Cholesteatoma is histologically equivalent to an epidermoid cyst and is composed of desquamated keratinizing stratified squamous epithelium forming a mass. They usually present with conductive hearing loss.
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%
- primary (no history of chronic otomastoiditis)
- secondary ( the vast majority):
- pars flaccida
- pars tensa
- external ear canal cholesteatoma
- mural cholesteatoma
- petrous apex cholesteatoma
Conventional non-contrast MR imaging with diffusion-weighted imaging is recommended in all patients with a suspicion of cholesteatoma. An MRI should be performed especially in patients with previous surgery for cholesteatoma since recurrence or residual tumour can be detected with great accuracy. If negative, it can obviate "second look" surgery. It is important to prepare the patient for the examination (clear the external auditory canal or the postoperative cavity) to avoid a false positive diagnosis.
The standard examination is a T2-weighted series in the coronal and axial plane, followed by a non-echoplanar DWI series (b-values 0, 1000). On the DWI images with b-value 1000 s/mm2, a cholesteatoma becomes apparent as a hyperintense area. The signal intensity should be higher than visible on the DWI images with b-value 0 s/mm2. On the ADC map, low signal should be visible in the same area, confirming the presence of diffusion restriction.
A CT scan should be added in those cases where a cholesteatoma is detected with MRI. CT is required for preoperative planning (reconstruction of ossicles if needed) and to exclude perforation of the bony tegmen.
Pars flaccida cholesteatoma originates in Prussak's space and usually extends posteriorly.
Pars tensa cholesteatoma originates in posterior mesotympanum and tends to extend posteromedially.
- cholesterol granuloma
In contrast with cholesteatoma, these show high signal on the ADC map. With these findings, recurrent cholesteatoma can be detected with 100% specificity. Cholesteatomas down to a size of 2 mm can be detected with this technique on a 1.5T MRI machine.
Further differential diagnosis is to be made with:
- cerumen: which shows similar image characteristics to cholesteatoma but is located in the external ear
- abscess formation in the middle ear: can also show similar imaging findings but has a completely different clinical appearance
Important CT features to comment on when reporting a cholesteatoma:
- erosions of the
- dehiscence of the
- the integrity of the
- aditus ad antrum and mastoid antrum
- oval and round window
- 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