Endaural Laser-Assisted Single-Stage Inside-Out Cholesteatoma Surgery (LASIC) to Treat Advanced Congenital Cholesteatoma.

OBJECTIVES: Investigation of endaural laser-assisted single-stage inside-out cholesteatoma surgery (LASIC) to treat advanced congenital cholesteatoma (ACC) by a modified staging system based on ossicle status. STUDY DESIGN: A retrospective case review. SETTING: A university hospital otology referral clinic. PATIENTS: Two hundred consecutive pediatric patients with ACC were enrolled. INTERVENTIONS: Endaural LASIC and postoperative temporal bone computed tomography (CT). MAIN OUTCOME MEASURES: Residual or recurrent CC and audiological outcomes. RESULTS: LASIC was feasible in 98.0% of patients. Single-stage ossiculoplasty was performed in 95.5% of patients. Hearing preservation to less than 20 dB was 59.2% and to the preoperative hearing level was 84.5%. Mastoid invasion did not result in worse recidivism or hearing loss (HL) but further invasion of the stapes superstructure (stage IV) significantly elevated both the recidivism (16.7%) and the risk for HL (to 84.8%) (p = 0.001). Ossicle preservation LASIC was frequently possible in stage III posterior type (75.6%), whereas it was rarely possibly (15.4%) in the anterior type. Incudostapedial joint (ISJ) invasion in the absence of cochleariform process (CP) invasion (III-posterior) did not increase the incidence of HL (6.1%) or recidivism (2.4%). However, simultaneous invasion of the CP and ISJ (III-anterior) elevated the risk of HL by 46.2% by ossicle removal, although recidivism was not increased (3.8%). CONCLUSIONS: Endaural LASIC for ACC achieved satisfactory recidivism (overall 7.5%, 16.7% in stage IV) comparable to early CC (17.2%). An ossicle status-based staging system was more efficient for correlation with audiologic and surgical outcomes of CC than that of mastoid invasion.

Bilateral Congenital Cholesteatoma.

OBJECTIVE: To report the first large case series of extremely rare bilateral congenital cholesteatoma (CC). STUDY DESIGN: A retrospective cohort study. SETTING: University hospital otology referral clinic. PATIENTS: Six hundred four children with surgically confirmed CC. MAIN OUTCOME MEASURES: The bilateral CCs were compared with the unilateral CCs. RESULTS: The incidence of bilateral CC was 3.6% (22/615) per case or 1.8% (11/604) per child. Bilateral CC did not differ from unilateral CC regarding its demographics, and invasiveness by the proportion of advanced CC as 31.8% (7/22) versus 28.2% (167/594). But the invasiveness or location randomly differed between the ears, that advanced CC per child was higher as 45.4% (5/11). Bilateral exploration was attempted with a concern for hearing loss, which featured a combination of laser myringotomy to treat early CC and endaural laser-assisted single-stage inside-out cholesteatoma surgery to treat advanced CC. Bilateral exploration was difficult in three children with initially negative otoendoscopy. Among the seven advanced CC, proportion of anterior type was 71.4% (5/7), who all exhibited more than 20 dB HL, but two posterior type retained normal hearing. Therefore, bilateral advanced CCs of anterior origin showed poorest hearing outcome as bilateral more than 20 dB HL, which were in two children. Six second-look operations and one third-look operation were required to treat six residual CCs (30%) in four children (40%), including bilateral residual CC in two (20%); such reoperations were significantly more frequent than in unilateral CC. CONCLUSION: The diagnosis of bilateral CC required high index of suspicion from TBCT, and early bilateral exploration.