Ear
Inflammatory / infectious / autoimmune / systemic disorders
Paget disease of temporal bone

Author: Nat Pernick, M.D. (see Authors page)

Revised: 8 February 2018, last major update October 2013

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Paget disease of temporal bone

Cite this page: Pernick, N. Paget disease of temporal bone. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/earpaget.html. Accessed July 22nd, 2018.
Definition / general
  • Also called osteitis deformans
  • Chronic progressive disease of increased bone turnover, affects skull and temporal bone in 70% of cases with hearing loss in half of these cases
  • Hearing loss is due to compression of cranial nerve VIII and apparently loss of mineral density of cochlear capsule (Laryngoscope 2004;114:598)
  • Familial cases are due to mutations in bone metabolism pathway genes (Nat Clin Pract Rheumatol 2006;2:270)
  • Causes enlargement and tortuosity of superficial temporal artery and its anterior branches
  • Also affects numerous structures of external, middle and inner ear
  • Affects 3% of population ages 40+, 11% at age 80+; men affected slightly more than women
  • 1% have transformation to osteosarcoma or other sarcoma with 5 year survival of < 10%
Microscopic (histologic) description
  • Osteolytic phase has extensive osteoclastic activity and bone resorption
  • Mixed / combined phase has osteoblastic activity exceeding osteoclastic activity
  • Osteoblastic phase has dense new bone with mosaic pattern of cement lines
Differential diagnosis