Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

Scientific Reports, Jun 2016

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.

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Congenital Aural Stenosis: Clinical Features and Long-term Outcomes

Abstract 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. Introduction 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 cholesteatoma1,2. 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 bones3. 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 surgery4. 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 size1. 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 EAC5,6,7,8,9,10,11. 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, and no studies focused on the stability of the hearing outcomes5,6,7. This study uses the largest sample of CAS, providing guidance in consultation and emphasizing important aspects for long-term follow-up. Results A total of 246 meatoplasty procedures were performed on 232 patients in the present study. The mean age at surgery was 11.5 ± 7.2 years (range: 2–50 years), and 84.9% (209/246) of the patients were younger than 18 years when the procedures were performed. The mean time to follow-up was 18.3 ± 19.7 months (range: 1–78 months). Demographic data For the purpose of this study, each of the 246 procedures was considered as an individual case, including 110 ears without cholesteatoma and 136 ears with cholesteatoma. In the cholesteatoma group, 53 ears were stages I and II, 55 ears were stage III and 28 ears were stage IV. There were 161 males with 97 cholesteatomas and 85 females with 39 cholesteatomas; 140 ears were affected on the right side with 90 cholesteatomas, and 106 ears were affected on the left side with 46 cholesteatomas; 57.3% (78/136) of the cholesteatoma patients also presented with infection, and 4.5% (5/110) of the patients without cholesteatoma presented with infection. To analyse the relationship between age and cholesteatoma formation, we divided the patients into four groups: age < 6, 6 ≤ age < 12, 12 ≤ age < 18 and age ≥ 18 years. Nearly half of the patients underwent surgery at 6 to 12 years old. A total of 32 meatoplasty procedures were performed in patients less than 6 years old. Except for 3 ears without cholesteatoma, the remaining 29 ears underwent surgery for cholesteatoma (Table 1). We conducted a multivariate logistic regression analysis for cholesteatoma formation. The results showed that males (OR, 2.401; 95% CI, 1.054–5.469, p = 0.037), the right side group (OR, 3.531; 95% CI, 1.618–7.706, p = 0.002); individuals aged <6 years (OR, 6.494; 95% C (...truncated)


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Chen-long Li, Ying Chen, Yong-zheng Chen, Yao-yao Fu, Tian-yu Zhang. Congenital Aural Stenosis: Clinical Features and Long-term Outcomes, Scientific Reports, 2016, Issue: 6, DOI: 10.1038/srep27063