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Hearing loss in Paget's disease: a temporal bone histopathology study
Panagiotis A Dimitriadis1, Doris-Eva Bamiou, Athanasios G Bibas
1UCL Ear Institute, London, UK.
Summary
Paget's disease of bone (PDB) can cause hearing loss. This study found fractured stapes footplates and bone remodeling near Cotugno's canal, potentially explaining hearing impairment in PDB patients.
Area of Science:
- Otolaryngology
- Pathology
- Genetics
Background:
- Hearing loss is common in Paget's disease of bone (PDB), often mixed and bilateral.
- The exact causes of hearing impairment in PDB are not fully understood.
Purpose of the Study:
- To investigate the histopathologic features of temporal bones in PDB patients.
- To identify pathological mechanisms contributing to hearing loss in PDB.
Main Methods:
- Archival human temporal bone study.
- Analysis of 8 temporal bone specimens from individuals diagnosed with PDB.
Main Results:
- Fractured stapes footplate in one specimen; stapes footplate fixation in two.
- Obliteration of Cotugno's canal by bone remodeling in 78% of specimens.
- Other findings include otic capsule microfissures, bleeding in scalae, strial atrophy, and cystic lesions.
Conclusions:
- First report of fractured stapes footplate as a cause of conductive hearing loss in PDB.
- Frequent, previously unreported bone remodeling around Cotugno's canal.
- Hypothesized link between Cotugno's canal obliteration, impaired venous drainage, and sensorineural hearing loss in PDB.
