Individuals with Tuberous Sclerosis Complex (TSC) have atypical white matter integrity and neural connectivity in the brain, including language pathways. To explore functional activity associated with auditory and language processing in individuals with TSC, we used electroencephalography (EEG) to examine basic auditory correlates of detection (P1, N2, N4) and discrimination (mismatch negativity, MMN) of speech and non-speech stimuli for children with TSC and age- and sex-matched typically developing (TD) children. Children with TSC (TSC group) and without TSC (typically developing, TD group) participated in an auditory MMN paradigm containing two blocks of vowels (/a/and/u/) and two blocks of tones (800 Hz and 400 Hz). Continuous EEG data were collected. Multivariate pattern analysis (MVPA) was used to explore functional specificity of neural auditory processing. Speech-specific P1, N2, and N4 waveform components of the auditory evoked potential (AEP) were compared, and the mismatch response was calculated for both speech and tones. MVPA showed that the TD group, but not the TSC group, demonstrated above-chance pairwise decoding between speech and tones. The AEP component analysis suggested that while the TD group had an increased P1 amplitude in response to vowels compared to tones, the TSC group did not show this enhanced response to vowels. Additionally, the TD group had a greater N2 amplitude in response to vowels, but not tones, compared to the TSC group. The TSC group also demonstrated a longer N4 latency to vowels compared to tones, which was not seen in the TD group. No group differences were observed in the MMN response. In this study we identified features of the auditory response to speech sounds, but not acoustically matched tones, which differentiate children with TSC from TD children.

结节性硬化症 (TSC) 患者的大脑白质完整性和神经连接（包括语言通路）不典型。为了探索 TSC 患者与听觉和语言处理相关的功能活动，我们使用脑电图 (EEG) 来检查语音和非语音刺激的检测（P1、N2、N4）和辨别（失配负性，MMN）的基本听觉相关性适用于患有 TSC 的儿童以及年龄和性别匹配的典型发育 (TD) 儿童。患有 TSC 的儿童（TSC 组）和没有 TSC 的儿童（通常是发育中的 TD 组）参与了包含两个元音块（/a/和/u/）和两个音调块（800 Hz 和 400 Hz）的听觉 MMN 范式。收集连续的脑电图数据。多变量模式分析（MVPA）用于探索神经听觉处理的功能特异性。比较听觉诱发电位 (AEP) 的语音特定 P1、N2 和 N4 波形分量，并计算语音和音调的失配响应。MVPA 表明 TD 组（而非 TSC 组）展示了语音和音调之间的高概率成对解码。AEP 成分分析表明，虽然与音调相比，TD 组对元音的响应 P1 幅度有所增加，但 TSC 组并未表现出对元音的这种增强的响应。此外，与 TSC 组相比，TD 组对元音的 N2 振幅更大，但对音调的响应却没有。与音调相比，TSC 组还表现出元音的 N4 潜伏期更长，这在 TD 组中未见。MMN 反应没有观察到组间差异。在这项研究中，我们确定了对语音的听觉反应的特征，但没有确定声学匹配的音调，这些特征可以区分 TSC 儿童和 TD 儿童。