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Otology & Neurotology 00:00Y00 2007, Otology & Neurotology, Inc.

Imaging Case of the Month

Bilateral Congenital Cholesteatoma

Enrico Pasanisi, Filippo Di Lella, Andrea Bacciu, Vincenzo Vincenti, and Salvatore Bacciu
` Sezione di Otorinolaringoiatria e Microchirurgia Otologica, Universita di Parma, Parma, Italy

Congenital cholesteatoma is a rare pathologic condition defined as the presence of an epidermal cyst behind an intact tympanic membrane with no history of ear infections, head trauma, or otologic procedures. Michaels (1) theorized its origin from embryonal remnants of squamous epithelium in the region of the anterior mesotympanum. Clinical observations confirm the fact that most congenital cholesteatomas arise in the anterosuperior quadrant; congenital cholesteatomas have also been reported occupying the posterior quadrant, the mastoid, and the petrous apex, thus questioning the adequacy of Michaels theory (2). Bilateral congenital cholesteatomas have been estimated to represent less than 3% of all congenital cholesteatomas (3), and to date, less than 15 cases have been reported in the international literature. In the reported case, both ears are involved by a similar pathology: clinical and computerized tomographic (CT) scan findings document, in fact, the presence of a mass lesion in both ears in the anterior mesotympanum and in contact with the handle of the malleus; the lesions appear as perfectly delimited spherical masses isodense to the soft tissues on high-resolution CT of the temporal bones (4). Imaging modality of choice for cholesteatomatous chronic otitis media and congenital cholesteatomas is represented by high-resolution CT scan of the temporal bones with bone window in the axial and coronal planes (Figs. 1 and 2); CT scans allow fine tridimensional evaluation of the bony structures of the middle ear, the ossicles, and the intratemporal facial nerve. Differential diagnosis is usually made with inflammatory changes in the middle ear (i.e., effusion, cholesterol granuloma, and inflammatory soft tissue) after primary surgical procedures. When the diagnosis is not certain after otomicroscopic examination and CT scan, magnetic resonance imaging with gadolinium is advised as, because of the specific signal patterns of the cholesteatomatous lesions (low signal intensity in T1 images and high signal intenAddress correspondence and reprint requests to Filippo Di Lella, M.D., Sezione di Otorinolaringoiatria e Microchirurgia Otologica, ` Universita di Parma, Via Gramsci 14-43100 Parma, Italy; E-mail:

FIG. 1. Axial CT scan with bone window. A spherical mass isodense to soft tissue can be seen in the anterior mesotympanum of both ears in contact with the malleus handle.

FIG. 2. Coronal CT scan with bone window (reconstruction). The lesions appear as perfectly delimited and symmetrical.

sity in T2 images with no enhancement after the administration of contrast), it adds valuable information on the nature of the lesion. REFERENCES
1. Michaels L. Origin of congenital cholesteatoma from a normally occurring epidermoid rest in the developing middle ear. Int J Pediatr Otorhinolaryngol 1988;15:51Y65. 2. Parisier S, Levenson MJ, Edelstein DR, Bindra GS, Han JC, Dolitsky JN. Management of pediatric congenital cholesteatomas. Am J Otol 1989;10:121Y3. 3. Friedberg J. Congenital cholesteatoma. Laryngoscope 1994;104:S1Y24. 4. Nemzek WR, Swartz JD. Temporal bone: inflammatory disease. In: Som PM, Curtin HD, eds. Head and Neck Imaging. Vol. 2. St. Louis, MO: Mosby, 2003:1184Y95.

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