Available online at www.sciencedirect.com
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 33 (2012) 782 – 783 www.elsevier.com/locate/amjoto
Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome☆ Eun Jung Lee, MD, PhD⁎, Yong Joo Yoon, MD, PhD Department of Otolaryngology–Head and Neck Surgery, Chonbuk National University School of Medicine, Research Institute of Clinical Medicine, Chonbuk National University, Jeon-ju, Korea Received 19 July 2012
Abstract
This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment. © 2012 Elsevier Inc. All rights reserved.
1. Introduction
2. Case report
The myelodysplastic syndrome (MDS) comprise a heterogeneous group of malignant stem cell disorders characterized by dysplastic and ineffective blood cell production (ie, apoptotic death) and a variable risk of transformation to acute leukemia [1]. It presents nonspecific signs and symptoms. Many patients with MDS are asymptomatic, with the diagnosis established upon routine laboratory screening. Otolaryngologic symptoms and signs such as vertigo, tinnitus, hearing loss, and facial weakness are common in hematologic diseases including acute leukemia, multiple myeloma, and Waldenstrom macroglobulinemia [2]. However, sudden hearing loss as an initial manifestation in hematologic diseases is a rare occurrence. To the best of our knowledge, the patient we reported is the first case of MDS with sudden onset of bilateral sensorineural hearing loss as the initial presenting symptom. The purpose of this study is to draw attention of otologists to the possible association of acute sensorineural hearing loss to MDS and other hematologic diseases.
A 76-year-old woman with no remarkable medical history came to our clinic complaining about sudden-onset bilateral hearing loss. The right-side hearing impairment occurred 3 days before her initial visit, and the left-side hearing loss occurred 1 day before the visit. She had mild vertigo in the absence of otalgia, otorrhea, tinnitus, or other neurologic symptoms. On initial physical examination, both external auditory canals and tympanic membranes were normal in appearance. Pure-tone audiometry revealed both profound sensorineural hearing losses of 120 dB on the right side and 100 dB on left side (Fig. 1). An auditory brainstem showed no response in both ears. With a diagnosis of bilateral sudden sensorineural hearing loss, the patient was admitted to our hospital and underwent further evaluation including routine hematologic examination. Hematologic workup demonstrated a leukocyte count of 11 120/mm 3 with 17% monocyte, a hemoglobin level of 9 g/dL with normal reticulocyte count, and a platelet count of 38 000/mm 3. For evaluation of unexplained anemia, thrombocytopenia, and monocytosis, peripheral blood smear and bone marrow aspirate were performed, and those results were consistent with the diagnosis of MDS and refractory cytopenia with multilineage dysplasia. On cytogenic study, −7 chromosome abnormality was identified. Abdominal ultrasonogram finding showed no hepatosplenomegaly. A brain magnetic resonance image did not show any definite evidence of intralabyrinthine hemorrhage and cerebellopontine angel lesion (Fig. 2), but found small subacute infarction located at the right parieto-occipital lobe with multifocal subacute infarctions in both parieto-occipital lobes. Echocardiography results showed no particular signs other than cardiomegaly. According to consultation
☆
Conflict of interest: None. ⁎ Corresponding author. Department of Otolaryngology–Head and Neck Surgery, Chonbuk National University School of Medicine, Geum-am dong, Jeonju, Chonbuk 561-180, Korea. Tel.: +82 63 250 1980; fax: +82 63 250 1986. E-mail address:
[email protected] (E.J. Lee). 0196-0709/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjoto.2012.08.003
E.J. Lee, Y.J. Yoon / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 33 (2012) 782–783
Fig. 1. Pure-tone audiometry and speech discrimination test.
with the hematologic department, there seemed to be no special treatment other than conservative treatment such as platelet transfusion because of the patient's age with good performance status. For treatment of bilateral sudden sensorineural hearing loss, steroid therapy was prescribed for 1 week and later tapered. Even after the 1-week treatment, there was no hearing improvement. At 3 months after the initial visit, the patient has had no change in her hearing loss.
3. Discussion The MDS encompasses a series of hematologic conditions characterized by chronic cytopenia (anemia, neutrope-
783
nia, thrombocytopenia) accompanied by abnormal cellular maturation [3]. This patient was diagnosed as having refractory cytopenia with multilineage dysplasia, which is characterized by less than 5% bone marrow blast but severe dysplasia in trilineage hematopoietic cells. The symptoms of MDS differ greatly in degrees, and some of the patients do not show symptoms. In this case report, the patient did not complain about the symptoms caused by MDS per se. To date, there are only 28 cases reported on sudden hearing loss in chronic myeloid leukemia [2]. Most of them occurred unilaterally. Particularities of this case's patient were that, first, there occurred a sudden hearing loss after the initial symptom of MDS, and second, it was a bilateral hearing loss. The reasons for sudden hearing loss in patients with hematologic disorder, especially in leukemia, are leukemic infiltrate, hemorrhage, and hyperviscosity blood [4]. The major reason is believed to be hyperviscosity due to an increased blood cell counts. The mechanism is believed to be hyperinfiltration of white blood cells in the microcirculation. As for the patient in question, there were no features such as increased blood cell counts or hyperviscosity, but it was the very first and rare case of sudden hearing loss diagnosed as MDS. In this study, it is believed that as the hemorrhagic tendency increased due to decreased platelets, hemorrhage or massive blood loss of the inner ear may have led to hearing impairment of the right at first and then of the left 2 days later. In addition, we assumed that in our patient, sudden hearing loss was due to occlusion of small arteries of the vertebra-basilar system. However, the presumed reason was not proven by radiologic image. Treatment of MDS can be divided into symptomatic treatment and aggressive treatment, which is the basic treatment of illness [3]. In this case report, given the patient's old age and general physical conditions, only conservative treatment such as platelet transfusion was carried out. Unfortunately, we did not observe any improvement in hearing after steroid therapy. In conclusion, the case presented is unique in that, to the best of our knowledge, there are no reported cases of bilateral sensorineural hearing loss as an initial symptom of MDS. This case informs that MDS could be considered one of the possible etiologies in patients with acute sensorineural hearing loss. References
Fig. 2. Axial, T1-weighted magnetic resonance image scan with gadolinium contrast shows no cochlear pathology and cerebellopontine angle.
[1] Albitar M, Manshouri T, Shen Y, et al. Myelodysplastic syndrome is not merely “preleukemia”. Blood 2002;100:791. [2] Genden EM, Bahadori RS. Bilateral sensorineural hearing loss as a first symptom of chronic myelogenous leukemia. Otolaryngol Head Neck Surg 1995;113:499-501. [3] Golshayan AR, Jin T, Maciejewski J, et al. Efficacy of growth factors compared to other therapies for low-risk myelodysplastic syndromes. Br J Haematol 2007;137:125. [4] Tsai CC, Huang CB, Sheen JM, et al. Sudden hearing loss as the initial manifestation of chronic myeloid leukemia in a child. Chang Gung Med J 2004;27:629-33.
