Otologic manifestations of amyloidosis

Objective: To describe otologic manifestations of amyloidosis. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Case report of a patient with bilateral external auditory meatal stenosis secondary to amyloidosis. Results: The patient is a 60-year-old man who presented with several months' history of bilateral ear blockage and drainage. Physical examination revealed markedly thickened skin in each ear in the area of concha and meatus that was associated with fissuring. The meatus were markedly stenosed. The medial aspect of the external auditory canal and the tympanic membrane were normal. He had partial improvement on medical therapy with topical steroid-containing eardrops and ointment. Past medical history was positive for multiple myeloma. Meatal biopsy was performed. Grossly, the tissue was hypovascular and markedly thickened. Histopathologic examination was consistent with amyloidosis. The patient was continued on topical steroid ointments, frequent car cleaning, and close follow-up. He was subsequently diagnosed with systemic amyloidosis. A review of the literature was done. Head and neck involvement with amyloidosis is reviewed, with emphasis on otologic manifestations. Conclusion: Otologic involvement with amyloidosis is extremely rare. The pathophysiology may be related to the local factors in the external auditory canal.