Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness

Abstract

Background:Cochlear implants (CI)fortherehabilitation of patients withprofound ortotalbilateral sensorineural hypoacusis represent theinitial useof electrical fields to provide audibility in cases where the use of sound amplifiers does not provide satisfactory results. Aims:Tocompare speech perception performance aftercochlear im-plantation in children withconnexin 26-associated deafness withthat of a control group of children with deafness of unknown etiology.Study Design: Retrospective comparative study.Methods:During theperiod from 2006 to, cochlear implantation wasperformed on26children. Eighteen ofthese children hadun-dergone genetic testsformutation oftheGapJunction Protein Beta 2 (GJB2) gene. Bi-allelic GJB2 mutations were confirmed in7 out of18examined children. Inorder toconfirm whether genetic fac-torshaveinfluence onspeech perception aftercochlear implantation, wecompared thepost-implantation speech performance ofseven children withmutations oftheGBJ2 (connexin 26)genewithseven other children whohadthewildtypeversion ofthisparticular gene. Thelatter were carefully matched according totheageat cochlear implantation. Speech perception performance wasmeasured before cochlear implantation, andoneandtwoyears afterimplantation. All thepatients werearranged in linewiththeappropriate speech percep-tioncategory (SPC). Non-parametric tests, Friedman ANOVA and Mann-Whitney's U test were used for statistical analysis. Results:Both groups showed similar improvements inspeech per-ception scores aftercochlear implantation. Statistical analysis didnot confirm significant differences between thegroups 12and24months after cochlear implantation.Conclusion:Theresults obtained in thisstudy showed anabsence of apparent distinctions in thescores ofspeech perception between the twoexamined groups andtherefore might havesignificant implica-tions in selecting prognostic indicators ofspeech perception follow-ing cochlear implantation.