Congenital Cholesteatoma Isolated to the Mastoid

Congenital cholesteatoma isolated to the mastoid presenting as stricture of the external auditory canal

“…The most common site of origin is the middle ear cavity, and the most rare is the mastoid process [2][3][4][5][6][7][8][9]. Moreover, congenital cholesteatoma isolated to the mastoid, not involving the middle ear, is very rare.…”

“…Moreover, congenital cholesteatoma isolated to the mastoid, not involving the middle ear, is very rare. From the worldwide literature [2][3][4][5][6][7][8][9][10], we were able to identify only 14 cases of congenital cholesteatoma isolated to the mastoid, including the present case. Warren et al [8] evaluated a series with mastoid congenital cholesteatoma and reported that 5 of 9 cases were isolated to the mastoid.…”

“…CT and MRI are invaluable in the diagnosis of congenital cholesteatoma, particularly that isolated to the mastoid [3]. CT shows a nonenhancing, expansile soft-tissue mass with varying degrees of bony erosion.…”

“…CT shows a nonenhancing, expansile soft-tissue mass with varying degrees of bony erosion. Thakkar et al [3] noted that congenital cholesteatoma isolated to the mastoid on CT could be confused with cholesterol granuloma, anomalous sigmoid sinus, or neoplasm. On MRI, cholesteatoma is hypo-or isointense to the brain on T1-weighted imaging and hyperintense on T2-weighted imaging.…”

“…Congenital cholesteatoma is an epidermoid cyst arising from congenital remnants of keratinizing squamous epithelium in the temporal bone, although the etiology remains controversial [2][3][4]. The most common site of origin is the middle ear cavity, and the most rare is the mastoid process [2][3][4][5][6][7][8][9].…”