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Updated: Jul 31, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Waldenstrom's macroglobulinemia and sensorineural hearing loss
M J Syms1, M E Arcila, M R Holtel
1House Ear Institute and House Ear Clinic, Los Angeles, CA 90057, USA.
Abstract:
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.
Insights
Waldenstrom's macroglobulinemia (WM) can cause hearing loss, a rare symptom. Treatment with Fludarabine improved hearing and neurological deficits in a patient with WM.
Area of Science:
- Hematology
- Neurology
Background:
- Waldenstrom's macroglobulinemia (WM) is an uncommon lymphoproliferative disorder characterized by the abnormal production of IgM monoclonal macroglobulin.
- Common symptoms include fatigue, bleeding, retinal lesions, lymphadenopathy, hepatosplenomegaly, neuropathy, anemia, and hyperviscosity.
Observation:
- Hearing loss is an unusual presenting symptom of WM, with only six prior cases reported.
- This case highlights a patient whose initial symptoms were hearing loss and sensory peripheral neuropathy.
Findings:
- The patient presented with Waldenstrom's macroglobulinemia, initially manifesting as hearing loss and progressive sensory peripheral neuropathy.
- Subsequent development of lymphadenopathy and hyperviscosity syndrome was observed.
- Treatment with Fludarabine resulted in significant improvement in hearing and neurological deficits.
Implications:
- This case underscores that hearing loss can be an initial presenting symptom of Waldenstrom's macroglobulinemia.
- It suggests hyperviscosity and thrombus formation as potential etiologies for WM-associated hearing loss.
- Effective treatment of WM can lead to the resolution of hyperviscosity-related symptoms, including hearing and neurological deficits.
