = .03).
Conclusion
GJB2-related deafness leads to significantly better cochlear implantation outcomes when compared with acquired deafness caused by environmental etiologies. However, GJB2 mutation is not associated with a significantly better prognosis when compared with those whose deafness results from either nonsyndromic hearing loss of unknown origin or other types of genetic mutations in the absence of other neurologic deficits.
Top-30
Journals
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Ear and Hearing
3 publications, 12.5%
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International Journal of Pediatric Otorhinolaryngology
2 publications, 8.33%
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Laryngoscope investigative otolaryngology
2 publications, 8.33%
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Zeitschrift fur Arbeits- und Organisationspsychologie
1 publication, 4.17%
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ORL
1 publication, 4.17%
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Otology and Neurotology
1 publication, 4.17%
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Trends in hearing
1 publication, 4.17%
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Journal of Clinical Medicine
1 publication, 4.17%
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Children
1 publication, 4.17%
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Frontiers in Cellular Neuroscience
1 publication, 4.17%
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Genes
1 publication, 4.17%
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Molecular Neurobiology
1 publication, 4.17%
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JARO - Journal of the Association for Research in Otolaryngology
1 publication, 4.17%
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Anatomical Record
1 publication, 4.17%
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PLoS Biology
1 publication, 4.17%
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European Journal of Human Genetics
1 publication, 4.17%
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Expert Review of Molecular Diagnostics
1 publication, 4.17%
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Cochlear Implants International
1 publication, 4.17%
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3
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Publishers
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Ovid Technologies (Wolters Kluwer Health)
4 publications, 16.67%
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MDPI
3 publications, 12.5%
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Springer Nature
3 publications, 12.5%
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Wiley
3 publications, 12.5%
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Elsevier
2 publications, 8.33%
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Taylor & Francis
2 publications, 8.33%
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Hogrefe Publishing Group
1 publication, 4.17%
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S. Karger AG
1 publication, 4.17%
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SAGE
1 publication, 4.17%
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Frontiers Media S.A.
1 publication, 4.17%
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Public Library of Science (PLoS)
1 publication, 4.17%
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Cold Spring Harbor Laboratory
1 publication, 4.17%
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- We do not take into account publications without a DOI.
- Statistics recalculated weekly.