Abstract

Congenital cholesteatoma is rare, and especially so for a benign mass to grow from the squamous epithelium and the petrous apex as a location for this cancer. Surgery of the petrous apex cholesteatoma is challenging due to the location of the lesion and the need to preserve adjacent structures such as the dura mater, internal carotid artery, and internal auditory canal. Imaging studies, including CT and MRI, were crucial in guiding the surgical approach. This report describes a 40-year-old male patient with a large congenital cholesteatoma involving the petrous apex and temporal bone. The patient presented with a headache, hearing loss that persisted despite prior medical treatment. The cholesteatoma was surgically marsupialized through translabyrinthine approach, and the patient has remained stable for over 20 years. This report underscores the importance of considering the anatomical location and relationships of the lesion and adjacent structures when determining the surgical approach for congenital cholesteatoma involving the petrous apex and temporal bone.

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