ABSTRACT
OBJECTIVE: To investigate the clinical symptoms, diagnosis and treatment of congenital petrous apex cholesteatoma. METHODS: A retrospective review of clinical symptoms, image findings and effect of operation in nine patients with congenital petrous apex cholesteatoma were performed. CLINICAL SYMPTOMS: two cases of congenital petrous apex cholesteatoma patients had peripheral facial paralysis, and progressively aggravated;four cases of peripheral facial paralysis were repeated, without hearing loss; three cases firstly appeared hearing loss, followed by paralysis. When hospitalized, nine cases of patients had peripheral facial paralysis and hearing loss. Seven patients were complicated with tinnitus, and four had vertigo. HRCT scans showed petrosal apex damage; MRI showed that petrosal apex was occupied;and the facial nerve electromyography and evoked potential examination showed moderate-severe neurogenic changes. All were treated by surgeries, among them, 8 cases were by middle fossa approach, 1 case was by middle cranial fossa-translabyrinth combined approach. Two cases whose labyrinthine segment of the facial nerve injured severely, were operated by end to end anastomosis; 3 cases with facial nerve compression thinning, were operated by facial nerve decompression; 4 cases were horizontal segment of facial nerve exposed and edema. None of them had recurrence in a follow-up of 6 to 32 months. 3 cases of peripheral facial paralysis were lessened in different degrees. CONCLUSIONS: Patients with congenital petrous apex cholesteatoma have peripheral facial paralysis in the early stage. Computed tomography scanning, magnetic resonance imaging and facial nerve imaging has important significance to clinical diagnosis, choosing the approach of operation and dealing with the facial nerve during operation.
