Related Experiment Video
Updated: Jul 23, 2025
Robot-Assisted Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma
Published on: December 15, 2023
Pediatric cholesteatoma associated with congenital aural atresia and stenosis
Olivia Kalmanson1, Christian Francom1, Owen Darr1
1Department of Otolaryngology, Children's Hospital Colorado, 13123 E 16th Ave, Aurora, CO 80045, United States; Department of Otolaryngology, The University of Colorado School of Medicine, 12631 E 17th Ave, Aurora, CO 80045, United States.
Insights
Fewer than 5% of pediatric patients with congenital aural atresia or stenosis develop cholesteatoma. Revision surgery is frequently needed for these cases, highlighting the importance of early screening.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Medical Imaging
Background:
- Congenital aural atresia (CAA) or stenosis (CAS) are conditions affecting the ear canal.
- Cholesteatoma is an abnormal skin growth in the middle ear.
Purpose of the Study:
- To investigate the characteristics, surgical management, and outcomes of cholesteatoma in pediatric patients with CAA or CAS.
- To determine the incidence and recurrence rates of cholesteatoma in this patient population.
Main Methods:
- A retrospective chart review was conducted at a tertiary care children's hospital.
- Data from pediatric patients diagnosed with CAA or CAS and associated cholesteatoma between 2003 and 2018 were analyzed.
Main Results:
- Canal cholesteatoma was identified in 4.3% of pediatric patients with CAA or CAS.
- The majority of patients (75%) had conductive hearing loss.
- Over 50% of patients required revision surgery due to cholesteatoma recurrence.
Conclusions:
- Acquired canal cholesteatoma is uncommon in pediatric patients with CAA/CAS.
- High rates of cholesteatoma recidivism necessitate frequent revision surgeries.
- Imaging screening for cholesteatoma in patients with CAA/CAS is recommended to prevent delayed diagnosis and associated complications.
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.
