The surgical management of labyrinthine fistulas caused by cholesteatoma remains controversial. Forty cases (41 ears) of labyrinthine fistulas were reviewed. This represented 10% of our total series of cholesteatomas in adults and children (426 ears). Clinical presentation, extent of disease, results of fistula testing and audiometric studies, and radiographic findings were analyzed. A canal wall-down procedure was performed in all but one patient. Generally an attempt was made to completely remove the cholesteatoma, to graft the fistulous area, and to reconstruct the middle ear mechanism in one stage. The matrix was preserved in patients with large fistulas where the involved ear was the only hearing one, when the matrix was adherent to the underlying optic duct, and in selected elderly persons. Long-term followup did not reveal a significant difference in hearing, degree of vertigo, or incidence of recidivism when those patients in whom the matrix was removed were compared with those in whom the matrix was preserved. The importance of recognizing the presence of a labyrinthine fistula preoperatively is stressed, along with the need to be prepared for an unexpected fistula. Operative management is described.

We report our experience with a one-stage surgery for pediatric cholesteatoma in 216 ears. Our technique is based on three main principles: (1) the surgery is individualized; (2) the goal of surgery is to completely remove cholesteatoma and related disease in one operation; and (3) the reconstruction is performed to provide both good hearing and a dry, trouble-free ear. The incidence of recidivism was 10.2%, and the rate achieved was 13.3% at 5 years and 24% at 10 years. Canal wall down surgery was the predominant procedure used. The incidence of intraoperative neurosensory hearing loss, vertigo, and facial nerve injury was extremely low. The postoperative cavity problems encountered were minimal.

One hundred twelve patients (116 ears) were treated for recurrent and residual cholesteatoma. A retrospective review revealed that 66% had undergone canal wall down mastoidectomy at the previous surgery. The surgical procedure at revision was selected on the basis of an intraoperative assessment of the extent of disease, and clinical prediction of eustachian tube function. The average period of follow-up was 3.4 years. Revision surgery was successful in providing the patient with a safe, dry ear in 105 (91%) of 116 cases. Surgical principles and hearing results are presented.

Cholesteatoma is a destructive process involving an accumulation of desquamated keratin arising from squamous epithelium that pathologically has invaded the middle ear or mastoid process. The clinical hallmarks of cholesteatomas, namely invasion of healthy tissues, migration, unrestrained proliferation, aggressiveness, recidivism, and uncoordinated differentiation predict the existence of defects in the normal biology and biochemistry of the cellular constituents that compose a cholesteatoma, as well as in the cellular interactions between these cells, the surrounding normal tissue, and the host. In the current report, we analyzed 11 cholesteatomas and matched healthy tissue for altered expression in four different cell surface peptidases, aminopeptidase A, aminopeptidase N, dipeptidyl peptidase IV, and neutral endopeptidase. We suggest that peptidases may modulate cell growth and differentiation by inactivating stimulatory signals (or conversely, by activating inhibitory signals).