Objective To investigate the value of CT in the diagnosis and treatment of children with inner ear malformation before surgery. Methods 120 children with inner ear malformation who were admittedto the Hearing DiagnosisCenter of the hospitalfrom January 2015 to December 2016 were selected as subjects, and they were examined by multislice spiral CT scan. Three children with normal inner ear structure were taken as controls. CT detection and classification of 120 children with inner ear malformation were statistically analyzed, and the value of CT in the diagnosis and treatment of children with inner ear malformation was analyzed. Results Of the 120 children examined by CT scan, there were 235 ears with inner ear malformations, and the numbers of cases with cochlear malformations [including cochlear aplasia (cochlear structure deletion), cochlear dysplasia (relatively smaller cochlea, cochlear spiral≤1 cycle)], vestibular malformations (vestibular enlargement or fusion with cochlea forming common cavity), semicircular canal malformations (small, expand or cracked semicircular canal), internal auditory canal malformations (internal auditory canal stenosis or enlargement), vestibular aqueduct malformations (vestibular aqueduct opening being trumpet shaped enlargementand middle diameter> 1.5cm) were 56, 59, 62, 22 and 96, respectively. There were a total of 20 ears (8.51%) with single structural malformations and 216 ears (91.91%) with more than 2 kinds of malformations. There were 115 cases (230 ears) with bilateral ears malformations in the 120 children with inner ear malformations, accounting for 95.83%. 6 children had different types of inner ear malformations. There were 5 cases (5 ears) with monaural malformation, accounting for 4.17%. Conclusion CT can clearly show the classification of inner ear malformation and the degree of malformation in the preoperative diagnosis and treatment of children with inner ear malformations, which is of important reference value for improving the accurate diagnosis rate of inner ear malformation and the selection of surgical plan.