Congenital cholesteatoma: predictors for residual disease and hearing outcomes.

Objective: To determine predictive factors for residual disease and hearing outcomes of surgery for congenital cholesteatoma (CC).

Design: Retrospective record review of surgery for CC from January 1, 1998, through December 31, 2010. The initial extent of CC was staged using the system as defined by Potsic et al.

Setting: Tertiary care children's hospital.

Patients: Eighty-one children (82 ears) underwent a total of 230 operations for CC. The mean (SD) age was 5.3 (2.9) years, and the mean follow-up was 4.3 years.

Intervention: Initial and subsequent operations for CC and audiologic evaluations.

Main Outcome Measures: Statistical analyses were performed to determine factors associated with increased residual disease for CC and poorer hearing outcomes.

Results: Higher initial stage of disease, erosion of ossicles, and removal of ossicles were significantly associated with increased likelihood of residual CC (46%, 50%, and 51%, respectively; P < .001). More extensive disease at initial surgery was associated with poorer final hearing outcomes (P < .05). Other significant findings included CC medial to the malleus (41.5%) or incus (54.3%), abutting the incus (51.3%) or stapes (63%), or enveloping the stapes (50%); all patients had increased residual disease (all P < .05). Excellent audiometric results (air-bone gap of ≤20 decibel hearing level) were obtained in 63 (77%) of the 82 ears.

Conclusions: More extensive initial disease, ossicular erosion, and the need for ossicular removal were associated with residual disease. On the basis of our data, the best chance for completely removing CC at initial surgery involves removing involved ossicles if they are eroded, if the CC is abutting or enveloping the incus or stapes, if the CC is medial to the malleus or incus, or if the matrix of the CC is violated. These results may help guide surgeons to achieve the best results for their patients.