Lateral semicircular canal (LSCC) dysplasia is a common malformation of the inner ear, which often results in hearing loss. The relationship between the extent of LSCC dysplasia and hearing loss severity is not well-understood. This case series aimed to investigate the differences in bony island radiologic measurements in patients with LSCC dysplasia according to hearing loss classification. Patients were recruited if they were less than 40 years old, underwent computed tomography scan (CTS) and had suspected LSCC dysplasia. The images were reviewed and the anterior-posterior diameter (APD), transverse diameter, and the surface area of the bony island were measured in millimeters. Audiometric data were used to classify hearing loss as profound hearing loss (PHL), conductive hearing loss (CHL), or mixed hearing loss (MHL). We also recorded patients’ demographics, and clinical symptoms such as vertigo and tinnitus. SPSS software was used for data analysis and a p value less than 0.05 was considered significant. Fourteen patients were recruited and the mean age of the participants was 20.4 ± 14.4 years. Bilateral LSCC dysplasia was detected in 12 patients, and unilateral dysplasia was observed in 2 patients (a total of 26 ears). CHL, MHL and PHL affected 13 (50%), 8 (31%), and 5 ears (19%), respectively. The mean APD was significantly smaller in MHL ears than in CHL ears (p = 0.03). Likewise, ears with MHL had smaller mean surface area than CHL ears, although the difference was not significant (p = 0.1). Vertigo or tinnitus was present in three patients. Unilateral and bilateral external auditory canal atresia was present in two patients, and one patient, respectively. We found that CHL was more common than profound hearing loss and MHL in patients with LSCC dysplasia. APD of bony island is a useful radiologic maker to distinguish between different types of hearing loss in this patient population. However, larger prospective studies are needed to confirm these findings and investigate the reasons for the observed smaller bony island in ears with MHL compared to those with CHL