A Case of Primary Macroglobulinemia Initially Presented as Sensorineural Hearing Loss

Abstract

Primary macroglobulinemia is called Waldenström’s macroglobulinemia (WM), which is defined as lymphoplasmacytic lymphoma (LPL). The excessive and abnormal production of immunoglobulin M (IgM) monoclonal macroglobulin protein in bone marrow is characteristic of WM.
Clinical signs and symptoms are presented by hyperviscosity with excessive IgM protein disordering the vessel flow with sensory and motor peripheral neuropathy derived from the myelin-associated glycoprotein (MAG) in IgM.
The clinical features in the hyperviscosity syndrome are the different kinds of mucosal hemorrhage, retinal vein thrombosis and hemorrhage, headache, vertigo, hearing loss and so on. However, sensorineural hearing loss is rarely caused by WM, and WM with hearing loss has been reported only in eight studies to the best of my knowledge. Although the etiology of the hearing loss is unclear, but the past reports have concluded that the most probable of the cause of the hearing loss is the hyperviscosity syndrome and reported that hearing loss improved with plasma exchange treatment or chemotherapy.
We experienced a case which proved to be WM after the recovery of sensorineural hearing loss with steroid therapy followed by plasma exchange and chemotherapy. The patient reached remission status for the WM and the hearing loss has not recurred.