Paget Disease

CHAPTER 54 Paget Disease

Ashok Srinivasan

Epidemiology

Paget disease, also called osteitis deformans, is a chronic disorder that involves approximately 3% of adults above 40 years of age and approximately 10% above 80 years. The etiology is unknown, though it has been attributed to genetic and infectious factors and there is familial and geographic clustering.

Clinical Features

Paget disease may be asymptomatic and detected incidentally or can present with involvement of one or multiple bones. Although Paget disease can involve various portions of the auditory apparatus like the external auditory canal, otic capsule, and petrous pyramid, ossicular involvement is rare. The most common clinical symptom in temporal bone involvement is hearing loss, which can be conductive, sensorineural, or mixed. Other symptoms that can occur include tinnitus and vertigo.

Pathology

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