The advent of cochlear implants has enormously improved the quality of sensory perception in deaf children. Notwithstanding these advantages, the current literature shows a substantial variability in language proficiency among implanted children. This case series explores the variability of language acquisition in congenitally deaf children with cochlear implants. We report 4 prelingually deaf children (; ), affected by a genetically determined bilateral deafness, due to GJB2 gene mutation Cx26. Each implanted child underwent a systematic assessment of speech perception and production, as well as of lexical, morphologic, and syntactic skills in both comprehension and production. Notwithstanding similar clinical histories and similarly good postimplant pure-tone audiometry, two of the four children fared very poorly in speech audiometry, whereas the other two children gained very good results. We suggest that the language impairment detected in (some) implanted children may not be fully accounted for by pure auditory thresholds and that may be the outcome of concomitant damage to core components of the child’s linguistic brain.

中文翻译：

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人工耳聋的聋前儿童的语言发展

耳蜗植入物的出现极大地改善了聋儿的感觉知觉质量。尽管有这些优点，但目前的文献显示植入儿童的语言能力存在很大差异。本案例系列探讨了先天性聋儿童耳蜗植入物中语言习得的变异性。我们报告了4名聋哑儿童（; ），由于GJB2基因突变Cx26，受到遗传性双耳聋的影响。每个植入的孩子都要进行系统的语音理解和产生评估，以及对理解和产生方面的词汇，形态和句法技巧的评估。尽管有相似的临床历史和类似的良好的植入后纯音听力测验，但四个孩子中的两个在语音测听方面表现很差，而另外两个孩子则取得了很好的效果。我们建议在（某些）植入儿童中检测到的语言障碍可能无法通过纯听阈来完全解决，这可能是对儿童语言大脑核心成分的同时损害的结果。