Waldenstrom's macroglobulinemia and sensorineural hearing loss

• Published in: American journal of otolaryngology Vol. 22; no. 5; pp. 349 - 353
• Main Authors: Syms, Mark J., Arcila, Maria E., Holtel, Michael R.
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.09.2001; Elsevier
• Subjects: Aged; Antibodies, Antinuclear - immunology; Audiometry, Speech; Auditory Threshold - physiology; Biological and medical sciences; Blood Protein Electrophoresis - methods; Blood Viscosity; Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology; Female; Hearing Loss, Sensorineural - complications; Hearing Loss, Sensorineural - diagnosis; Hematologic and hematopoietic diseases; Humans; Immunoglobulin M - blood; Medical sciences; Non tumoral diseases; Other diseases. Hematologic involvement in other diseases; Otorhinolaryngology. Stomatology; Severity of Illness Index; Waldenstrom Macroglobulinemia - complications; Waldenstrom Macroglobulinemia - immunology; Audiometry; Auditory disorder; IgM; Age 70-79; Hyperviscosity syndrome; Immunoglobulinopathy; Lymphoproliferative syndrome; ENT disease; Adult; Electrophoresis; Female; Perception hearing loss; Diagnosis; Waldenstrom macroglobulinemia; Human; Immunopathology; Purine nucleotide; Etiopathogenesis; Exploration; Malignant hemopathy; Case study; Symptomatology; Concomitant disease; Chemotherapy; Fludarabine; Treatment; Antimetabolic; Cytopathology
• ISSN: 0196-0709; 1532-818X
• DOI: 10.1053/ajot.2001.26494

Waldenstrom's macroglobulinemia, (WM) first described in 1944, is an uncommon disease caused by the abnormal production of immunoglobulin M monoclonal macroglobulin. Presenting signs and symptoms most frequently include fatigue or generalized weakness; tendency to bleed from mucosal surfaces; characteristic retinal lesions, including dilated and tortuous retinal veins, retinal hemorrhages, and exudates; lymphadenopathy; hepatosplenomegaly; sensory motor peripheral neuropathy; worsening normochromic anemia; increased sedimentation rate; and extremely high serum viscosity. Hearing loss is an unusual 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 sensory peripheral neuropathy with subsequent development of lymphadenopathy and hyperviscosity syndrome. Treatment with Fludarabine lead to improvement of her hearing and neurological deficits, as well as resolution of her other hyperviscosity-related symptoms. (This is a US government work. There are no restrictions on its use.)