Background

Our goal was to assess for the first time early vocalizations as precursors to speech in audio-video recordings of infants with tuberous sclerosis complex (TSC).

Methods

We randomly selected 40 infants with TSC from the TSC Autism Center of Excellence Research Network data set. Using human observers, we analyzed 74 audio-video recordings within a flexible software-based coding environment. During the recordings, infants were engaged in developmental testing. We determined syllables per minute (volubility), the number of consonant-vowel combinations, such as ‘ba’ (canonical babbling), and the canonical babbling ratio (canonical syllables/total syllables) and compared the data with 2 groups of typically developing (TD) infants. One comparison group's data had come from a laboratory setting, while the other's had come from all-day Language Environment Analysis recordings at home.

Results

Compared with TD infants in laboratory and all-day Language Environment Analysis recordings, entry into the canonical babbling stage was delayed in the majority of infants with TSC, and the canonical babbling ratio was low (TD mean = 0.346, SE = 0.19; TSC mean = 0.117, SE = 0.023). Volubility level in infants with TSC was less than half that of TD infants (TD mean = 9.82, SE = 5.78; TSC mean = 3.99, SE = 2.16).

Conclusions

Entry into the canonical stage and other precursors of speech development were delayed in infants with TSC and may signal poor language and developmental outcomes. Future studies are planned to assess prediction of language and developmental outcomes using these measures in a larger sample and in more precisely comparable recording circumstances.

中文翻译：

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结节性硬化症的早期发声发展

背景

我们的目标是首次评估早期发声作为患有结节性硬化症 (TSC) 的婴儿的音频视频记录中语音的前兆。

方法

我们从 TSC 自闭症卓越研究中心网络数据集中随机选择了 40 名患有 TSC 的婴儿。通过使用人类观察员，我们在基于软件的灵活编码环境中分析了 74 段音频-视频记录。在录音期间，婴儿进行了发育测试。我们确定了每分钟的音节数（音量）、辅音-元音组合的数量，例如“ba”（标准牙牙学语）和标准牙牙学语比率（标准音节数/总音节数），并将数据与 2 组典型发育中的孩子（ TD)婴儿。一个比较组的数据来自实验室环境，而另一个则来自家里全天的语言环境分析记录。

结果

与实验室和全天语言环境分析记录中的TD婴儿相比，大多数TSC婴儿进入典型牙牙学语阶段的时间较晚，典型牙牙学语比率较低（TD均值=0.346，SE=0.19；TSC均值= 0.117，SE = 0.023）。TSC 婴儿的容量水平低于 TD 婴儿的一半（TD 平均值 = 9.82，SE = 5.78；TSC 平均值 = 3.99，SE = 2.16）。

结论

患有 TSC 的婴儿进入规范阶段和其他语言发育前兆的时间有所延迟，这可能预示着语言和发育结果较差。未来的研究计划在更大的样本和更精确的可比记录环境中使用这些措施来评估语言和发展结果的预测。