SAPHO syndrome of the temporomandibular joint associated with sudden deafness

• Published in: American journal of neuroradiology : AJNR Vol. 20; no. 5; pp. 902 - 905
• Main Authors: MARSOT-DUPUCH, K, DOYEN, J. E, GRAUER, W. O, DE GIVRY, S. C
• Format: Journal Article
• Language: English
• Published: Oak Brook, IL American Society of Neuroradiology 01-05-1999
• Subjects: Acquired Hyperostosis Syndrome - complications; Acquired Hyperostosis Syndrome - diagnosis; Biological and medical sciences; Diseases of the osteoarticular system; Hearing Loss, Sudden - etiology; Humans; Interventional; Magnetic Resonance Imaging; Male; Mandibular Diseases - diagnosis; Medical sciences; Middle Aged; Miscellaneous. Osteoarticular involvement in other diseases; Temporomandibular Joint Disorders - complications; Temporomandibular Joint Disorders - diagnosis; Tomography, X-Ray Computed; Human; Temporal bone; Diseases of the osteoarticular system; Auditory disorder; Temporomandibular joint; Exploration; Male; Bone disease; Arthritis; Nuclear magnetic resonance imaging; Hearing loss; Case study; Osteitis; Arthropathy; Medical imagery; ENT disease; Inaugural sign
• ISSN: 0195-6108; 1936-959X

We report a case of arthritis of the temporomandibular joint (TMJ) associated with sclerosing osteomyelitis of the mandible and temporal bone, causing deafness. The presence of a palmoplantar pustulosis established the diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is defined by the association of characteristic osteoarticular and dermatologic manifestations, with diffuse sclerosing osteomyelitis of the mandible being a part of this entity. We review the literature of SAPHO syndrome with mandibular manifestations and discuss the mechanisms of inflammatory spread from the TMJ to the cochlea. To our knowledge, this is the first description of skull base involvement in a patient with SAPHO syndrome leading to sudden deafness.