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.

Evaluation and Management of Aural Atresia; Review Article

“…Patients with CAS who did not have cholesteatoma often had meatoplasty at the age of six, when they were mature enough to grasp the reasoning behind the treatment and help with postoperative care. Except for patients under the age of six years, age was not an exclusion factor in CAS patients with cholesteatoma, and there was no significant difference in cholesteatoma production between age groups [2].…”

“…Another reason is that patients now understand and comply with postoperative instructions. The development of an external canal cholesteatoma, a known complication that can result in irreversible damage to the middle ear, is an exception to this age [2,24,25,7].…”

“…CAS is characterised by Cole and Jahrsdoerfer as an EAC with a diameter of 4 mm or less that frequently occurs in association with grade 1 and grade 2 microtia [2]. Congenital Aural Atresia (CAA) is a congenital ear deformity that affects the development of the external ear canal (EAC) to varying degrees.…”

“…Patient age and stenosis size are two essential factors to consider while dealing with CAS1. Patients with a stenosis of 2 mm or less are at a high risk of developing cholesteatoma and should be operated on [8]. There have been no large-scale investigations of the clinical aspects and longterm consequences of CAS to yet, and no research have detailed the critical parameter of determining the diameter of the EAC [2,[8][9][10][11][12][13][14].…”

Anatomy and Development of the Mammalian External Auditory Canal: Implications for Understanding Canal Disease and Deformity

“…Restenosis is prevented by performing a wide canalplasty, although this may have cosmetic implications. As there is no native canal skin to use, the neo-lumen has no capacity to clear desquamated debris, requiring regular aural microsuction ( Li et al, 2016 ). Hearing outcomes are typically poor despite the opening of the canal and patients may still need a hearing augmentation device as well.…”