Cholesteatoma is a non-neoplastic, keratinized squamous epithelial lesion that affects the temporal bone. The middle ear is the most frequent, while the isolated cholesteatoma of the mastoid is rar...

https://journals.sagepub.com/doi/pdf/10.1177/11795476211014032

Isolated Congenital Mastoid Cholesteatoma with no Involvement of Aditus Ad Antrum and Middle Ear.

Key Clinical Message Congenital mastoid confined cholesteatoma is a very rare entity with only 30 reported cases worldwide. We describe the presentation and treatment of this condition in a 14‐year‐old boy, with maintenance of normal hearing and ear canal function.

Congenital mastoid‐isolated cholesteatoma in a 14‐year‐old boy

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. 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. 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. 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.

Beyond the otoscope: an imaging review of congenital cholesteatoma

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Isolated Congenital Mastoid Cholesteatoma with no Involvement of Aditus Ad Antrum and Middle Ear.