Pediatric cholesteatoma associated with congenital aural atresia and stenosis

Am J Otolaryngol. 2023 Nov-Dec;44(6):103986. doi: 10.1016/j.amjoto.2023.103986. Epub 2023 Jul 6.


Objective: Investigate presenting features, associated surgical treatment, and outcomes in patients with cholesteatoma associated with congenital aural atresia (CAA) or stenosis (CAS).

Methods: Colorado Multiple Institution Review Board approval was obtained. A retrospective chart review was performed at a single tertiary care children's hospital of all pediatric patients with congenital aural atresia or stenosis with associated cholesteatoma from January 1, 2003, to October 15, 2018.

Results: Of the 278 patients identified with CAA or CAS, twelve (4.3 %) were found to have a canal cholesteatoma. There was a male predominance (8:4). Nine patients (75 %) had conductive loss and three (25 %) had mixed loss. Four patients (33.3 %) exhibited canal cholesteatomas extending into the middle ear or mastoid cavity. All patients underwent surgery, and 25 % of patients required revision canalplasty while 58 % of patients required revision surgery for cholesteatoma recidivism. The average age at the time of surgery was 11.3 ± 3.7 years.

Conclusion: Fewer than 5 % of pediatric patients with congenital aural atresia or stenosis were diagnosed with an acquired canal cholesteatoma. The need for revision surgery was common, occurring in >50 % of cases. Screening patients with CAA/CAS for cholesteatoma with imaging is recommended to avoid the morbidity of delayed identification.

Keywords: Canal cholesteatoma; Congenital aural atresia; Congenital aural stenosis; Pediatric cholesteatoma.

MeSH terms

  • Adolescent
  • Child
  • Cholesteatoma*
  • Constriction, Pathologic / surgery
  • Ear / abnormalities
  • Ear Canal
  • Female
  • Humans
  • Male
  • Retrospective Studies

Supplementary concepts

  • Aural Atresia, Congenital