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Light Chain Amyloidosis Presenting as Bilateral External Auditory Canal Obstructing Masses.

Shenon Sethi1, Agne Paner2, Tochukwu Okwuosa3

  • 1Department of Pathology.

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
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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.

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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.