Case Presentation: A 63-year old woman presented with progressive, severe bilateral hearing loss over the course of two months with complete loss of hearing over the week prior to presentation. She had associated imbalance and mild generalized fatigue. She had not received medical care for many years prior to this admission. She denied head trauma, prolonged exposure to noise or ototoxins, tinnitus, fever, ear pain, or sinus congestion. She took no medications. Review of systems was otherwise negative.Physical exam confirmed bilateral sensorineural hearing loss. Laboratory testing showed lymphocytic predominance of leukocytes, and mild anemia and thrombocytopenia. Comprehensive metabolic panel showed Cr 1.38, as well as a large protein-albumin gap (serum total protein 9 g/dL and albumin 2 g/dL). Brain imaging was normal.
Further diagnostic testing included serum protein electrophoresis demonstrating IgM 8728 mg/dL and serum viscosity 27.3 centipoises (cP) (normal <1.5 cP). Bone marrow biopsy confirmed lymphoplasmacytic lymphoma consistent with Waldenström macroglobulinemia (WM). The patient received 5 days of continuous total plasmapheresis for hyperviscosity syndrome and was initiated on chemotherapy for B cell lymphoma. Despite reduction of serum viscosity to near normal, the patient did not regain hearing during her hospitalization. She was discharged home with follow up for ongoing chemotherapy and audiology.
Discussion: Waldenström macroglobulinemia (WM) is a lymphoproliferative lymphoma characterized by bone marrow infiltration by lymphoplasmacytic lymphoma (LPL) and circulating monoclonal IgM. The large size and pentameric configuration increases serum viscosity and impairs blood flow through the microcirculatory system. Hyperviscosity syndrome (HVS) is present in up to 30% of patients with WM. HVS is more likely when serum viscosity rises above 4 cP. The level of serum viscosity in this patient is among the highest reported in the literature. Typical neurologic manifestions of HVS include visual disturbances, headache, ataxia, vertigo, tinnitus, and rarely seizure and coma.
Current treatment for HVS is plasmapheresis, the most effective method of removing IgM from circulation. The very high level of viscosity at presentation combined with the duration of symptoms prior to seeking medical care likely contributed to the persistence of her symptoms despite aggressive therapy. Of eleven published case reports of WM presenting with some degree of sensorineural hearing loss, we believe that this case represents the third in which bilateral sensorineural hearing loss is the only presenting symptom.
Conclusions: Though rare, hyperviscosity syndrome can have potentially devastating consequences if not recognized and treated rapidly. For patients presenting with symptoms of HVS, hospitalists are imperative to promptly arrive at the diagnosis and to the coordination of complex care required to assure the best possible outcomes.