The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of congenital aural stenosis (CAS). This study presents a retrospective review of patients who underwent meatoplasty for CAS at a tertiary referral hospital from 2008 to 2015. A total of 246 meatoplasty procedures were performed on 232 patients in the present study. We performed multivariate regression analysis. Except in the age < 6 years group, no significant difference was observed among different age groups for cholesteatoma formation, p > 0.05. Except for the stenosis of the external auditory canal (EAC) (>4 mm) group, the other stenosis of EAC groups were not associated with cholesteatoma formation, p > 0.05. Postoperative air-bone gaps (ABG) less than 30 dB occurred in 77.3% (99/128) of the patients, and the Jahrsdoerfer score was associated with postoperative ABG, p < 0.001. The complication rate of CAS was 13.8% (20/144), and males showed a higher risk for postoperative complications (OR, 6.563; 95% CI, 1.268-33.966, p = 0.025). These results indicate that meatoplasty was an effective surgical intervention for CAS, showing a stable hearing outcome with prolonged follow-up. There was no significant difference between the cholesteatoma and no cholesteatoma groups for hearing outcomes, p > 0.05.Congenital aural stenosis (CAS) carries a much greater risk of cholesteatoma compared with congenital aural atresia (CAA). Cole and Jahrsdoerfer defined CAS as an external auditory canal (EAC) with a diameter of 4 mm or less that frequently occurs in conjunction with grade 1 and grade 2 microtia. This diameter was selected because none of the patients with canal openings larger than 4 mm developed cholesteatoma 1,2 . The diagnosis of CAS is based on clinical examination (anamnesis, physical and audiometric evaluation) and imaging, particularly high-resolution computed tomography (HRCT) of the temporal bones 3 . The treatment of EAC cholesteatoma depends on the extent of the disease. Small lesions can typically be controlled with regular debridement, combined with the administration of topical antibiotics, while large and destructive lesions require surgery 4 . The data on patients with CAS are limited, and the fundamental knowledge of CAS is still based on Cole and Jahrsdoerfer (1990). Two important parameters to consider when managing with CAS are patient age and stenosis size 1 . Patients with stenosis sizes of 2 mm or less are at high risk for developing cholesteatoma and should undergo surgery; however, the data in the present study challenged this viewpoint. To date, there are no large sample studies focusing on the clinical features and long-term outcomes of CAS, and no studies have described the important parameter of measuring the diameter of EAC [5][6][7][8][9][10][11] . The aim of the present study was to comprehensively evaluate the clinical features and long-term outcomes of CAS, and challenge the previous viewpoint. Previous studies analysing the clinical features of CAS involved small sample sizes,...