Bilateral sudden sensorineural hearing loss as an initial presentation of
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.
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 macroglob-
ulinemia . 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.
2. 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
with 17% monocyte, a hemoglobin
level of 9 g/dL with normal reticulocyte count, and a platelet count of
. For evaluation of unexplained anemia, thrombocyto-
penia, 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 hepatosple-
nomegaly. A brain magnetic resonance image did not show any
definite evidence of intralabyrinthine hemorrhage and cerebellopon-
tine 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
Available online at www.sciencedirect.com
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 33 (2012) 782 –783
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
E-mail address: firstname.lastname@example.org (E.J. Lee).
0196-0709/$ – see front matter © 2012 Elsevier Inc. All rights reserved.