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A Woman Who Couldn't Hear

      Keywords

      To the Editor:
      A 43-year-old female resident of Bihar (eastern part of India) complained of severe headache and bilateral hearing loss for 2 weeks. She also reported low-grade fever and left upper abdomen fullness for the past 1 month. Examination revealed mild pallor and palpable liver (5 cm) and spleen (8 cm). Neurological examination was unremarkable. Pure tone audiometry suggested bilateral sensorineural hearing loss. Blood tests revealed hemoglobin of 8.0 g/dL, white cell count of 2.72 × 109/L, platelets of 90 × 109/L, and erythrocyte sedimentation rate of 68 mm/hour. Peripheral blood smear showed normocytic normochromic red cells, marked rouleaux formation, and no evidence of atypical cells or hemoparasites. Serum biochemistry revealed marked hypergammaglobulinemia (proteins 11.5 g/dL, albumin 2.8 g/dL). Liver and kidney function tests, serum calcium, lactate dehydrogenase, and uric acid were normal. Serum protein electrophoresis showed polyclonal hypergammaglobulinemia. Serum immunofixation failed to identify the monoclonal band and free light chain ratio was normal. Tuberculin test and malaria serology were negative. Viral markers, human immunodeficiency virus, serology, and anti-nuclear antibody were unremarkable. Abdominal ultrasound showed massive hepatosplenomegaly without any evidence of portal cavernoma, or portosystemic collaterals. Esophago-gastroduodenoscopy was normal. Chest x-ray was normal. No bone lesions were found on the skeletal survey. Bone marrow aspirate showed numerous intracellular and extracellular Leishman-Donovan bodies, consistent with visceral leishmaniasis (Figure). Serology for RK-39 antigen was positive.
      • Torres-Guerrero E
      • Quintanilla-Cedillo MR
      • Ruiz-Esmenjaud J
      • Arenas R.
      Leishmaniasis: a review.
      ,
      • Sundar S
      • Benjamin B.
      Diagnosis and treatment of Indian visceral leishmaniasis.
      Patient was treated with 30 doses of amphotericin B deoxycholate (1 mg/kg/day), given on alternate days.
      • Sundar S
      • Benjamin B.
      Diagnosis and treatment of Indian visceral leishmaniasis.
      There was complete resolution of cytopenias, improvement in hearing, and disappearance of organomegaly at end of the treatment. Repeat bone marrow aspirate performed after 1 month did not show any Leishman-Donovan bodies.
      Figure
      FigureMicrophotograph of the bone marrow aspirate smears (Wright-Giemsa stain, 100 X) showing (black arrows) numerous intracellular (A) and extracellular (B) Leishman-Donovan bodies.
      Serum hyperviscosity could manifest with bilateral sensorineural hearing loss. In the setting of massive splenomegaly, hyperviscosity may be related to the following: 1) high white cell counts, seen in chronic myeloid leukemia,
      • Benneh AA
      • Olayemi E
      • Baidoo K
      • Adjei DN
      Parameters that contribute to hearing loss in CML patients.
      2) hematocrit, seen in polycythemia vera,
      • Dogan EE
      • Uslu M
      • Comert Ozkan M
      • et al.
      The effect of polycythemia vera on hearing functions: evaluation of twenty-one patients.
      or 3) hypergammaglobulinemia, either monoclonal associated with Waldenstrom's macroglobulinemia,
      • Syms MJ
      • Arcila ME
      • Holtel MR
      Waldenstrom's macroglobulinemia and sensorineural hearing loss.
      or polyclonal associated with tropical infections (malaria and visceral leishmaniasis).
      • Zhao SZ
      • Mackenzie IJ.
      Deafness: malaria as a forgotten cause.
      ,
      • Hashim FA
      • Ahmed AE
      • el Hassan M
      • et al
      Neurologic changes in visceral leishmaniasis.
      If left untreated, visceral leishmaniasis could cause bone marrow suppression (pancytopenia), immune dysfunction (recurrent bacterial infections), cachexia, hypoalbuminemia, edema, liver dysfunction (jaundice, ascites), renal impairment (proteinuria, hematuria, azotemia secondary to glomerulonephritis or interstitial nephritis), or bleeding diathesis (thrombocytopenia and liver dysfunction). Rarely, disseminated intravascular coagulopathy and hemophagocytic lymphohistiocytosis can ensue.
      • Torres-Guerrero E
      • Quintanilla-Cedillo MR
      • Ruiz-Esmenjaud J
      • Arenas R.
      Leishmaniasis: a review.
      ,
      • Clementi A
      • Battaglia G
      • Floris M
      • Castellino P
      • Ronco C
      • Cruz DN
      Renal involvement in leishmaniasis: a review of the literature.
      • Mishra P
      • Dixit A
      • Chatterjee T
      • et al.
      Disseminated intravascular coagulation as an unusual presentation of Kala-azar: report of two cases.
      • Ranjan P
      • Kumar V
      • Ganguly S
      • et al.
      Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis: varied presentation.
      A high-index of suspicion must be maintained for visceral leishmaniasis in patients presenting with a massively enlarged spleen, particularly those who are residents of endemic areas. Early treatment with amphotericin B could be curative.

      References

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        Diagnosis and treatment of Indian visceral leishmaniasis.
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        Parameters that contribute to hearing loss in CML patients.
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        The effect of polycythemia vera on hearing functions: evaluation of twenty-one patients.
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        Waldenstrom's macroglobulinemia and sensorineural hearing loss.
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        Deafness: malaria as a forgotten cause.
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        Neurologic changes in visceral leishmaniasis.
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        Disseminated intravascular coagulation as an unusual presentation of Kala-azar: report of two cases.
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