Abstract
We present the case of a 33-year old woman with osteogenesis imperfecta (OI) with progressive hearing loss and persisting vertigo. On CT-scan, symmetric extensive lucency in the pericochlear bony otic capsule and promontorium was demonstrated. The MRI-scan demonstrated symmetric areas of increased signal intensity on the T2-images with moderate contrast enhancement in the same regions. These findings correlate histologically by undermineralized thickened bone, the hallmark of OI. Hearing loss is an important clinical feature in patients with OI. The value of temporal bone imaging lies in additional confirmation of the diagnosis, determining the extent disease and excluding concomitant pathology.