Synovitis, acne, palmoplantar pustulosis (PPP), hyperostosis, and osteitis (SAPHO) are the characteristic features of SAPHO syndrome. A 53-year-old woman had been treated for PPP for 2 years. She complained of hearing loss in the right ear, and otitis externa was diagnosed. The pure-tone audiogram (PTA) indicated mild hearing loss in the right ear, and her hearing continued to deteriorate despite recovery from inflammation. Her tympanogram was of the As-type, and acoustic reflex was absent in the right ear. A computed tomography (CT) scan revealed bilateral normal ossicles and cochleas. Bone scintigraphy revealed tracer uptake in the bilateral sternoclavicular joints, glenohumeral joints, and the capital humerus. She was hospitalized for arthralgia, and the pain was controlled with steroid therapy. Her right hearing deteriorated soon after the tapering of the steroid; her hearing recovered after cyclosporine therapy was initiated. The first tonsil provocation test showed increased blood cells in the urine, and the second test showed exacerbation of pustulosis. Despite immunosuppressant therapy, the arthritis attacks and hearing loss persisted; therefore, tonsillectomy was performed, which improved PPP. However, her hearing remained unchanged after the operation. We considered that irreversible changes might have already developed in the ossicular joints, and ossicular reconstruction was performed. Thereafter, her hearing and earache improved.