Citation, DOI & article data
Cholesteatomas are histologically-equivalent to an epidermoid cyst and are composed of desquamated keratinizing stratified squamous epithelium forming a mass.
They may be asymptomatic, or may present with conductive hearing loss, dizziness, or otorrhea.
The mass is lined by epithelium (facing inwards) which continues to grow, thereby shedding additional cells into the mass. Their cholesterol content (which is not always present) is responsible for their name, although 'keratoma' is probably a more apt term.
Cholesteatomas of the temporal bone and middle ear can be divided into:
- congenital cholesteatoma: accounting for only 2%
- acquired cholesteatoma: 98%
- external ear canal cholesteatoma
- mural cholesteatoma
- petrous apex cholesteatoma
A CT scan is an important component of assessing patients with possible cholesteatoma. It is not as specific as MRI but is able to obtain excellent bony details required for preoperative planning (reconstruction of ossicles if needed) and to exclude perforation of the bony tegmen.
The pars flaccida cholesteatoma originates in Prussak space and usually extends posteriorly, while the pars tensa cholesteatoma originates in the posterior mesotympanum and tends to extend posteromedially.
Conventional non-contrast MR imaging with diffusion-weighted imaging is recommended in all patients with suspected cholesteatoma. An MRI should be performed especially in patients with previous surgery for cholesteatoma since recurrence or residual tumor can be detected with great accuracy, whereas appearances on CT can be non-specific. If negative, it can obviate "second look" surgery. Ideally, patients should be prepared for the examination (clear the external auditory canal or the postoperative cavity) to avoid a false-positive diagnosis.
A typical examination comprises T2-weighted series in the coronal and axial planes and a non-echo planar DWI series (b-values 0, 1000). On T2-weighted images they appear hyperintense. On the b= 1000 images, cholesteatomas appear hyperintense with low values on ADC map similar to that of brain parenchyma (note, the signal characteristics are the same as intracranial epidermoid cysts).
With these findings, recurrent cholesteatomas as small as a few millimeters can be detected with near 100% specificity.
Treatment and prognosis
Generally, surgical excision is eventually necessary 9. Excision is often performed with tympanoplasty ± mastoidectomy ± ossicular reconstruction, depending on extent of disease 9.
The main differentials, which unlike cholesteatoma show a high signal on the ADC map, are:
- cholesterol granuloma
Further differential diagnoses include:
- cerumen: which shows similar image characteristics to cholesteatoma but is located in the external ear canal
- abscess formation in the middle ear: can also show similar imaging findings but has a completely different clinical presentation
Important CT features to comment on when reporting a cholesteatoma:
- erosions of the
- dehiscence of the
- the integrity of the
- the presence of cholesteatoma in the sinus tympani (which is the most hidden recess of the middle ear): to avoid residual disease
- 1. Swartz JD, Loevner LA. Imaging of the Temporal Bone. Thieme Medical Pub. (2008) ISBN:1588903451. Read it at Google Books - Find it at Amazon
- 2. Dubrulle F, Souillard R, Chechin D et-al. Diffusion-weighted MR imaging sequence in the detection of postoperative recurrent cholesteatoma. Radiology. 2006;238 (2): 604-10. doi:10.1148/radiol.2381041649 - Pubmed citation
- 3. De foer B, Vercruysse JP, Bernaerts A et-al. Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection. Radiology. 2010;255 (3): 866-72. doi:10.1148/radiol.10091140 - Pubmed citation
- 4. Lingam RK, Bassett P. A Meta-Analysis on the Diagnostic Performance of Non-Echoplanar Diffusion-Weighted Imaging in Detecting Middle Ear Cholesteatoma: 10 Years On. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 38 (4): 521-528. doi:10.1097/MAO.0000000000001353 - Pubmed
- 5. Yung M, Tono T, Olszewska E, Yamamoto Y, Sudhoff H, Sakagami M, Mulder J, Kojima H, İncesulu A, Trabalzini F, Özgirgin N. EAONO/JOS Joint Consensus Statements on the Definitions, Classification and Staging of Middle Ear Cholesteatoma. The journal of international advanced otology. 13 (1): 1-8. doi:10.5152/iao.2017.3363 - Pubmed
- 6. Tono T, Sakagami M, Kojima H, Yamamoto Y, Matsuda K, Komori M, Hato N, Morita Y, Hashimoto S. Staging and classification criteria for middle ear cholesteatoma proposed by the Japan Otological Society. Auris, nasus, larynx. 44 (2): 135-140. doi:10.1016/j.anl.2016.06.012 - Pubmed
- 7. Rosito LS, Netto LF, Teixeira AR, da Costa SS. Classification of Cholesteatoma According to Growth Patterns. JAMA otolaryngology-- head & neck surgery. 142 (2): 168-72. doi:10.1001/jamaoto.2015.3148 - Pubmed
- 8. Rutkowska J, Özgirgin N, Olszewska E. Cholesteatoma Definition and Classification: A Literature Review. The journal of international advanced otology. doi:10.5152/iao.2017.3411 - Pubmed
- 9. Hunter JB, Zuniga MG, Sweeney AD, Bertrand NM, Wanna GB, Haynes DS, Wootten CT, Rivas A. Pediatric Endoscopic Cholesteatoma Surgery:. (2016) Otolaryngology–Head and Neck Surgery. 154 (6): 1121-7. doi:10.1177/0194599816631941 - Pubmed