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FLUCTUATING MIXED TYPE HEARING LOSS ASSOCIATED WITH SAPHO SYNDROME

Kumiko Yukawa1, Sachie Kawaguchi1, Yukari Okubo2, Mamoru Suzuki1

1Department of Otolaryngology, Tokyo Medical University, Tokyo, Japan/2Department of Dermatology, Tokyo Medical University, Tokyo, Japan

INTRODUCTION: SAPHO syndrome is featured by synovitis, acne, palmoplantar pustrosis, hyperostosis and osteitis. It is a seronegative syndrome of bone and joint lesions, frequently localized to the anterior chest wall, sternoclavicular joints and vertebrae. We describe a patient of SAPHO syndrome with unilateral hearing loss, that might be caused by involvement of the auditory ossicules.

CASE REPORT: A 53-year-old woman with SAPHO syndrome was referred to our hospital for the indication of tonsillectomy on May 2005. She had been treated as palmoplantar pustrosis for two years. The result of the tonsil provocation test showed increased blood cell in the urine. She complained of hearing loss in the right ear from January 2006 and returned our hospital. Her right ear drum was dull and diagnosed as an external otitis media. PTA showed mild hearing loss on the right ear and it became worse despite the recovery from the inflammation. Her tympanogram revealed As type on the right ear and A type on the left ear. Acoustic reflex was absent in the right ear. CT scan showed bilateral normal ossicles and cochleas. Bone scintigraphy showed tracer uptake on the bilateral sternoclavicular joints, glenohumeral joints and capital of humerus. She was hospitalized in March 2006 for the pain control of the arthritis. The pain was controlled with steroid therapy, but her hearing dropped to 53.7dB, soon after the tapering of the steroid. However, the hearing improved to 26.2dB. After the second tonsil provocation test, her pustrosis got worse. We recommended her tonsillectomy. She was admitted again because of the arthritis and treated with the immunosuppressant. She came to our clinic on March 2007 complaining of the right hearing loss and earache. Because of the persistent attacks of arthritis and hearing loss, she finally agreed to have the tonsillectomy on coming June.

DISCUSSION: This patient had a mixed type of hearing loss related to the involvement of the ossiclar joints by SAPHO syndrome. The clinical findings related to the hearing loss indicate the results of inflammation of the synovial ossicular joint, which is followed by stiffness of the ear drum and ossicular chain.