Primary Nasopharyngeal Amyloidosis: Unexpected Cause of Unilateral Hearing Loss

Year 2018, Volume: 1 Issue: 3, 79 - 83, 06.03.2019

Meltem Bozacı Kılıçoğlu , Bayram Şahin , Levent Aydemir , Gülçin Yeğen Şenol Çomoğlu

Abstract

Primary nasopharyngeal amyloidosis (PNA) is an extremely rare condition described in the otorhinolaryngology literature, for which the standard therapy still does not exist. Herein, we discuss an atypically presented PNA case that caused a nasopharyngeal mass and the conductive-type hearing loss. A 54-year-old male patient presented with aural fullness and hearing loss in the right ear lasting for 2 weeks. A physical examination with nasal endoscopy indicated an irregular mass lesion on the right half of the nasopharynx. Otomicroscopy showed otitis media with effusion (Type B tympanogram) in the right ear. Magnetic resonance imaging (MRI) revealed the presence of a contrast-enhanced soft tissue mass arising from the lateral nasopharyngeal wall. A biopsy was performed with nasal endoscopy under
local anesthesia, and histopathological evaluation was reported as primary (AL type) amyloidosis. Therefore, the patient was accepted as PNA due to the absence of systemic involvement. One year later, the patient was admitted due to hoarseness. A histopathological examination of the right ventricle was reported as primary amyloidosis. PNA is an exceedingly rare condition, and it typically occurs as an isolated manifestation of localized amyloidosis. However, the clinician must be aware of the association with nasopharyngeal carcinoma and the possibility of plasma cell dyscrasias, especially multiple myeloma and lymphoproliferative diseases. In the absence of systemic disease evidence, localized amyloidosis of the nasopharynx and larynx may be treated conservatively.

Keywords

Primary amyloidosis , nasopharyngeal amyloidosis , amyloid , hearing loss , nasopharyngeal mass

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