Totally Endoscopic Surgery for Congenital Cholesteatoma

By June 7, 2019

Purcell, Patricia1; James, Adrian1; Federici, Gaia2; Villari, Domenico2; Presutti, Livio2
1 Department of Otolaryngology – Head & Neck Surgery, Hospital for Sick Children, University of Toronto, Canada
2 Otolaryngology – Head & Neck Surgery Department, University Hospital of Modena, Modena, Italy

This study assesses the feasibility and outcomes of trans-canal totally endoscopic ear surgery (TEES) for congenital cholesteatoma over 10 years of experience.

– Study design: 
Retrospective case series
-Setting: Two tertiary referral centers
– Patients: 35 children with congenital cholesteatoma without mastoid involvement; mean age 7 years (range 1.8 – 16 years; eight children <4 years age) and mean follow up 4.5years (range 0.5 – 10 years).
– Interventions: Cholesteatoma removal using TEES

– Main outcome measures: Cholesteatoma extent according to EAONO/JOS and Potsic stage; residual or recurrent cholesteatoma; post-op hearing outcome (4-tone average pure tone audiogram (PTA)) in children old enough for PTA (3 ears excluded).

Cholesteatoma was localized to one subsite of the middle ear (EAONO/JOS Stage 1) in 10 (29%) cases. More extensive cases (EAONO/JOS Stage 2) were more likely to be associated with ossicular erosion (Potsic Stage 3) (18/24 versus 1/10 (p=0.001, Fisher Exact test)). No cases of residual cholesteatoma occurred. Two cases of “recurrent” (i.e. newly acquired) cholesteatoma occurred (6%). Post-op air conduction hearing thresholds were normal (<25dB HL) in all 11 EAONO/JOS or Postic Stage 1 cases and 5/21 (24%) higher Stage cases (mean 15 vs 36dB HL respectively; p<0.001 t-test). Bone conduction was unchanged (<5dB).

The TEES approach is feasible and effective for removal of congenital cholesteatoma that does not extend into the mastoid. Better hearing results are obtained with less extensive disease, but even larger disease is associated with very low rates of recidivism using TEES.  As minimally-invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.