Presentation
Bilateral conductive hearing loss.
Patient Data
There are patchy areas of abnormal bone demineralization in the fissula ante fenestram and around the basal turn of the cochlea bilaterally. Additional bone demineralization around the labyrinthine segments of the facial nerve canals on both sides.
Moderate aerosolized mucosal secretions in the sphenoid sinuses and ethmoidal air cells. Moderate mucosal thickening and retention cysts in the maxillary sinuses.
Case Discussion
This patient has a past medical history of osteogenesis imperfecta type I. This case depicts the classic imaging manifestations of osteogenesis imperfecta in the otic capsules involving the fenestral and retrofenestral structures.
The differential diagnosis of symmetric bilateral bone demineralization of the otic capsules includes osteogenesis imperfecta (as in this case), fenestral and retrofenestral otosclerosis, and otosyphilis.
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