1Nottingham Cochlear Implant Programme, Ropewalk House, Nottingham, United Kingdom.
Ear Hear. 2011 Nov-Dec;32(6):690-8. doi: 10.1097/AUD.0b013e31821f0538.
We previously presented evidence for the existence of a "disproportionate language impairment" (DLI) in a small sample of children using a cochlear implant (CI) who had a good surgical outcome and who were otherwise unimpaired, including typical nonverbal intelligence quotient. Herein, we studied a larger sample of children with DLI who were CI users and, as before, paired them with closely matched, "Control" children who were CI users but had more typical language skills. The primary aim was to assess the communication skills of siblings of each group of CI users to seek evidence for the inheritance of language and more general communicative impairments in the DLI group. We also investigated laterality as a possible coindicator of impairment among the CI users.
Prelingually deaf children using a single CI and presenting with severe unexplained language problems (DLI-CI, N = 25) were compared with a group of children using a CI who did not present with such difficulties (Control-CI, N = 25). The groups were matched on age, gender, etiology, age at implantation, implant manufacturer, duration of deafness, and CI experience. Members of both groups completed a series of nonverbal and verbal tests used to identify specific language impairment in normal-hearing (NH) children, and tests of cerebral laterality. Parents completed the Children's Communication Checklist (CCC-2) for each of their children (i.e., the CI users and all their siblings; sibling results were grouped as DLI-Sibling and Control-Sibling) and a questionnaire regarding family history of hearing, speech, language, and literacy problems of the children's first-degree relatives.
Significant differences of performance on all language tests were found between the DLI-CI and the Control-CI groups. No difference was found between the groups on performance intelligence quotient or auditory memory. CCC-2 results indicated lower General Communication Composite and higher Social Interaction Deviance Composite scores in the DLI-CI than in the Control-CI group. All children in the DLI-CI group produced abnormal overall profiles compared with less than half the children in the Control-CI group. CCC-2 results for the siblings showed a significant association between sibling group and communication; 35% in the DLI-Sibling group produced abnormal profiles compared with 8% in the Control-Sibling group. Co-occurring deafness and language problems in a small number of siblings and other family members did not account fully for these communication difficulties, but there was a higher prevalence of these difficulties in the families of both groups than is reported in NH families. Laterality tasks did not show significant differences between the groups of CI users, although there were trends for children in the DLI-CI group to have less dominant hand preference and eye-hand dominance than those in the Control-CI group and in NH children.
Language tests, including the CCC-2, could identify communication difficulties in children using CIs, leading to prompt identification, informed intervention strategies, and managed expectations. Evidence from their siblings suggests that these difficulties may not all be determined by their deafness or device characteristics but through the same heritable and environmental factors that influence language development in all children.
我们之前在一组使用人工耳蜗(CI)的儿童中发现了“语言障碍失调”(DLI)的证据,这些儿童手术效果良好,且其他方面无障碍,包括典型的非言语智商。在此,我们研究了一组较大的 DLI 儿童,他们均为 CI 用户,并与之前的“对照组”相匹配,这些儿童也为 CI 用户,但语言技能更典型。主要目的是评估每组 CI 用户的兄弟姐妹的沟通能力,以寻求 DLI 组语言和更普遍的沟通障碍遗传的证据。我们还研究了偏侧性作为 CI 用户障碍的一个可能的指示符。
研究了一组因语言问题而使用 CI 的儿童(DLI-CI,N=25),这些儿童因语言问题而使用 CI,而未出现这种困难的儿童为对照组(Control-CI,N=25)。这两组儿童在年龄、性别、病因、植入年龄、植入设备制造商、耳聋持续时间和 CI 使用经验方面相匹配。两组儿童均完成了一系列非言语和言语测试,用于识别正常听力(NH)儿童的特定语言障碍,并测试大脑的偏侧性。父母为每个孩子完成了儿童沟通检查表(CCC-2),即 CI 用户及其所有兄弟姐妹(兄弟姐妹的结果分为 DLI-兄弟姐妹和 Control-兄弟姐妹),并完成了有关其一级亲属听力、言语、语言和读写问题家族史的问卷。
DLI-CI 组和 Control-CI 组在所有语言测试中的表现均存在显著差异。两组儿童在智商或听觉记忆方面没有差异。CCC-2 结果表明,与对照组相比,DLI-CI 组的一般沟通综合评分较低,社会互动异常评分较高。与对照组相比,DLI-CI 组的所有儿童产生的总体图谱均异常,而对照组中不到一半的儿童图谱异常。CCC-2 结果显示,兄弟姐妹组与沟通之间存在显著关联;DLI-兄弟姐妹组中有 35%的儿童产生异常图谱,而对照组中有 8%的儿童产生异常图谱。少数兄弟姐妹和其他家庭成员的失聪和语言问题并不能完全解释这些沟通困难,但与 NH 家庭相比,两组家庭的这些困难更为普遍。偏侧性任务未显示 CI 用户组之间的显著差异,但 DLI-CI 组的儿童与对照组和 NH 儿童相比,惯用手偏好和眼手优势的程度较低。
包括 CCC-2 在内的语言测试可以识别使用 CI 的儿童的沟通困难,从而及时识别、提供知情的干预策略并进行管理预期。他们兄弟姐妹的证据表明,这些困难可能并非完全由听力丧失或设备特征决定,而是由影响所有儿童语言发展的相同遗传和环境因素决定的。