Abstract Background Williams Syndrome (WS) is a neurodevelopmental disorder caused by microdeletion on chromosome 7. Hearing loss (HL) is common in this population but is rarely taken seriously. Previous studies had small sample sizes and mixed conclusions, and few studies have investigated HL in children with WS. Objectives To investigate audiological characteristics of children with WS, analyze the influence factors, and to provide scientific basis for further improvement of ear and hearing care in children with WS. Methods Children with WS aged 0-18yrs, followed up in the Department of Pediatric Healthcare of the Children’s Hospital of Zhejiang University School of Medicine from June 2020 to June 2024 were enrolled in this study. Children aged 0-18yrs who came in the same period for health examination were matched as the control group. Both groups underwent a series of audiological examinations such as tympanogram, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR) and pure-tone audiometry (PTA), to analyze the audiological characteristics of WS at different ages, and their difference with control group. Tympanogram and DPOAE were suggested to retest 1 year later and the results of first and second test were also compared. Results Tympanogram and DPOAE were completed in 130 WS and control subjects, ranging in age from 1.0 to 12.4 years in the WS group and 0.8–13.1 years in the control group. The passing rate of tympanogram and DPOAE in WS was significantly decreased when compared with control group (p