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Updated: Feb 14, 2026
Endoscopic Cholesteatoma Surgery
Published on: January 19, 2022
Challenges and outcomes of cholesteatoma management in children with Down syndrome
Saied Ghadersohi1, Bharat Bhushan2, Kathleen R Billings2
1Northwestern University Feinberg School of Medicine, Department of Otolaryngology-Head and Neck Surgery, Chicago, IL, United States.
Insights
Children with Down syndrome (DS) diagnosed with cholesteatoma often present with ear infections and hearing loss. Management involves surgery, with many requiring hearing aids post-operatively.
Area of Science:
- Otolaryngology
- Genetics
- Pediatric Surgery
Background:
- Children with Down syndrome (DS) have a higher risk of chronic otitis media and Eustachian tube dysfunction, increasing cholesteatoma susceptibility.
- Diagnosing and managing cholesteatoma in this population presents unique challenges.
Purpose of the Study:
- To outline strategies for diagnosing, surgically treating, and managing cholesteatoma in pediatric patients with Down syndrome.
Main Methods:
- A retrospective case series analyzed 14 patients (17 ears) with Down syndrome and cholesteatoma over 9 years.
- Data included presenting symptoms, pre-operative imaging, surgical approaches, and post-operative outcomes.
Main Results:
- Otorrhea and hearing loss were common symptoms; CT scans often showed mastoid sclerosis and ossicular erosion.
- While canal wall up (CWU) was initially favored, 40% ultimately required canal wall down (CWD) procedures.
- Post-operative complications included retraction pockets and recurrent disease; 70.6% of ears were fitted with hearing aids or FM systems.
Conclusions:
- Cholesteatoma in Down syndrome is linked to otorrhea, hearing loss, and specific CT findings.
- CWU surgical approaches were common, but CWD was often necessary.
- Post-operative hearing rehabilitation, including hearing aids, is frequently required.
Introduction:
The high incidence of chronic otitis media with effusion and Eustachian tube dysfunction in children with Down syndrome (DS) may predispose them to cholesteatoma formation. Establishing the diagnosis, choosing the appropriate operative intervention, and post-operative care can be challenging.
Objective:
To describe management strategies for cholesteatoma diagnosis, surgical treatment, and post-operative management in children with Down syndrome.
Methods:
Retrospective case series of 14 patients (17 total ears) with Down syndrome diagnosed with cholesteatoma over a 9-year period.
Results:
A total of 14 patients with cholesteatoma (3 with bilateral disease) were analyzed. Thirteen ears (76.5%) had ≥2 tympanostomy tubes insertions prior to cholesteatoma diagnosis, and otorrhea and hearing loss were the most common presenting symptoms. Common pre-operative CT scan findings included mastoid sclerosis and ossicular erosion. The average age at first surgery was 9.8 years, and the average follow-up was 4.3 years. For acquired cholesteatoma, most ears were managed with canal wall up (CWU) approaches, but ultimately 6/15 (40.0%) required canal wall down (CWD) approaches. Postoperatively, 3 (20.0%) ears developed new tympanic membrane retraction pockets, but no recurrent cholesteatoma. Four (26.7%) ears developed recurrent disease, and 3 (20.0%) had residual disease at secondary procedures. Ossiculoplasty was performed in 4 ears. Twelve (70.6%) ears were rehabilitated with hearing aids or FM systems.
Conclusions:
The diagnosis of cholesteatoma in Down syndrome was associated with otorrhea, hearing loss, and CT scan findings of ossicular erosion and mastoid sclerosis. Most cases were managed with CWU surgical approaches. Hearing aid use was common post-operatively.
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