Bilateral Deafness of Vascular Origin
RAMESH K. KHURANA, M.D.,

PHILIP P. O ' D O N N E L L ,

M.D.,

CHARLES M. SUTER, P H . D . , A N D M. INAYATULLAH,

M.D.

521

SUMMARY Two patients with reversible bilateral deafness of vascular etiology are reported. Serial
audiometric studies in the first patient before and after a brainstem stroke indicated retrocochlear auditory
dysfunction. The second patient showed bitempora] infarction on cranial CT scan which was subsequently confinned postmortem.
Stroke, Vol 12, No 4, 1981

REPORTS of bilateral lesions causing auditory
deficit in man are infrequent.1 In 1967, for example,
Jaffe reported 4 patients with bilateral deafness
among 143 patients who developed sudden sensorineural hearing loss.1 Deafness of vascular etiology
is stated to occur unilaterally and permanent impairment of hearing is a frequent sequel.3 In this paper we
describe 2 patients with reversible, sudden bilateral
deafness of vascular origin.

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Patient 1
At age 50, a hypertensive black man developed dizzy spells. These spells occurred irregularly, 3 to 4
times a day and lasted 3-4 minutes. They were
described as a "feeling of being drunk" and were
associated with low-pitched tinnitus and slurred
speech. Neurological examination, including visual
fields and optokinetic nystagmus, was normal. Electronystagmography was normal and audiometry
revealed mild, bilateral, high frequency sensorineural
hearing loss with good speech discrimination (see
table). He was prescribed aspirin. Two months later
he complained of aggravation of episodic dizziness,
double vision, numbness marching down the left arm
and band-like headaches while using the right hand for
painting walls and ceiling. His blood pressure was
110/80 mm Hg in both arms; the patient being on
thiazide diuretics and potassium supplement. Neurological examination was again normal. Roentgenograms of the skull revealed calcification of the basilar
artery posterior to the clivus and of the carotids overlying the sella turcica. Brain scan, electroencephalogram and arch aortogram were normal. Cerebrospinal fluid examination, including glucose and protein
values, was also normal.
Two weeks later, after having used the bathroom,
the patient suddenly felt vertiginous with a tendency to
fall either sideways, forward or backward. He complained of roaring in both ears and deafness. He
denied dysarthria, dysphagia, diplopia, focal weakness, sensory loss, or loss of consciousness. General
physical examination was normal. Neurological exFrom the Department of Neurology (Drs. Khurana and
O'Donnell), Otolaryngology (Dr. Sutcr) and Medicine (Dr.
Inayatullah), University of Maryland Hospital, Baltimore, MD
21201.
Reprints: Dr. Khurana, Dept. Neurology, University of
Maryland School of Medicine, 22 South Greene St., Baltimore,
MD 21201.

amination disclosed an alert individual with normal
higher cortical functions. Examination of cranial
nerves revealed bilateral counterclockwise rotatory
nystagmus as well as vertical nystagmus, both accentuated upon standing. He had bilateral hearing loss,
right greater than left; and cold caloric stimulation of
the left ear caused nystagmus to the right while the
right ear stimulation produced no response. Examination of the motor and sensory systems, including plantar responses, were normal. His station was broadbased and ataxic, with a tendency to fall to the left.
Tests of coordination revealed an intention tremor of
the left upper extremity. Laboratory data on admission included normal results of complete blood count,
electrolytes, glucose and electrocardiogram.
Selective cerebral angiography showed normal
carotid circulation. The arch study revealed marked
stenosis of the right vertebral artery at its origin but
this artery could not be selectively catheterized. Selective angiography of the left vertebral artery showed
significant stenosis with minimal flow into the
posterior fossa. The intracranial portion of the basilar
artery could not be visualized.
Audiometry revealed bilateral sensorineural deficit
with poor speech discrimination on the right (table).
There was a type IV Bekesy audiogram in the right ear
as well as total loss of the acoustic reflex in that ear. In
the left ear there was loss of the acoustic reflex at 2
and 4 KHZ. Acoustic reflex decay was present at 1
KHZ in the left ear.
The patient continued to improve and 2 weeks later
neurologic findings included rotary nystagmus to the
left, improved hearing, broadbased gait and a tendency
to fall to the left. Audiometry demonstrated mild improvement in speech reception threshold (table).
In another 2 weeks, the patient developed vertical
upward nystagmus, horizontal nystagmus, left peripheral seventh nerve paresis, right hemiparesis with
a right Babinski sign and incoordination of the right
extremities. The patient continued to improve and was
discharged on aspirin.
During the next 3 months, he was able to hear people talk and able to walk unaided. Nine months after
discharge, he showed mild right hemiparesis, normal
plantar responses and normal cerebellar and sensory
findings. Audiometry showed recovery of hearing to
the pre-infarction level (table). There was a type II
Bekesy audiogram in the right ear and the acoustic
reflex had returned except at 4 KHZ. There was no
acoustic reflex decay in either ear.

STROKE

522

VOL

12, No

4, JULY-AUGUST

1981

TABLE Audiometric Results: Patient 1
Time

3 months before stroke
3 days after stroke
16 days after stroke
10 months after stroke

Hearing Lou
500
260

20/20
65/35
45/40
20/5

10/10
70/35
45/35
20/5

1000

dBHL
2000

AD/AS
8000H1
4000

15/15
75/35
50/25
10/5

25/25
60/40
55/30
25/25

30/35
70/55
50/55
35/40

One year later, brainstem evoked audiometry was
performed. His behavioral thresholds for the click
stimulus were 30 and 25 dBHL, AS and AD respectively. Wave V latencies and I-V latency differences
were normal for both sides. However, there appeared
to be a consistent reduction in wave III amplitude for
the right ear stimulation as compared to the left.

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Patient 2
A 24-year-old right-handed black man suffered the
sudden onset of a left hemiparesis with dysarthria.
Serological tests for syphilis, erythrocyte sedimentation rate, lupus erythematosus cell preparation,
antinuclear antibody titers, blood culture, echocardiography and cerebrospinal fluid examination were
normal. The electroencephalogram showed a right
frontotemporal delta wave focus. Cranial computed
tomography revealed a right temporal infarction.
Multiple distal branch occlusions of the right middle
cerebral artery complex were evident on cerebral
angiography. The patient improved gradually.

40/40
75/60
80/55
55/50

SRT

PB
%

15/10 92/92
70/35 Poor/92
50/25 28/84
15/5
96/92

Five months later, the patient experienced sudden
deafness while watching television. Physical examination now revealed a hitherto undetected loud holosystolic murmur at the cardiac apex. He was afebrile
and without signs of peripheral emboli. Neurological
examination showed the patient to be completely deaf
for spoken language as well as non-language sounds.
He could not be startled by sudden loud noises. Communication was achieved by writing notes and occasional paragraphic errors were noted. Spontaneous
speech was fluent with infrequent phonetic paraphasias. The remainder of the neurological examination was normal except for the residual left
hemiparesis.
A repeat cranial computed scan showed a new area
of damage in the left temporoparietal region (fig. 1).
Correspondingly, new theta activity in the left midtemporal area was present on the electroencephalogram. The cerebrospinal fluid was normal. A repeat
echocardiogram revealed an intracardiac mass attached to the anterior mitral valve leaflet. Intravenous heparin was started.
On the second hospital day, brainstem evoked
audiometry was performed with 70 decibel rarefaction
clicks delivered monaurally at a rate of 10.3 clicks per
second. Well-defined responses with normal absolute
latencies of waves I, III, and V were obtained
bilaterally. The patient's behavioral hearing threshold
could not be tested.

alii inf«r«t

dTibolic infarction

FIGURE 1. Computerized axial tomogram showing temporoparietal lesion on the left and temporal lesion on the right.

-*

FIGURE 2. Horizontal section of the cerebral hemispheres
demonstrating tumor emboli in the left superior temporal
and the right middle frontal convolutions. An area of
encephalomalacia is seen in the right superior temporal
gyms.

BILATERAL DEAFNESS OF VASCULAR ORIGIN/AT/iwana et al.
On the fourth hospital day a myxomatous tumor
was removed from his mitral valve and a prosthetic
valve inserted. In the immediate postoperative period
the patient's hearing was improved so that he could
comprehend spoken language but he continued to encounter difficulty conversing if extraneous noises, e.g.
television, were present. He declined further neurootologic assessment. The final histologic diagnosis of
the tumor was malignant myxosarcoma.
Three weeks later, the patient died of an acute pcricardial tamponade. Postmortem examination disclosed tumor emboli in the left superior temporal and
right middle frontal convolutions (fig. 2). Microscopic examination of the emboli showed myxosarcomatous tissue identical to the surgical pathologic
specimen from the heart. An old infarction was also
present in the right superior temporal gyms. Sections
of the brainstem were normal.
Discussion

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These 2 patients illustrate the 2 different sites of
vascular damage to the auditory pathways that
produced bilateral deafness. The first patient
developed deafness due to brainstem involvement as
indicated by the absent caloric response of the right
ear, vertical nystagmus, a left peripheral facial
paresis, left-sided intention tremor and right hemiparesis. In addition, the audiometric studies demonstrated retrocochlear dysfunction.4 Deafness and
neurologic dysfunction gradually improved.
Our second patient developed transient deafness for
all sounds due to bitemporal infarction from a myxosarcoma of the mitral valve as documented by the
cranial CT scan and autopsy."- * The normal brainstem auditory evoked responses suggested the intactness of auditory pathways from the acoustic nerve
to inferior colliculi7 and no lesion affecting the brainstem was found postmortem. These patients suggest
that vertebral-basilar insufficiency should be included
in the differential diagnosis of bilateral sudden
deafness' and that computed tomography and neurootological studies can help in the precise localization
of the lesion to the auditory pathways.
It is interesting to note that compared to vestibular
disturbances cochlear disturbances in vertebrobasilar

523

insufficiency are rare.9 For example, Fisher, in his
study of 112 cases of vertebrobasilar insufficiency,
reported dizziness in 86 patients while only 10 patients
complained of deafness.8 This contrast becomes more
apparent when one observes the anatomical proximity
of the peripheral and central cochlear and vestibular
pathways, lack of collaterals to the labyrinthine artery
and the limited cochlear tolerance for ischemia in the
experimental and human studies.10"11 Could it be that a
unilateral or incomplete bilateral deafness goes unnoticed by the patient and the physician? If this is so,
audiometric evaluation of patients with vertebralbasilar insufficiency may show the incidence of partial
deafness to be higher than previously reported.
Acknowledgment
The authors wish to thank Mrs. Anne Dimiceli for secretarial
help.

References
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Neurology (Minneap) 25: 286-289, 1975
2. Jaffe BF: Sudden deafness, an otologic emergency. Arch
Otolaryng 86: 55-60, 1967
3. Fisher CM: Vertigo in cerebrovascular disease. Arch Otolaryng
85: 529-534, 1967
4. DeJong RN: The Acoustic Nerve. In The Neurologic Examination, Chapter 14. Hagerstown, Maryland, Harper and Row,
1979, pp 199-224
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