Long-Latency Auditory Evoked Potentials in Adults with Williams Syndrome.

Abstract

Background:  Williams syndrome (WS) is a genetic, multisystemic, neurodevelopmental disorder, in which studies have demonstrated the presence of auditory deficits such as conductive and sensorineural hearing loss, cochlear weakness, and subclinical signs of hearing impairment. However, few studies have assessed this population's central auditory system.

Purpose:  To analyze long-latency auditory evoked potential (LLAEP) in adults with WS and compare them with those obtained from neurotypical individuals with no hearing complaints.

Research design:  A cross-sectional observational study with subjects who were submitted to LLAEP assessment with the oddball paradigm and tone-burst stimuli.

Study sample:  A total of 30 individuals of both sexes, aged 18 to 37 years-15 had WS and comprised the study group (SG) and 15 did not have either the syndrome or hearing complaints and comprised the control group (CG); they were matched for sex and age.

Results:  The LLAEP analysis showed larger P1-N1 and P2-N2 amplitudes but smaller N2-P3 amplitude in SG, which also had delayed latencies in all components compared with CG.

Conclusion:  Adults with WS had increased neuronal response in identifying stimulus characteristics, attentional difficulties in auditory tasks, and deficits in auditory information processing speed.