Auditory processing abnormalities in fragile X syndrome (FXS) may contribute to difficulties with language development, pattern identification, and contextual updating. Participants with FXS (N = 41) and controls (N = 27) underwent auditory event-related potentials during presentation of an oddball paradigm. Data was adequate for analysis for 33 participants with FXS and 27 controls (age 4-51 y, 13 females [FXS]; 4-54 y, 11 females [control]). Participants with FXS showed larger N1 and P2 amplitudes, abnormal lack of modulation of P1 and P2 amplitudes and P2 latency in response to oddball stimuli ) relative to controls: Females with FXS were more similar to controls. Participants with FXS showed a marginal speeding of the P2 latency, suggesting potentiation to oddball stimuli rather than habituation. Participants with FXS showed a heightened N1 habituation effect compared to controls. Gamma power was significantly higher for participants with FXS. Groups did not differ on mismatch negativity. Both controls and participants with FXS showed similar developmental trajectories in P1 and N1 amplitude, P2 latency, and gamma power, but not for P2 amplitude. One month retest analyses performed in 14 participants suggest strong test-retest reliability for most measures. Individuals with FXS show previously demonstrated increased response amplitude and high frequency neural activity. Despite an overall normal developmental trajectory for most measures, individuals with FXS show age-independent but gender-dependent decreases in complex processing of novel stimuli. Many markers show strong retest reliability even in children and thus are potential biomarkers for clinical trials in FXS.

中文翻译：

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脆性 X 综合征中对新声音的神经反应的发展：潜在的生物标志物。

脆性 X 综合征 (FXS) 中的听觉处理异常可能导致语言发展、模式识别和上下文更新的困难。FXS (N = 41) 和对照组 (N = 27) 的参与者在呈现古怪范式期间经历了与听觉事件相关的电位。数据足以分析 33 名 FXS 参与者和 27 名对照（年龄 4-51 岁，13 名女性 [FXS]；4-54 岁，11 名女性 [对照]）。相对于对照组，患有 FXS 的参与者表现出较大的 N1 和 P2 振幅、异常缺乏 P1 和 P2 振幅的调制以及 P2 对古怪刺激的反应的潜伏期）：患有 FXS 的女性与对照组更相似。患有 FXS 的参与者表现出 P2 潜伏期的边际加速，表明对古怪刺激的增强而不是习惯化。与对照组相比，FXS 的参与者表现出更高的 N1 习惯效应。患有 FXS 的参与者的伽马功率显着更高。组在错配消极性方面没有差异。患有 FXS 的对照组和参与者在 P1 和 N1 幅度、P2 潜伏期和伽马功率方面都表现出相似的发展轨迹，但 P2 幅度却没有。对 14 名参与者进行的 1 个月重测分析表明，大多数测量具有很强的重测信度。患有 FXS 的个体先前表现出增加的反应幅度和高频神经活动。尽管大多数措施的总体发展轨迹正常，但 FXS 个体在新刺激的复杂处理中表现出与年龄无关但与性别相关的下降。