Connell Sarah S, Angeli Simon I, Suarez Hamlet, Hodges Annelle V, Balkany Thomas J, Liu Xue Z
University of Miami Ear Institute, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
Otolaryngol Head Neck Surg. 2007 Oct;137(4):596-602. doi: 10.1016/j.otohns.2007.02.017.
To evaluate the speech perception and language development with cochlear implants (CI) of DFNB1 children in comparison with non-DFNB1 deaf children.
Retrospective case series.
Academic tertiary center.
Thirty-one congenitally deafened children, screened for GJB2 allele variants, were followed for an average 32 months after CI. With the use of age-appropriate testing, 75% of DFNB1 and 53% of non-DFNB1 children achieved open set word recognition (speech perception category [SPC] level 6). Multivariate analysis showed that SPC was primarily dependent on duration of CI use, but not on the cause of hearing loss. In Reynell language tests, DFNB1 children showed more consistent and quicker gains than non-DFNB1 children.
Although children with CI with DFNB1 show faster gains in Reynell scores, duration of CI use appears to have a greater effect on speech perception than DFNB1 status.
Identification of DFNB1 children is useful in counseling of CI outcomes.
评估DFNB1型儿童与非DFNB1型聋儿使用人工耳蜗（CI）后的言语感知和语言发育情况。
回顾性病例系列研究。
学术三级中心。
对31名先天性耳聋儿童进行GJB2等位基因变异筛查，在植入人工耳蜗后平均随访32个月。通过使用适合年龄的测试，75%的DFNB1型儿童和53%的非DFNB1型儿童达到了开放式单词识别（言语感知类别[SPC]6级）。多变量分析表明，SPC主要取决于人工耳蜗的使用时长，而非听力损失的原因。在雷内尔语言测试中，DFNB1型儿童比非DFNB1型儿童表现出更持续、更快的进步。
尽管DFNB1型人工耳蜗植入儿童在雷内尔评分中进步更快，但人工耳蜗的使用时长似乎比DFNB1状态对言语感知的影响更大。
识别DFNB1型儿童有助于人工耳蜗植入效果的咨询。
