Objective To analyze the characteristics of distorted product otoacoustic emissions (DPOAE) in children with Williams syndrome (WS) and provide clinical basis for the follow-up of the target population in the future. Methods Children with WS who were diagnosed and followed up in the Department of Pediatric Health Care from June 2019 to June 2023 were selected as the study objects. Children who came to the hospital for routine physical examination during the same period were taken as the control group. The difference of DPOAE between WS group and control group and the difference of DPOAE among WS children of different ages were compared. Results A total of 76 children with WS (42 boys and 34 girls) were included, with a median age of 4.7 (2.6-6.7) years. The control group consisted of 51 patients (32 boys and 19 girls) with a median age of 3.9 (2.9-5.2) years. There was no significant difference in DPOAE passing rate among different age groups in WS group (P>0.05). The DPOAE passing rate of WS group was 78.8%, lower than that of control group (97.0%), and the difference was statistically significant (P<0.001). In the 3-5 year-old group, the passing rate of DPOAE in the WS group was 76.6%, lower than that of the control group (96.7%). In the group of 6 years or older, the passing rate of DPOAE in the WS group was 69.6%, lower than that in the control group (100%), and the differences were statistically significant (P<0.05). There were statistically significant differences in the signal-to-noise ratio (SNR) of 2000Hz and 4000Hz between different age groups in WS (P<0.05). The SNR of 2000Hz in the 3 to 5 years old group was lower than that of the < 3 years old group, and the SNR of 2000Hz and 4000Hz in the ≥6 years old group was lower than that of the < 3 years old group, with statistical significance (P<0.05). The SNR of DPOAE at 2000, 3000, 4000 and 5000Hz at all ages in WS group was lower than that in the control group, with statistical significance (P<0.05). Conclusions Hearing screening abnormality is very common in children with WS, and there are manifestations of subclinical hearing loss. It is recommended to carry out long-term regular hearing follow-up for this group in order to detect hearing loss early and take corresponding intervention measures in time.