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      Imaging in otosclerosis: A pictorial review

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          Abstract

          Otosclerosis is an otodystrophy of the otic capsule and is a cause of conductive, mixed or sensorineural hearing loss in the 2nd to 4th decades of life. Otosclerosis is categorised into two types, fenestral and retrofenestral. Imaging plays an important role in the diagnosis and management of otosclerosis. High-resolution CT (HRCT) of the temporal bone using 1-mm (or less) thick sections is the modality of choice for assessment of the labyrinthine windows and cochlear capsules. MRI has limited application in the evaluation of the labyrinthine capsules but is useful for assessment of the cochlear lumen prior to cochlear implantation in patients with profound hearing loss. The treatment of fenestral otosclerosis is primarily surgical with stapedectomy and prosthesis insertion. Patients with retrofenestral otosclerosis and profound hearing loss are treated medically using fluorides, but may derive significant benefit from cochlear implantation. This pictorial review aims to acquaint the reader with the pathology and clinical features of otosclerosis, the classical imaging appearances on CT and MRI, a radiological checklist for preoperative CT evaluation of otosclerosis, imaging mimics and a few examples of post-stapedectomy imaging and complications.

          Teaching points

          Otosclerosis causes conductive, sensorineural and mixed hearing loss in adults.

          HRCT of the temporal bone is the diagnostic imaging modality of choice.

          Stapedectomy is used to treat fenestral otosclerosis.

          Fluorides and cochlear implantation are used to treat retrofenestral otosclerosis.

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          Most cited references16

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          Otosclerosis: incidence of positive findings on high-resolution computed tomography and their correlation to audiological test data.

          Computed tomographic (CT) scanning with slices of 1 mm or more has not been sufficient to demonstrate otosclerotic foci in most cases to date. We investigated the validity of CT scans with a 0.5-mm cubical scan technique, with and without planar reconstruction, and correlated these findings with audiological data. Forty-four temporal bone CT scans from 30 patients with conductive or mixed hearing loss were evaluated. Otosclerotic foci were visualized in 74% of the cases. With reconstruction at the workstation, the sensitivity increased to 85%. Whereas in fenestral otosclerosis a correlation was found between the size of the focus and the air-bone gap, no correlation was seen between the size of the focus and bone conduction thresholds with cochlear involvement. Otosclerotic foci in patients treated with sodium fluoride were smaller than those in patients without treatment. This finding may indicate a beneficial effect of sodium fluoride on otosclerotic growth. High-resolution CT scans are a valid tool that can be used to confirm, localize, and determine the size of clinically suspected otosclerotic foci.
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            CT grading of otosclerosis.

            The CT grading system for otosclerosis was proposed by Symons and Fanning in 2005. The purpose of this study was to determine if this CT grading system has high interobserver and intraobserver agreement.
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              Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications.

              To collect data from a large number of cochlear implant recipients with otosclerosis and to make an assessment of these patients' clinical characteristics, computed tomographic scans, surgical findings, and complications, and to quantify the occurrence of postoperative facial nerve stimulation. Retrospective multicenter study. Fifty-three patients with otosclerosis from four cochlear implant centers in the United Kingdom and The Netherlands were reviewed. Sixty surgical procedures were performed in these patients: 57 devices were placed in 56 ears. The computed tomographic imaging demonstrated retrofenestral (cochlear) otosclerotic lesions in the majority of patients. Although not statistically significant, the extent of otosclerotic lesions on the computed tomographic scan as categorized in three types tends to be greater in patients with rapidly progressive hearing loss, in patients in whom there is surgically problematic insertion of the electrode array, and in patients with facial nerve stimulation. In four patients, revision surgery had to be performed. Twenty of 53 (38%) patients experienced facial nerve stimulation at various periods postoperatively. Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.
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                Author and article information

                Contributors
                +65-90147121 , purohitbela@yahoo.co.in
                Journal
                Insights Imaging
                Insights Imaging
                Insights into Imaging
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                1869-4101
                9 February 2014
                9 February 2014
                April 2014
                : 5
                : 2
                : 245-252
                Affiliations
                Department of Radiology, University Hospitals Leuven, 3000 Leuven, Belgium
                Article
                313
                10.1007/s13244-014-0313-9
                3999364
                24510845
                7c7a3ae8-670b-4a2f-8705-28b8c2c444ab
                © The Author(s) 2014

                Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.

                History
                : 11 November 2013
                : 5 January 2014
                : 13 January 2014
                Categories
                Pictorial Review
                Custom metadata
                © The Author(s) 2014

                Radiology & Imaging
                otosclerosis,fenestral,retrofenestral,hrct temporal bone,stapedectomy
                Radiology & Imaging
                otosclerosis, fenestral, retrofenestral, hrct temporal bone, stapedectomy

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