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To evaluate the relationship between vestibular function and gross motor development in children with inner ear malformations.A total of 195 children (average age: 2.4 ± 1.6 yr) with profound hearing loss, defined as a hearing threshold greater than 80 dB, participated in this study. Evaluation of vestibular function was assessed in all children using a damped rotational chair test. Gross motor development was assessed by examining the age of acquisition of head control and independent walking. Temporal bone computed tomography scans were conducted in 190 of the 195 children.Among the 195 children, 31 children (15.9%) showed a reduced response in the rotational chair test. Of the 17 children who were followed up with repeat rotational chair tests, 16 showed more obvious nystagmus during follow-up compared with the initial examination. The reduced response group showed significant delays in head control and independent walking compared with the normal response group (p < 0.01). Forty-one children (21.6%) demonstrated inner ear malformations and showed significantly delayed acquisition of head control and independent walking (p < 0.01) compared with children with normal vestibular function. Children with inner ear malformations with a reduced response in the rotational chair test had severe vestibular (p < 0.05) and lateral semicircular canal (p < 0.01) malformations as the most common findings.Structural inner ear malformations are associated with vestibular dysfunction and delayed gross motor development. Our findings confirm that there is a higher incidence of reduced vestibulo-ocular reflex in children with vestibular and semicircular canal malformations.