Waldenstrom's macroglobulinemia and sensorineural hearing loss

Waldenstrom's macroglobulinemia, (WM) first described in 1944, is an uncomm on disease caused by the abnormal production of immunoglobulin M monoclonal macroglobulin. Presenting signs and symptoms most frequently include fatig ue or generalized weakness; tendency to bleed from mucosal surfaces; charac teristic retinal lesions, including dilated and tortuous retinal veins, ret inal hemorrhages, and exudates; lymphadenopathy; hepatosplenomegaly; sensor y motor peripheral neuropathy; worsening normochromic anemia; increased sed imentation rate; and extremely high serum viscosity. Hearing loss is an unu sual presenting symptom of WM; only 6 cases are reported in literature. The etiology of hearing loss is unclear; however, hyperviscosity and thrombus formations are the most likely causes. We present a patient diagnosed with WM whose initial presenting symptoms were hearing loss and progressive sens ory peripheral neuropathy with subsequent development of lymphadenopathy an d hyperviscosity syndrome. Treatment with Fludarabine lead to improvement o f her hearing and neurological deficits, as well as resolution of her other hyperviscosity-related symptoms.