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Hearing loss due to acute leukemia

      To the Editor
      Ear involvement has rarely been described in patients with acute or chronic leukemia (
      • Gotay V.
      Unusual otological manifesta-tions of chronic lymphocytic leukemia.
      ). Otologic symptoms are usually related to bleeding, tumoral infiltration, or infection. We report 2 patients in whom hearing loss led to a diagnosis of acute leukemia.
      A 31-year-old man was admitted with a 1-month history of severe left external otitis. Laboratory examination revealed a leukocyte count of 5.3 × 109/L with 28% blasts, 37 × 109/L platelets, and a hemoglobin level of 91 g/L. Examination of the bone marrow established a diagnosis of acute myeloid leukemia (type 2 FAB). The patient underwent chemotherapy, followed by autologous bone marrow transplantation. A complete hematologic remission was obtained and the otitis resolved. Eight months later, left external otitis recurred, and a blood count confirmed a relapse of leukemia. Salvage chemotherapy led to partial remission and resolution of the otitis. One month later, the patient presented with external otitis, hearing loss, and uncompleted left facial palsy. Computed tomographic (CT) scans and magnetic resonance imaging (MR) images of the left temporal bone showed diffuse inflammatory content in the tympanic cavity and adjacent air cells, without destruction of the ossicles and labyrinth (Figure 1) Surgical exploration revealed a left middle-ear chloroma. Palliative external irradiation (30 Gy) achieved local control, and oral chemotherapy was administered, with supportive care. The patient died 3 months later of disseminated invasive aspergillosis.
      Figure thumbnail GR1
      Figure 1Horizontal computerized tomographic scan through the attic, with diffuse inflammation in the tympanic cavity (long arrow) and the squamous bone (short arrows). Note the slight modification of the bony pattern of the apex (curved arrow).
      A 55-year-old man experienced sudden left sensorineural hearing loss followed by progressive right conductive hearing loss during the next 2 months. He was diagnosed with otitis media, but therapy with oral antibiotics was ineffective. He was then admitted due to the onset of trigeminal neuralgia. Examination revealed bilateral otitis media with a thickened ear drum. The skin of the external auditory canal was thickened as well. Right myringotomy showed a bloody effusion of which the culture was sterile. A CT scan showed that the middle-ear cavities and skin were filled with soft tissue, and there was erosion of the cortical bone of the apex. A blood count showed 3.5 × 109/L leukocytes with 82% myeloblasts. Bone marrow examination confirmed acute myeloid leukemia (type 1 FAB). Cerebrospinal fluid was positive for blast cells. The patient underwent combination chemotherapy with idarubicin and cytosine arabinoside, along with intrathecal chemotherapy. Complete remission was obtained, and his hearing returned. Control MRI was performed with no evidence of tumor burden. The patient received two cycles of consolidation chemotherapy and 18-Gy of cranial radiation; complete remission persisted 6 months after diagnosis.
      Several cases of leukemic infiltration of the ear have been described in patients with chronic or acute myeloblastic or lymphoblastic leukemia (
      • Gotay V.
      Unusual otological manifesta-tions of chronic lymphocytic leukemia.
      ,
      • Paparella M.M.
      • Berlinger N.T.
      • Oda M.
      • El Fiky F.
      Otological manifestations of leukemia.
      ). Involvement of the cochlea and vestibule have also been reported (
      • Paparella M.M.
      • Berlinger N.T.
      • Oda M.
      • El Fiky F.
      Otological manifestations of leukemia.
      ,

      Schuknecht HF. Neoplastic growth. In: Schuknecht HF, ed. Pathology of the Ear. Cambridge, Mass: Harvard University Press; 1976:446.

      ). Free-floating cells are found in the perilymphatic spaces of the inner ear with or without hemorrhage (

      Schuknecht HF. Neoplastic growth. In: Schuknecht HF, ed. Pathology of the Ear. Cambridge, Mass: Harvard University Press; 1976:446.

      ). Hyperleukocytosis or acute myeloid leukemia subtypes 4 and 5 predispose patients to develop chloromas, which may occur in the ear, sometimes overlying the VIIth or VIIIth nerve, and lead to facial palsy and hearing loss, as in our patients (
      • Paparella M.M.
      • Berlinger N.T.
      • Oda M.
      • El Fiky F.
      Otological manifestations of leukemia.
      ,
      • Zappia J.J.
      • Bunge F.A.
      • Koopman Jr, C.F.
      • McClatchey K.D.
      Facial nerve paresis as the presenting symptom of acute leukemia.
      ). Chloromas sometimes precede the onset or relapse of leukemia by a few months (
      • Nieman R.S.
      • Barcos M.
      • Berard C.
      • et al.
      Granulocytic sarcoma a clinicopathologic study of 61 biopsied cases.
      ,
      • Kurtz J.E.
      • Maloisel F.
      • Andrès E.
      • et al.
      Syndrome abdominal aigu d’allure chirurgicale et leucémie aiguë.
      ). Because of the possibility of infection (eg, herpes zoster), examination of biopsy specimens is important for the diagnosis but may be difficult (especially in the middle ear) because of thrombocytopenia or an acquired coagulopathy (
      • Kurtz J.E.
      • Maloisel F.
      • Andrès E.
      • et al.
      Syndrome abdominal aigu d’allure chirurgicale et leucémie aiguë.
      ,
      • Lechner K.
      • Geissler K.
      • Jager U.
      • et al.
      Treatment of acute leukemia.
      ). However, CT scans and MR images are likely to discriminate between infection and tumor involvement of the middle and inner ear. In our patients, treatment quickly led to resolution of the otologic manifestations and complete hematologic remission.

      References

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        Unusual otological manifesta-tions of chronic lymphocytic leukemia.
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        • Paparella M.M.
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        • Oda M.
        • El Fiky F.
        Otological manifestations of leukemia.
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      1. Schuknecht HF. Neoplastic growth. In: Schuknecht HF, ed. Pathology of the Ear. Cambridge, Mass: Harvard University Press; 1976:446.

        • Zappia J.J.
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        Facial nerve paresis as the presenting symptom of acute leukemia.
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