Congenital Cholesteatoma Presenting as a Post-Auricular Fistula

Congenital middle ear cholesteatoma is a keratinizing squamous epithelial cyst that classically presents as a white 'pearl' in either the anterosuperior or posterosuperior quadrants behind an intact tympanic membrane. The presenting features of congenital cholesteatoma depend upon the initial site of the disease. The most common presentation of this disease is a conductive hearing loss or a facial palsy of gradual onset. Other presentations include incapacitating vertigo with nystagmus, a feeling of fullness in the ear, frequent otalgia, an abnormal eardrum noted at physical examination for an unrelated condition and meningitis. Recently, we experienced a case of congenital cholesteatoma that was presented as a post-auricular fistula. In considering the management of such a fistula, it would be important to recognize the possibility that the disease may present in this way.