Zarchi Omer, Avni Chen, Attias Josef, Frisch Amos, Carmel Miri, Michaelovsky Elena, Green Tamar, Weizman Abraham, Gothelf Doron
Behavioral Neurogenetics Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel.
Institute for Clinical Neurophysiology and Audiology, Rabin Medical Center and Schneider Children's Medical Center, Petah Tikva, Israel.
Psychophysiology. 2015 Jun;52(6):782-9. doi: 10.1111/psyp.12407. Epub 2015 Jan 21.
The neurophysiologic aberrations underlying the auditory hypersensitivity in Williams syndrome (WS) are not well defined. The P1-N1-P2 obligatory complex and mismatch negativity (MMN) response were investigated in 18 participants with WS, and the results were compared with those of 18 age- and gender-matched typically developing (TD) controls. Results revealed significantly higher amplitudes of both the P1-N1-P2 obligatory complex and the MMN response in the WS participants than in the TD controls. The P1-N1-P2 complex showed an age-dependent reduction in the TD but not in the WS participants. Moreover, high P1-N1-P2 complex was associated with low verbal comprehension scores in WS. This investigation demonstrates that central auditory processing is hyperactive in WS. The increase in auditory brain responses of both the obligatory complex and MMN response suggests aberrant processes of auditory encoding and discrimination in WS. Results also imply that auditory processing may be subjected to a delayed or diverse maturation and may affect the development of high cognitive functioning in WS.
威廉姆斯综合征（WS）中听觉过敏背后的神经生理异常尚未明确界定。对18名WS患者的P1-N1-P2必发复合波和失匹配负波（MMN）反应进行了研究，并将结果与18名年龄和性别匹配的发育正常（TD）对照者的结果进行了比较。结果显示，WS患者的P1-N1-P2必发复合波和MMN反应的波幅均显著高于TD对照者。P1-N1-P2复合波在TD对照者中呈现出随年龄增长而降低的趋势，但在WS患者中并非如此。此外，WS患者中较高的P1-N1-P2复合波与较低的语言理解得分相关。这项研究表明，WS患者的中枢听觉处理功能亢进。必发复合波和MMN反应的听觉脑反应增强表明WS患者存在听觉编码和辨别异常过程。结果还表明，听觉处理可能经历延迟或不同的成熟过程，并可能影响WS患者高级认知功能的发展。
