Median recognition scores obtained by the cochlear implanted groups and the normal-hearing group.

<p>Median recognition scores of (A) phonemes, (B) lexical tones and (C) words obtained by the prelingual group, the postlingual group and the normal-hearing (NH) group in audiovisual (AV) and auditory-only (AO) modes at the 3 presentation levels. The asterisk marks indicate significant difference between AV and AO modes. The horizontal bars indicate significant difference between groups. The vertical error bars represent 95% confidence interval.</p

Demographical background of the prelingually deaf group, the postlingually deaf group and the normal-hearing (NH) group.

<p>IQR: Interquartile range; ImpSide: Side of ear with a implant; OnsetDeaf: Onset of deafness; DuraDeaf: Duration of deafness; AgeImp: Age at implantation; DuraImp: Duration of implant use; SDT: Speech detection threshold; SRT: Speech recognition threshold; ImpPTA: Pure tone average of the implanted ear; NonImpPTA: Pure tone average of the non-implanted or normal-hearing ear; VisionCon: Vision condition.</p><p>Demographical background of the prelingually deaf group, the postlingually deaf group and the normal-hearing (NH) group.</p

Spearman correlation coefficients between deafness-related parameters, test conditions and recognition scores.

<p>OnsetDeaf: Onset of deafness; DuraDeaf: Duration of deafness; AgeImp: Age at implantation; DuraImp: Duration of implant use; n/s: Not significant; n/a: Not applicable.</p><p>*Spearman's correlation coefficients adjusted by sex and age.</p>1<p>Scoring type coded as word  = 1, tone  = 2, phoneme  = 3.</p>2<p>Mode coded as auditory-only  = 1, audiovisual  = 2.</p><p>Only significant correlations are shown.</p><p>Spearman correlation coefficients between deafness-related parameters, test conditions and recognition scores.</p

Mean scores on speech perception tests for the LVAS group and Non-LVAS group.

<p>The mean scores for four speech perception tests obtained one year after implantation and at the most recent follow-up visit by the LVAS group and the non-LVAS group.</p

CAP and SIR scores in subjects broken down by age at implantation (before and after 3.5 years) and genotypes (<i>GJB2</i>, <i>SLC26A4</i> and no mutations).

<p>CI, cochlear implant; Pre-op/post-op, preoperative/postoperative; CAP, Categories of Auditory Performance scale; SIR, Speech Intelligibility Rating scale; Easy sentence, easy-sentence perception test.</p><p>* Compared using the Mann-Whitney <i>U</i> test as a post hoc test; significance was reached when p < α/3 = 0.017 (Bonferroni correction) for multiple comparisons of the three groups (<i>GJB2</i>, <i>SLC26A4</i> and no mutations).</p><p>CAP and SIR scores in subjects broken down by age at implantation (before and after 3.5 years) and genotypes (<i>GJB2</i>, <i>SLC26A4</i> and no mutations).</p

Long-Term Cochlear Implant Outcomes in Children with <i>GJB2</i> and <i>SLC26A4</i> Mutations

<div><p>Objectives</p><p>To investigate speech and language outcomes in children with cochlear implants (CIs) who had mutations in common deafness genes and to compare their performances with those without mutations.</p><p>Study Design</p><p>Prospective study.</p><p>Methods</p><p>Patients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study. All patients underwent mutation screening of three common deafness genes: <i>GJB2</i>, <i>SLC26A4</i> and the mitochondrial 12S rRNA gene. The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale, speech perception tests and language skill tests.</p><p>Results</p><p>Forty-eight patients were found to have confirmative mutations in <i>GJB2</i> or <i>SLC26A4</i>, and 123 without detected mutations were ascertained for comparison. Among children who received CIs before 3.5 years of age, patients with <i>GJB2</i> or <i>SLC26A4</i> mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p = 0.001 for CAP; p = 0.004 for SIR) and year 5 (p = 0.035 for CAP; p = 0.038 for SIR). By contrast, among children who received CIs after age 3.5, no significant differences were noted in post-implant outcomes between patients with and without mutations (all p > 0.05).</p><p>Conclusion</p><p><i>GJB2</i> and <i>SLC26A4</i> mutations are associated with good post-implant outcomes. However, their effects on CI outcomes may be modulated by the age at implantation: the association between mutations and CI outcomes is observed in young recipients who received CIs before age 3.5 years but not in older recipients.</p></div

Number of subjects with mutations in <i>GJB2</i>, <i>SLC26A4</i> and the mitochondrial 12S rRNA gene.

<p>CI, cochlear implantation; AgeHL, age at detection of hearing loss; AgeCI, age at implantation; Mito. 12S rRNA, mitochondrial 12S rRNA gene; n/a, not available.</p><p>Number of subjects with mutations in <i>GJB2</i>, <i>SLC26A4</i> and the mitochondrial 12S rRNA gene.</p

Review of studies on language/speech outcomes in cochlear implanted patients with mutations in <i>GJB2</i>, <i>SLC26A4</i> or the mitochondrial 12S rRNA gene.

<p>Mito. 12S rRNA, mitochondrial 12S rRNA gene; CI, cochlear implant; IOWA Matrix, IOWA Matrix Level B closed-set sentence test; GASP, Glendonald Auditory Screening Procedure; (IT-)MAIS, (Infant-Toddler) Meaningful Auditory Integration Scale; CAP, Categories of Auditory Performance scale; SIR, Speech Intelligibility Rating scale; n/a, not applicable.</p><p>Review of studies on language/speech outcomes in cochlear implanted patients with mutations in <i>GJB2</i>, <i>SLC26A4</i> or the mitochondrial 12S rRNA gene.</p

Demographic information of 171 subjects with and without mutations in <i>GJB2</i>, <i>SLC26A4</i> and the mitochondrial 12S rRNA gene.

<p>Pre-op, preoperative; HA, hearing aid; CI, cochlear implant; HL, hearing loss.</p><p>Demographic information of 171 subjects with and without mutations in <i>GJB2</i>, <i>SLC26A4</i> and the mitochondrial 12S rRNA gene.</p

CAP and SIR scores in all subjects, subjects implanted before the age of 3.5 years and subjects implanted after 3.5 years.

<p>CI, cochlear implant; Pre-op, preoperative; Post-op, postoperative; CAP, Categories of Auditory Performance scale; SIR, Speech Intelligibility Rating scale.</p><p>* Compared with "No mutations" using Mann-Whitney <i>U</i> test.</p><p>CAP and SIR scores in all subjects, subjects implanted before the age of 3.5 years and subjects implanted after 3.5 years.</p