Background

Little is known regarding fitting parameters and receptive and expressive language development in cochlear-implanted children (CCI) with profound sensorineural hearing loss (SHL) who are diagnosed with Autism Spectrum Disorder (ASD). The aim of the study was to evaluate a group of ASD CCI users in order to describe their ASD clinical features and CCI outcomes; report on the average electrical charge requirements; and evaluate the possible correlations between electrical and psychophysical outcomes with ASD characteristics.

Materials and methods

A multicentre observational study of 22 ASD children implanted in four cochlear implant (CI) centers. Data concerning profound SHL diagnosis, ASD diagnosis, CI timing and CI compliance were collected. Sound Field (SF) was assessed through repeated behavioural measurements. Categories of Auditory Perception (CAP) and Categories of Language (CL) were used to evaluate speech perception and language skills at short (≤2 yrs), medium (5 yrs) and long term (>10 yrs) follow-up. Fitting parameters such as comfortable thresholds, pulse-width (pw, μsec) and clinical units converted into units of charge/phase were collected. The diagnosis of ASD was acquired by the referral neuropsychiatric department and severity was assessed through the Diagnostic and Statistical Manual of Mental Disorders (DSM-V) and the Childhood Autism Rating Scale (CARS).

Results

At the final follow-up session the median SF threshold for CI outcomes was 30 dB HL (min 15 – max 60). CAP score was extremely variable: 45.5% showed no improvement over time and only 22% of children reached CAP scores of 5–7. CL 45.5% showed no improvement over time and score was 1–2 in the majority of ASD children (72.7%), while only 18.2% reached the highest level of language skills. There were no statistically significant differences at each follow-up between subjects with or without comorbidities. CAP and CL were inversely correlated with DSM-V A and B domains, corresponding to lower speech and language scores in children with more severe ASD symptoms, and maintained their correlation at mid and long follow-ups whilst controlling for age at CI. Electrical charge requirements did not correlate with SF or age at implant but did inversely correlate with ASD severity. With regards to CI compliance: only 13.6% children (3) with severe DSM-V A/B levels and CARS score were partial/intermittent users.

Conclusion

The present study is a targeted contribution to the current literature to support clinical procedures for CI fitting and audiological follow-up in children with ASD. The findings indicate that the outcomes of CI use and the fitting procedures are both influenced by the severity of the ASD symptoms rather than the demographic variables or associated disorders.

中文翻译：

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自闭症谱系障碍 (ASD) 儿童的人工耳蜗植入：结果和植入物适配特征

背景

对于被诊断患有自闭症谱系障碍 (ASD) 的患有重度感音神经性听力损失 (SHL) 的人工耳蜗植入儿童 (CCI) 的拟合参数以及接受性和表达性语言发展知之甚少。该研究的目的是评估一组 ASD CCI 用户，以描述他们的 ASD 临床特征和 CCI 结果；报告平均电费要求；并评估电气和心理物理结果与 ASD 特征之间可能的相关性。

材料和方法

在四个人工耳蜗 (CI) 中心植入的 22 名 ASD 儿童的多中心观察性研究。收集了有关深度 SHL 诊断、ASD 诊断、CI 时间和 CI 依从性的数据。通过重复行为测量评估声场 (SF)。听觉感知类别 (CAP) 和语言类别 (CL) 用于评估短期（≤ 2 年）、中期（5 年）和长期（> 10 年）随访时的言语感知和语言技能。收集拟合参数，例如舒适阈值、脉宽（pw、μsec）和转换为电荷/相位单位的临床单位。ASD 的诊断由转诊神经精神科获得，严重程度通过精神疾病诊断和统计手册 (DSM-V) 和儿童自闭症评定量表 (CARS) 进行评估。

结果

在最后一次随访时，CI 结果的中位 SF 阈值为 30 dB HL（最小 15 – 最大 60）。CAP 评分变化很大：45.5% 的儿童没有随着时间的推移而改善，只有 22% 的儿童达到 5-7 的 CAP 评分。CL 45.5% 随着时间的推移没有改善，大多数 ASD 儿童 (72.7%) 的得分为 1-2，而只有 18.2% 的儿童达到了语言技能的最高水平。有或没有合并症的受试者在每次随访时没有统计学上的显着差异。CAP 和 CL 与 DSM-V A 和 B 域呈负相关，对应于具有更严重 ASD 症状的儿童的较低的言语和语言分数，并在中长期随访时保持相关性，同时控制 CI 的年龄。电荷需求与 SF 或植入年龄无关，但与 ASD 严重程度呈负相关。关于 CI 依从性：只有 13.6% 的 DSM-V A/B 水平和 CARS 评分严重的儿童 (3) 是部分/间歇性使用者。

结论

本研究是对当前文献的有针对性的贡献，以支持 ASD 儿童 CI 拟合和听力学随访的临床程序。研究结果表明，CI 使用的结果和拟合程序都受到 ASD 症状严重程度的影响，而不是人口统计学变量或相关疾病。