Paget's disease of the temporal bone

Area of Science:

• Otolaryngology
• Orthopedics
• Genetics

Background:

• Osteitis deformans Paget is a common, heritable bone disease affecting primarily the axial skeleton.
• It impacts 3% of individuals over forty, with a higher prevalence in males.
• Skull and temporal bone involvement occurs in approximately two-thirds of patients.

Purpose of the Study:

• To detail the clinical, radiological, and pathological manifestations of Paget's disease affecting the temporal bone.
• To explain the structural changes in the temporal bone due to the disease.
• To present examples of associated tumor formations and vascular changes.

Main Methods:

• Review of clinical, radiological, and pathological findings in patients with Paget's disease of the temporal bone.
• Detailed examination of temporal bone architecture, including the petrous pyramid, middle ear, and inner ear capsule.
• Photomicrographic documentation of disease manifestations.

Main Results:

• Progressive temporal bone involvement can alter its position, size, and architecture.
• Hearing impairment affects 30-50% of cases, and vestibular dysfunction occurs in 20-25%.
• Tumor formations and vascular changes are noted in affected temporal bones.

Conclusions:

• Paget's disease of the temporal bone can lead to significant auditory and vestibular deficits.
• Understanding these manifestations is crucial for diagnosis and management.
• The disease's impact on cranial bones warrants further investigation.