With the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended.To identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis.This study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared.The cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X2 = 18.228, P<0.001) (oVEMP: X2 = 7.528, P = 0.023). Significant inter-group differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups.Patients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training.Copyright © 2023 Elsevier B.V. All rights reserved.