Abstract
Congenital cholesteatoma (CC) is a non-neoplastic lesion of keratin debris lined by epithelium found in the temporal bone. It is the lesser-known sibling of the acquired cholesteatoma and may be classified as congenital middle ear cholesteatoma and congenital petrous bone cholesteatoma. The incidence is rising, probably owing to increased recognition and advances in imaging modalities. Cone beam CT provides detailed anatomical information, highlighting quadrant location, ossicular involvement, and mastoid extension. MRI aids in lesion characterization and detection of complications. The classification systems for congenital middle ear and petrous bone cholesteatoma are helpful in the preoperative workup and have a role in predicting postoperative recurrence rates. Management almost invariably involves surgical intervention aimed at preserving middle and inner ear function. Follow-up of CC is mainly based on MRI together with otoscopic examination. Non-echo planar diffusion-weighted imaging, especially, has proven essential for detecting residual disease. This review article emphasizes the significance of imaging in the timely diagnosis and management of CCs. CLINICAL RELEVANCE STATEMENT: This article underscores the crucial role of imaging for prompt detection, preoperative assessment, and postoperative follow-up of CCs, a condition with rising incidence associated with potentially severe complications. KEY POINTS: Timely diagnosis of CCs is imperative for avoiding complications. Imaging is key in detection, preoperative evaluation, and postoperative management. Cone Beam CT and non-echo planar DWI represent state-of-the-art imaging techniques.