Superior semicircular canal dehiscence (SSCD) is a rare entity recently described whose typical clinical symptomatology is represented by dizziness triggered by a variation of pressure. We reported a case of SSCD which was diagnosed thanks to computed tomography (CT) scan of the petrous bone conducted systematically in front of mixed deafness with normal eardrum. The SSCD was bilateral and was revealed by mixed deafness on the left side and perception deafness on the right with a normal eardrum without the notion of vertigo. The cervical vestibular evoked myogenic potential (cVEMP) and an ultra-high resolution CT scan of the petrous bones in coronal and sagittal sections allowed the diagnosis. The SSCD should be considered in the presence of any conductive or mixed hearing loss with a normal eardrum. The CT scan in coronal and sagittal submillimetric sections allows the diagnosis.
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