Ear
Inflammatory / infectious / autoimmune / systemic disorders
Paget disease of temporal bone
Author: Nat Pernick, M.D.
Last author update: 1 October 2013
Last staff update: 30 September 2020
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Paget disease of temporal bone
Table of Contents
Definition / general | Microscopic (histologic) description | Differential diagnosis | Additional referencesCite this page: Pernick N. Paget disease of temporal bone. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earpaget.html. Accessed June 3rd, 2023.
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
- Otosclerosis: younger age, doesn't affect skull
