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Updated: Aug 16, 2026
Endoscopic Cholesteatoma Surgery
Published on: January 19, 2022
[Long term results of congenital middle ear cholesteatoma in children]
A Benhammou1, D-Q Nguyen, K El Makhloufi
1Service d'ORL, CHU de Grenoble, BP 217, 38043 Grenoble Cedex 9.
Insights
Congenital cholesteatoma in children requires early detection and treatment. The intact canal wall technique offers a conservative approach with satisfactory long-term functional results when diagnosed early.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Medical Research
Context:
- Congenital cholesteatoma (CC) is a rare but aggressive middle ear disease in children.
- Early diagnosis is crucial for effective management and optimal outcomes.
- Atypical otitis media with effusion and unilateral hearing loss are key indicators.
Purpose:
- To evaluate the long-term surgical and functional outcomes of congenital cholesteatoma in pediatric patients.
- To assess the efficacy of the intact canal wall (ICW) technique in treating pediatric CC.
- To emphasize the importance of early detection and multidisciplinary follow-up.
Summary:
- A retrospective study analyzed 32 children with 33 CC cases, with a mean age of 6 years.
- The intact canal wall technique, often requiring two stages, was employed in most cases.
- Functional success, defined by a 5-year postoperative air-bone gap ≤ 20 dB HL, was achieved in 50% of patients.
Impact:
- The ICW technique is recommended for pediatric CC due to its conservative nature and effectiveness.
- Early diagnosis by ENT and pediatric physicians significantly improves functional results.
- This study highlights the need for heightened clinical suspicion and long-term surveillance for CC in children.
Objectives:
To report the long term results of congenital cholesteatoma of the middle ear in children.
Patients And Methods:
Retrospective study of thirty two children with 33 congenital cholesteatoma (1 bilateral cholesteatoma) operated on by the same surgeon. The mean age was 6 years. The most common clinical presentation was unilateral hearing loss. A trans-canal approach was performed in 4 cases and an intact canal wall technique in 29 cases. A two staged surgery was necessary in 28 patients, whom a residual cholesteatoma was observed in 7 patients. A third stage was performed in 11 patients because of a residual cholesteatoma in 2 two cases and a functional failure in 9 cases.
Results:
At five years postoperative audiometry (air bone gap inferior or equal to 20 dB HL), a functional success was obtained in 50% of cases.
Conclusion:
The diagnosis of congenital cholesteatoma must be evoked in every atypical otitis media with effusion or unilateral transmission hypoacusis. The intact canal wall technique in two stages is the most appropriate treatment, since congenital cholesteatoma in children appears aggressive in a well pneumatized mastoid. The choice for an intact canal wall technique is also justified by the ambition of a conservative surgery in the young child. The functional results are generally satisfactory in early diagnosed cases. Thus, ENT and pediatric physicians have a unique role in detecting congenital cholesteatoma in the early period of life, and in ensuring a long term follow-up.
