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Psychophysiology. 2015 Jun;52(6):782-9. doi: 10.1111/psyp.12407. Epub 2015 Jan 21.

Hyperactive auditory processing in Williams syndrome: Evidence from auditory evoked potentials.

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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.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Department of Communication Sciences & Disorders, Haifa University, Haifa, Israel.
Biochemical Genetics Laboratory, Felsenstein Medical Research Center, Petah Tikva, Israel.
Nes-Ziyyona-Beer Yaakov Mental Health Center, Nes-Ziyyona, Israel.
Research Unit, Geha Mental Health Center, Petah Tikva, Israel.


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.


Auditory processing; Evoked response potentials (ERP); Hyperacusis; Williams syndrome

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