Fig. 1

CT scans and MRI showing massive osteolysis of the skull bones. a−c The CT scans show that despite osteolysis affecting most of the temporal bone, the cochlear bony capsule was preserved and is almost intact (arrow in c). MRI revealed that the temporal lobe of the brain had herniated into the melted mastoid (d). The CT scans reveals progressive osteolysis of the temporal bone (asterisk in c and e) comparing with previous imaging obtained when he was 18 years old (e). 3D reconstructed CT clearly shows osteolysis of the skull bones; in addition to the right temporal bone (*), other affected bones include the mandibular bone, zygomatic bone, and contralateral temporal bone (arrowheads in f)