We reported a case of Paget's disease presenting fluctuating hearing loss together with repeated dizziness and vertigo attacks. Pure tone audiometry in this patient was performed successively during follow-up periods. She visited our hospital 13 years ago with slight low tone sensorineural hearing loss in her left ear. Sensorineural hearing loss in her right ear and sensorineural and conductive hearing loss in left side deteriorated. Results of her latest examinations were as follows. Glycerol test was positive and inverted reflex was recorded in her right ear. CT showed severe calcification in skull bone and stenos of bilateral internal auditory canal. Endolymphatic hydrops, compression of VIII nerve in internal auditory canal, damage of cochlear hair cell and changes in middle ear conductive system were possible etiology of hearing loss and vertigo. Oral bisphosphonate is used to prevent deterioration of these symptoms. Evaluation of auditory and vestibular function should be continued.
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