title: Hyperactive auditory processing in Williams syndrome: Evidence from auditory evoked potentials.
abstract: 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.
authors.affiliation: 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.
authors.forename: Omer
authors.initials: O
authors.lastname: Zarchi
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Chen
authors.initials: C
authors.lastname: Avni
authors.affiliation: Institute for Clinical Neurophysiology and Audiology, Rabin Medical Center and Schneider Children's Medical Center, Petah Tikva, Israel.|Department of Communication Sciences & Disorders, Haifa University, Haifa, Israel.
authors.forename: Josef
authors.initials: J
authors.lastname: Attias
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.|Biochemical Genetics Laboratory, Felsenstein Medical Research Center, Petah Tikva, Israel.
authors.forename: Amos
authors.initials: A
authors.lastname: Frisch
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.|Biochemical Genetics Laboratory, Felsenstein Medical Research Center, Petah Tikva, Israel.
authors.forename: Miri
authors.initials: M
authors.lastname: Carmel
authors.affiliation: Biochemical Genetics Laboratory, Felsenstein Medical Research Center, Petah Tikva, Israel.
authors.forename: Elena
authors.initials: E
authors.lastname: Michaelovsky
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.|Nes-Ziyyona-Beer Yaakov Mental Health Center, Nes-Ziyyona, Israel.
authors.forename: Tamar
authors.initials: T
authors.lastname: Green
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.|Biochemical Genetics Laboratory, Felsenstein Medical Research Center, Petah Tikva, Israel.|Research Unit, Geha Mental Health Center, Petah Tikva, Israel.
authors.forename: Abraham
authors.initials: A
authors.lastname: Weizman
authors.affiliation: Behavioral Neurogenetics Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel.|Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Doron
authors.initials: D
authors.lastname: Gothelf
mesh_terms: D000161:Acoustic Stimulation; D000293:Adolescent; D000328:Adult; D001303:Auditory Cortex / Q000503:physiopathology*; D002648:Child; D004569:Electroencephalography; D005072:Evoked Potentials, Auditory / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D011930:Reaction Time / Q000502:physiology; D018980:Williams Syndrome / Q000503:physiopathology*; D055815:Young Adult
keywords: Auditory processing; Evoked response potentials (ERP); Hyperacusis; Williams syndrome