Case 48: osteogenesis imperfecta of the temporal bone.

A 36-year-old African-American woman who had a history of fluctuating bilateral mixed hearing loss that was worse on the left side than on the right complained of worsening of hearing on the right side during follow-up. At physical examination, she was a short obese woman who was wheelchair bound. The scleras were discolored. The tympanic membranes were intact. Audiogram findings demonstrated profound hearing loss on the left side and moderately severe mixed conductive and sensorineural hearing loss on the right side. In the past, she had fluctuations in her hearing with overall progressive deterioration; steroid treatment was unsuccessful. Unenhanced computed tomographic (CT) scans of the petrous temporal bones were obtained with a bone algorithm. Transverse scans of the right side (Fig 1a, 1b) and coronal scans (Fig 1c, 1d) of both sides were obtained.