Congenital middle ear cholesteatomas in children: our experience in 34 cases.

Objectives: To evaluate clinical data, extensions, residual disease rate, and functional results in cases of congenital cholesteatoma in pediatric patients compared with cases of acquired forms.

Study Design And Setting: In a retrospective study conducted at a single tertiary care center over a decade, 34 congenital cholesteatomas (mean patient age, 6.6 years) isolated from a series of 215 cholesteatomas in children were treated surgically and followed up for an average of 83 months. Surgical treatment consisted of the closed technique (CT), open technique tympanoplasty in CT [TOT], radical mastoidectomy (RM), or Rambo's technique. The main outcome measures were surgical findings, residual lesion rate, and hearing assessment.

Results: The first surgical procedure was CT in 85.3%, TOT in 8.8%, RM in 2.9%, and Rambo's technique in 2.9% of patients. Two, 3, and 4 operations were necessary in 76.5%, 11.4%, and 11.4% of children, respectively. Residual lesions were observed in 34.6% of those multi-operated patients (7 CT and 2 TOT), 24.1% in those treated with CT, and 29.2% in those treated with CT with a planned second look. Mean postoperative pure tone average and air-bone gap were 26 and 21 dB, respectively. A speech reception threshold of less than 30 dB HL was achieved in 66% of patients. A total of 26 ossiculoplasties had to be performed (8 partial and 18 total); the mean postoperative air-bone gap was lower after the former (15 dB) than after the latter (22 dB). A high-frequency pure-tone hearing loss of more than 10 dB was uncommon (5.8%). Unsurprisingly, the residual lesion rate seems to be higher for congenital than for acquired cholesteatomas, but hearing results are significantly better.

Conclusion: In most cases, staged CT appears to be the best technique to treat these lesions, which often develop in a well-pneumatized mastoid.