Cholesteatoma in Congenital Aural Atresia and External Auditory Canal Stenosis: A Systematic Review

Abstract

Objective Patients with congenital external auditory canal (EAC) abnormalities are at risk of developing cholesteatoma and often undergo surveillance imaging to detect it. The aims of this systematic review are to determine the incidence of cholesteatoma in patients with congenital aural atresia (CAA) and patients with congenital EAC stenosis and to investigate the most common age of cholesteatoma diagnosis. This information will help clinicians decide which patients require surveillance scanning, as well as the timing of imaging. Data Sources Ovid MEDLINE, Embase, CENTRAL, and Web of Science databases. Review Methods A systematic literature review following the PRISMA guidelines was performed. The data sources were searched by 2 independent reviewers, and articles were included that reported on CAA or congenital EAC stenosis with a confirmed diagnosis of cholesteatoma. The selected articles were screened separately by 3 reviewers before reaching a consensus on the final articles to include. Data collection on the number of patients with cholesteatoma and the age of diagnosis was performed for these articles. Results Eight articles met the inclusion criteria. The incidence of cholesteatoma was 1.7% (4/238) in CAA and 43.0% (203/473) in congenital EAC stenosis. The majority of patients with congenital EAC stenosis that developed cholesteatoma were diagnosed at age <12 years. Conclusion CAA is associated with a low risk of cholesteatoma formation, and surveillance imaging is unnecessary in asymptomatic patients. EAC stenosis is strongly associated with cholesteatoma, and a surveillance scan for these patients is recommended prior to 12 years of age with close follow-up into adulthood.

Long-term Outcomes of Surgical Intervention for Symptomatic Aural Atresia and Stenosis

“…In this study, two patients under six years had done surgery and restenosis occurred in one of them. It may attribute this child was not old enough to comply with postoperative care (3,5). Thereby, for those younger patients, the lesion extent should be considered, and adequate communication is necessary before surgery.…”

“…Both aural atresia and stenosis may trap epithelial cells and potentially carriers a great risk of developing cholesteatoma (5). Chan's systematic review reported that the incidence of cholesteatoma is 43.0% in CAS and 1.7% in CAA (5). Close follow-up is commonly recommended to asymptomatic patients, such as the CAS patients with EAC diameter ≥ 2mm (3) However, for symptomatic aural atresia and stenosis, especially for patients with cholesteatoma, most studies agreed with prompt surgery (6, 7).…”

“…Additionally, postoperative care also serves to prevent early-stage restenosis. After these intraoperative and postoperative interventions, a high rate of postoperative recurrence is still reported ranging from 9-36% (5,6,8). A potential pathology for restenosis is broproliferative in ammatory response (16).…”

Cholesteatoma: Canalplasty for External Auditory Stenosis in a Pediatric Patient

“…The surgical objectives of canalplasty for EAC stenosis are both to improve conductive hearing and to facilitate the clearance of squamous and ceruminous debris from the EAC. Failure of the canal to clear squamous debris can lead to canal cholesteatoma, which is common in cases of EAC stenosis with a prevalence of 43% demonstrated in a recent systematic review [5]. Some reports estimate the prevalence in cases of canal stenosis to be as high as 91% [1,5,6].…”

Comparison of Outcomes of Surgery Versus Implantable Device for the Treatment of Hearing Loss Associated With Congenital Aural Atresia: A Systematic Review and Meta-Analysis

“…There is an association with syndromes including Treacher-Collins, Goldenhar, and Crouzon, although most cases are idiopathic and believed to be multifactorial (2). Individuals with CAA can have delayed speech and language development, a slightly increased risk of cholesteatoma development, or significant cosmetic concerns, but the most common associated problem is conductive hearing loss due to agenesis of the ear canal and related ossicular chain malformations (3)(4)(5). It is this substantial loss of hearing that often leads families to pursue treatment, and there are now a multitude of interventions available.…”