Cochlear neurodysplasia (CND) is recognized as a contributing factor to sensorineural hearing loss in children. This study aimed to investigate the relationship between modiolus density on high-resolution computed tomography (HRCT) and CND, and to evaluate its performance in diagnosing CND. This retrospective study collected HRCT images of 34 patients diagnosed with unilateral neurological hearing loss in the Children's Hospital of Chongqing Medical University from March 2018 to December 2023, who were also diagnosed with unilateral CND by computed tomography (CT) and magnetic resonance imaging (MRI) hydroimaging. CT values of the modiolus and petrous bone were measured on the affected and healthy sides, in addition to determining the width of cochlear nerve foramina and the width of internal auditory tract. The receiver operator characteristic (ROC) curve was used to evaluate the diagnostic performance of these features. Simultaneously, comparisons were conducted with parameters obtained from normal children. A total of 29 patients without CND were randomly selected as a control group. The unilateral sensorineural hearing loss group had 34 patients, comprising 18 males and 16 females, with a median age of 4.5 years, ranging from 0.7 to 11 years. The normal children group consisted of 20 males and 9 females, with a median age of 5.9 years, ranging from 0.5 to 12.0 years. Statistically significant differences were observed in the CT values of the modiolus, modiolus/petrous bone CT value ratio, width of cochlear nerve foramina, and width of internal auditory tract between the affected and healthy sides in patients with unilateral sensorineural hearing loss (P<0.05). The area under the ROC curve (AUC) of the modiolus CT value and the width of cochlear nerve foramina for the diagnosis of unilateral sensorineural hearing loss was 0.98 [95% confidence interval (CI): 0.95-1.00] and 0.99 (95% CI: 0.98-1.00), respectively. the modiolus density was significantly elevated in the affected sides in patients with unilateral CND. The optimal cut-off value of modiolus CT values was 983 Hounsfield unit (HU). The elevated density of the modiolus on HRCT holds significant value in diagnosing CND.