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.
1
Introduction
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 . 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 . 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.
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Case report
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 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.
