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Joseph Junewick, MD FACR
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Congenital Cholesteatoma

Case Detail

Anatomy: Neck-Face
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 3 years ago
Tags: PEDS
Modality/Study Types: CT
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3 year old male with pearl white tympanic membrane noted on well-child examination.

Case Images


Congenital Cholesteatoma


CT – Noncontrast axial and coronal images of the left temporal bone show a small rounded soft tissue mass medial to the ossicular chain (contiguous with the cochlear promontory and malleus) but not associated with ossicular erosion.


Congenital cholesteatomas are usually clinically asymptomatic and may occur anywhere in the temporal bone – middle ear cavity, mastoid air cells, petrous apex and the squama. Macroscopically, cholesteatomas are pearly gray or yellow circumscribed lesions. The pathophysiology is thought to be related to persistent fetal epithelium, epidermoid cyst equivalent. Ossicular erosion is unusual with congenital cholesteatomas or occurs late in the course. Congenital cholesteatomas can be associated with external auditory atresia and branchial cleft remnants.


Barth K, Huber AM, Stampfli P, et al. Neuroradiology of cholesteatomas. AJNR (2011); 32:221-229.

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