Learning Objectives:

Objectives: We analyze the clinical presentation, imaging findings, and surgical treatment of 10 patients with acquired cholesteatoma with labyrinthine destruction, skull base erosion, or intracranial extension.

Study Design: A retrospective case series at a tertiary referral center.

Methods: From 1997 to 2015, 6 males and 4 females, age 12 to 73 (mean, 42.3), years were treated for acquired cholesteatoma with skull base or labyrinthine invasion. Two patients had no prior surgery while 8 had an average of two prior procedures. All 10 complained of hearing loss, 3 had otorrhea, 3 had acute facial palsy, 3 had otalgia and 2 presented with progressive imbalance. Follow-up ranged from 8 to 216 (mean, 76.6) months. Audiometric, CT and MRI findings are compared to intraoperative outcomes.

Results: At surgical exploration, 2 patients had cholesteatoma with destruction of the cochlea, 4 had skull base invasion and 4 had intracranial involvement. Five patients required temporal bone obliteration, 2 had radical cavities with exteriorization of the petrous apex, and 3 required modified radical cavities. One patient with VII palsy recovered to HB grade III. One patient with labyrinthine destruction maintained residual hearing post op. No patients had additional complications from their definitive surgical procedures.

Conclusions: Acquired cholesteatoma with labyrinthine destruction, skull base extension, and intracranial involvement can have surprisingly subtle presentations. Balancing disease exteriorization with preserving labyrinthine function requires prudent radiological workup and surgical planning. Disease eradication is often not possible. Long-term clinical follow-up with periodic imaging and aggressive debridement is often necessary for disease control.