Abstract Objective: To compare the comprehensive developments between children with large vestibular aqueduct syndrome (LVAS) and children without LVAS and investigate the risk factors for developments of LVAS children. Design: A retrospective propensity score matching analysis. Setting: Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing, China. Method: 70 children with LVAS (41 boys, median of 28.50 months, range 6-72 months, hearing threshold: 82.54±12.58 dB nHL) and 70 gender-, age-, and auditory- matched overall children were recruited as LVAS and non-LVAS group, respectively. The developments of children were assessed by Gesell development schedules. Then the overall development and sub-development results were compared between the two groups. Regression analyses were used to assess the association between potential risk factors and developments of LVAS subjects. Results: Compared with normal developmental metrics, LVAS children and non-LVAS children both had developmental delay (both P<.001), which occurred not only in verbal but also in non-verbal aspects (all P<.05) except gross motor in non-LVAS subjects. Whereas, the deaf children of two groups had the similar performance including all sub-developments (all P>.05). For the developments of LVAS children, the age of intervention was risk factor (B<0, P<.05) and the developments of children older than 12 months was worse than those of children younger than 12 months (P<.05). Conclusion: Children with LVAS had the equal developmental level in comparison with children with same hearing impairments. Age of diagnosis was the risk factor for developments of LVAS children and the obvious delay of developments stared from 12 months.