Sudden deafness as a manifestation of the rupture of a
cerebral arteriovenous malformation
C. A. PÉREZ FERNÁNDEZ, MD, M. ARMENGOT CARCELLER, PhD, J. R. ALBA GARCÍA, MD,
C. G. LOZANO DE ARNILLA GARCÍA, MD, and J. BASTERRA ALEGRÍA, PhD, Valencia, Spain

S udden deafness is defined as sudden or rapidly
progressing neurosensory hypoacusis of at least 30
dB in 3 contiguous audiometric frequencies. In
most cases the underlying cause is not known;
hearing may or may not recover, and recurrence is
possible.1,2
A number of etiologies have been proposed,
including viral infection, particularly following
pseudo-influenza processes of the upper airways.
The most frequently implicated viruses are adenovirus, coxsackievirus, influenza, rubella, measles,
herpes, and parotiditis virus.3 Vascular pathology
is the most frequent cause in elderly patients. In
this context, 3 vascular mechanisms have been
defined4: hemorrhage, thrombosis, and vascular
spasm. Other less commonly cited causes include
autoimmune diseases and allergies. A characteristic common to all of these mechanisms is cochlear
ischemia, which produces hypoxia-anoxia of the
organ of Corti.
Occasionally, sudden deafness may be attributable to life-threatening central nervous disorders.
The present study describes the case of a patient
with sudden deafness due to an exceptional and
serious cause: the rupture of a cerebral arteriovenous malformation (AVM).
CASE REPORT
A 53-year-old man presented to the Emergency
Service with right ear tinnitus and hypoacusis of
sudden onset, without other symptoms. Audiometry identified right neurosensory hypoacusis with
an auditory threshold of 70 dB. The patient was
admitted to the Ear, Nose and Throat Service for
From the General Hospital and Medical School, Valencia,
Spain.
Reprint requests: C. Aranzazu Pérez Fernández, C/Avenida
Principal 61, 24271 Llamas de la Ribera, Leon, Spain; e-mail,
arapi61@hotmail.com.
Otolaryngol Head Neck Surg 2003;128:592-4.
Copyright © 2003 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc.
0194-5998/2003/$30.00 ⫹ 0
doi:10.1016/mhn.2003.138
592

intravenous vasodilatory treatment. In the hours
following admission, the patient developed an important headache with vomiting. A brain computed tomography (CT) scan was therefore requested, which showed the presence of a right
subarachnoid hemorrhage (Fig 1A). The patient
was moved to the Neurology Service for continued vasodilatory treatment and evaluation. Magnetic resonance imaging (MRI) and magnetic resonance angiography showed the presence of an
AVM in the right temporo-parieto-occipital region
(Fig 1B and C). Nimodipine was administered
intravenously, followed by improvement in hearing and headache. The patient is presently stable,
with occasional headaches, and is pending surgery
or selective embolization.
DISCUSSION
Sudden-onset deafness is not an exceptional ear,
nose, and throat emergency. The symptoms are
sudden hypoacusis accompanied by tinnitus. Approximately 30% of patients also present dizziness
with a gyrating sensation and instability, associated with nausea and vomiting– generally related
to severe loss of hearing.5 A correct anamnesis is
important in such cases, placing special emphasis
on the possible antecedents of cardiopulmonary
disease, endocrine and metabolic disorders (diabetes, hypercholesterolemia), and neurologic pathology (cerebrovascular stroke, etc).
Audiometry can define the degree of hearing
loss. A basic neurologic examination is required to
exclude possible neurologic deficits capable of
inducing central pathology.
The appearance of intense headache and vomiting in such situations is suggestive of a central
neurologic origin, such as acute hemorrhage. The
most common presenting symptom related to subarachnoid hemorrhage is described as “the worst
headache of a person’s life” in 85% of cases. The
onset of the headache may or not be associated
with a brief period of consciousness, seizure, vomiting, or meningismus. Hearing loss is related in
5% of patients.6

Otolaryngology–
Head and Neck Surgery
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PÉREZ FERNÁNDEZ et al 593

Fig 1. A, Axial CT view showing a hyperdense image corresponding to subarachnoid hemorrhage at the perimesencephalic level. B, T2-weighted axial MRI acquisition showing a wavy circumscribed image with signal vacancy and no mass
effect, in the right hemisphere at temporo-parieto-occipital level, corresponding to a very large AVM. C, Magnetic
resonance angiography (second acquisition, axial plane). Note drainage of the malformation through thick veins that
in turn empty into the great cerebral vein, straight sinus, and superior longitudinal sinus.

Otolaryngology–
Head and Neck Surgery
April 2003

594 PÉREZ FERNÁNDEZ et al

In our patient, hypoacusis was the manifestation
of subarachnoid hemorrhage secondary to rupture
of a cerebral AVM. The inner ear may have been
damaged in 2 ways: (1) by the presence of erythrocytes surrounding the nerves within the inner
auditory canal and following the nerve fibers on
entering the Rosenthal canal and in the fluids of
the inner ear7 and/or (2) thorough vasospasm originating at brain level in situations of hemorrhage.8
The fact that hearing recovered after the provision
of vasodilatory medication points to the latter
cause in our patient.
A CT study should be made in patients with
these symptoms to exclude subarachnoid bleeding.
If the presence of hemorrhage is confirmed, angiography or magnetic resonance angiography is
indicated after stabilization of the patient, to define
the underlying cause.
Most nontraumatic subarachnoid hemorrhages
(85%) are due to the rupture of intracranial saccular aneurysms, whereas 10% are attributable to
cerebral AVMs and 5% to other causes.9
AVMs are the most dangerous congenital vascular malformation. They consist of anomalous
vessels that join the arterial and venous systems.
Most constitute arteriovenous fistulas in which the
participating vessels increase in size and grow
over time. Intracranial AVMs occur in about 0.1%
of the population, which is one tenth the incidence
of intracranial aneurysms.10 The main clinical
signs and symptoms are headache and seizures. In
a large percentage of cases, AVMs initially manifest as intracerebral hemorrhage.11 Such bleeding
is generally intraparenchymal and is less commonly found at the subarachnoid level.
The treatment of AVMs is indicated whenever
associated bleeding has occurred or the patient has
epileptic seizures. The therapeutic options range
from surgery12 to selective embolization and ste-

reotaxic irradiation.13 Patient monitoring usually
suffices in asymptomatic cases.
CONCLUSIONS
Sudden deafness can be the first manifestation of
severe central pathology such as acute subarachnoid
hemorrhage. In cases of sudden deafness accompanied by some other atypical sign or symptom (especially neurologic), a central cause should be evaluated as a first etiologic possibility.1
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