CONGENITAL CHOLESTEATOMA: RADIOLOGIC EVALUATION AND PERSONAL EXPERIENCE

S. Armağan Incesulu

Eskisehir Osmangazi University, Faculty of Medicine, Department of Otolaryngology-Head&Neck Surgery, Meselik-Eskisehir, Turkey

The congenital origin of cholesteatoma has been hypothesized in 1800. Congenital cholesteatoma of the petrous portion of temporal bone was reported in 1938. Congenital cholesteatoma (CC) may locate in petrous portion of the temporal bone or in the middle ear. CC in the middle ear can be defined as a whitish mass in the middle ear with the intact tympanic membrane that arises from embryonal inclusions or rests of epithelial cells. Previous otologic procedure, otorrhea and perforation and abnormalities on the middle ear are exclusion criteria to diagnose middle ear cholesteatoma. It is the most frequent type and the symptom of conductive hearing loss appears in the young age, however, it can be seen in every age. Awareness of the problem is the main reason for an early diagnosis. Moreover, having the better diagnostic tools such as endoscopes, better otoscopes and otomicroscopes as well as better radiologic evaluation make the diagnosis easier. High-resolution computed tomography (CT) and magnetic resonance imaging (MRI) are the main radiological evaluation methods of congenital cholesteatoma. A CT scan confirms the location as well as the size of middle ear cholesteatoma. It gives us a lot of details for surgery, however, it is not enough to define any mass in the middle ear or cranium as cholesteatoma. All the findings are indirect. Moreover, the CT scan produces a high dose of radiation. This is especially harmful for young children, which is why the cone-beam CT scan has recently become the recommended modality. MRI shows a homogenous lesion that is hypo-intense in T1-weighted images and hyper-intense in T2-weighted images. A delayed contrast enhanced MRI sequence is another type of evaluation. However, it is time consuming and requires repeated scanning 30–120 minutes after contrast administration. Diffusion-weighted imaging (DWI) detects the molecular diffusion of water within the scanned tissue. Cholesteatoma exhibits a high-signal intensity on a DWI. The sensitivity and specificity of DWI has been found 69.4% and 92.8%, respectively in a recent study.1 Between 2006 and 2012, six patients with congenital cholesteatoma were seen. Details of them can be found in Table 1. High-resolution CT scan and MRI were used for diagnosis. The MRI machine in the hospital has some major limitations such as low resolution and the production of relatively thick image sections. They have distorted shapes due to artifacts. Since it does not give us excellent details, we asked for a baseline MR scan after the first operation for follow up. Except for one patient, all cases presented with extensive cholesteatoma in the temporal bone. Fullness was the main symptom in the case with CC in the middle ear and trans-canal surgical management has been performed. The mass was removed without touching the ossicular chain. One patient has been diagnosed incidentally. Another one presented with recurrent facial palsy, while other patients complained about hearing loss or dizziness. Trans-mastoid surgery was performed. Congenital cholesteatoma may originate in different sites within the temporal bone. There are various symptoms. According to the location, different surgical techniques can be performed. Intensive and long-term follow up is mandatory for this group of patients.

Table 1. Details of patients

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Reference

1.Ganaha A, Outa S, Kyuuna A, Matayoshi S, Yonaha A, Oyadomari M, Miyara T, Tono T, Suzuki M. Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma. Auris Nasus Larynx 38(3):329–334, 2011


Address for correspondence: Prof. Dr. S. Armagan Incesulu, Eskisehir Osmangazi University, Department of Otolaryngology-Head& Neck Surgery, Meselik-Eskisehir, Turkey. armaganincesulu@yahoo.com

Cholesteatoma and Ear Surgery – An Update, pp. 137–138

Edited by Haruo Takahashi

2013 © Kugler Publications, Amsterdam, The Netherlands