SAPHO Syndrome of the Temporomandibular Joint Associated with Sudden Deafness

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fig 1.
57-year-old man with SAPHO syndrome.
A, Axial contrast-enhanced T1-weighted MR image (500/15) reveals focal enhancement of the dura (large arrow) of the temporal bone, spreading into the internal auditory meatus with enhancement of the cochlea (small arrow). An enhancing soft-tissue mass of the middle ear, outlining the bony ossicles, is also present.
B, Axial contrast-enhanced T1-weighted MR image of the infratemporal fossa (540/15) (1 cm below A) shows a large soft-tissue mass around the TMJ (arrowhead), involving the lateral pterygoid muscle (arrow). Note the low intensity signal of the bone marrow of the condyle.
C, Axial CT scan of the left temporal bone shows abnormal endosteal sclerosis of the temporal squama (arrow) with subcortical erosions (arrowhead). A soft-tissue mass (arrowhead) engulfs the incudomalleal joint.
D, Axial CT scan (2 cm below C) shows osteosclerosis of the mandibular ramus (arrow).
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fig 2.
Palmoplantar pustulosis. Characteristic aseptic pustular and hyperkeratotic lesions involve palmar surface of the hand. Similar lesions were present on the soles of the feet of this patient (reproduced by permission of Dr. F. Hayem, Paris)