Purpose: The aim of this study was to evaluate and compare the postural stability and sensory integration disorders of pediatric cochlear implant users with and without inner ear malformations. Material and Methods: The control group consists of 20 cochlear implant users with normal cochlea whereas the study group comprises 20 cochlear implant users with inner ear malformation. Forty children, between the ages of 5 to 12 years, were participated in the current study. Hearing thresholds with cochlear implant of all subjects were determined. Computerized Dynamic Posturography (CDP) and Ayres Southern California Sensory Integrity Tests were performed to all subjects. Results: There was a significant difference between the combined balance scores and vestibular ratio of the children in both groups in CDP (p=0.00). Sensory integration test scores of the children in the study group were found lower than the control group. Visual shape perception (p=0.019) scores of the control group, midline crossing (p=0.03) and right-left discrimination (p=0.00) scores of the study group were improved with the age. A significant difference was found between the right-left discrimination scores of two groups before the age of nine (p=0.02). Discussion: It was determined that the balance skills of the children with inner ear malformations were more affected than the children with a normal cochlea. The children with cochlear implants might frequently encounter with sensory integrity disorders. Early evaluation of the postural stability and the sensory integrity disorders and selection of the appropriate rehabilitation programs were recommended. Key Words: Hearing loss; Postural balance; Sensory disorders; Cochlear implant