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Updated: Feb 8, 2026
06:33
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
8.1K
Light Chain Amyloidosis Presenting as Bilateral External Auditory Canal Obstructing Masses
Shenon Sethi1, Agne Paner2, Tochukwu Okwuosa3
1Department of Pathology.
Summary
Systemic amyloidosis, a rare condition, can manifest as ear canal masses. Early diagnosis of AL (amyloid light chain) amyloidosis is crucial for better patient outcomes.
Area of Science:
- Nephrology
- Oncology
- Otolaryngology
Background:
- Systemic amyloidosis is a rare plasma cell dyscrasia.
- External auditory canal involvement is an exceptionally rare manifestation.
Purpose of the Study:
- To report a rare case of bilateral external auditory canal amyloidosis.
- To highlight the diagnostic challenges and importance of early detection.
Main Methods:
- Histologic examination of ear canal biopsies.
- Laser mass spectrometry for amyloid typing.
- Systemic work-up including bone marrow biopsy and kidney biopsy.
Main Results:
- Biopsies revealed AL kappa-type amyloid deposits in the dermis of both auditory canals.
- Systemic evaluation confirmed plasma cell dyscrasia and amyloid deposition in the kidney.
- This case represents one of few reported instances of bilateral external auditory canal amyloidosis.
Conclusions:
- External auditory canal amyloidosis is a rare but significant manifestation of systemic amyloidosis.
- Accurate and timely diagnosis through appropriate biopsy and typing is essential for management.
- Early treatment of systemic light chain amyloidosis is critical for improving patient prognosis.
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