Semicircular canal dysplasia
History of Down syndrome, complaining of right sided otalgia and bilateral hearing loss.
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- axial projection showing partial opacification of the right middle ear cavity, namely the epi and mesotympanum, by isoattenuating soft tissue density extending to the aditus ad antrum and ipsilateral mastoid air cells. This is associated with blunting of the scutum, erosion of the posterior facial nerve bony canal, as well as ill-definition of the anterior crura of the right stapes, consistent with right chronic otomastoiditis with cholesteatoma formation
- axial and coronal projections showed abnormal morphology of the semicircular canals bilaterally with bilaterally widened and shortened lateral semicircular canals, small fragmented central bony islands, and dilated vestibules
The inner ear is commonly affected in patients with Down syndrome resulting in several anomalies. The semicircular canals are one of the most commonly affected inner ear structures, especially the lateral one. The average size of the central bony island is 3.7 mm, while a bony island measuring less than 3 mm in pathognomonic. The semicircular canals may be short, dilated, wide, or aplastic.