Staging and management of primary cerebellopontine cholesteatoma.

Primary cerebellopontine angle (CPA) cholesteatoma grows slowly and silently in the subarachnoidal spaces. The diagnosis is often late, when the lesion has reached large dimensions. Surgical removal is the only available therapy. Fifteen consecutive cases of CPA cholesteatoma managed at a tertiary otoneurosurgical referral unit between September 1985 and April 1999 were reviewed. The study population, consisting of seven males and eight females, had a mean age of 44 years of age (range 21-69) at the time of surgery. The clinical, audiological and radiological presentations were examined. The tumours were classified according to the Moffat classification of CPA cholesteatomas. In 67 per cent of cases the presenting symptom was related to the vestibulo-cochlear nerve. The average duration of symptoms was 23 months (ranging from one month-10 years). The hearing preservation approaches were utilized the most (11 cases), while the translabyrinthine approach alone, or in association with a middle fossa craniotomy, was performed in four cases. Tumour removal was total in 12 cases and subtotal in three cases. In cases undergoing hearing preservation surgery the mean pre-operative pure tone average (PTA) for the frequencies 0.5, 1, 2, and 3 kHz was 19.3 dB HL (SD 13.84) and the mean pre-operative speech discrimination score (SDS) was 89.8 per cent (SD 5.97). In 44.4 per cent of patients the hearing was preserved and the mean post-operative PTA was 20.29 dB HL (SD 15.84). In five patients post-operative complications occurred. No peri- or post-operative death occurred in this series, one patient developed a recurrence 15 years after the initial surgery.