両側進行性高度難聴を示した骨Paget病の一例

抄録

We encountered a patient treated for bilateral sudden deafness, whose hearing level became progressively worse. She showed high alkaline phosphatase activity, high urinary hydroxyproline excretion, and typical temporal bone X-ray findings. Her hearing loss (HL) was a high frequency sensorineural HL and a low frequency air-bone gap. Paget's disease of the bone or fibrous dysplasia was suspected, and a temporal bone biopsy was performed and Paget's disease was diagnosed. Oral alendronate effectively improved her condition.