Rare disease

CASE REPORT

A cardiac cause for deafness
Kushal Naha,1 G Vivek,2 Ranjan K Shetty,2 Lorraine Simone Dias1
1

Department of Medicine,
Kasturba Medical College,
Manipal University, Manipal,
Karnataka, India
2
Department of Cardiology,
Kasturba Medical College,
Manipal University, Manipal,
Karnataka, India
Correspondence to
Dr G Vivek,
vivekgraman@gmail.com

SUMMARY
We describe a case of a 49-year-old diabetic man with a
history of myocardial infarction, presenting with deafness
for 2 weeks. Initial assessment by otorhinolaryngologists
was suggestive of sensorineural hearing loss.
Subsequently, the cardiac evaluation showed an apical
clot in the left ventricle. Careful neurological
reassessment and a cranial MRI yielded a diagnosis of
cortical deafness with auditory agnosia secondary to
bilateral temporal infarcts. Doppler and MRI ruled out
carotid artery thrombosis. The temporal infarcts were
therefore considered to be cardioembolic in origin.
Cardioembolic stroke is an important differential
diagnosis for patients presenting with neurological
symptoms on a background of cardiac disease.

BACKGROUND
Cardioembolic strokes account for up to 20% of all
ischemic strokes, but often produce disproportionately greater disability than non-embolic strokes
due to their potential for involving larger intracranial arteries.1 Characteristic features of cardioembolic strokes include infarcts of multiple discrete
vascular territories, and alternating right and left
hemispherical involvement.2 Recognition of the
cardioembolic stroke is essential not only because
of the morbidity associated with them, but also
because of the significant reduction in the risk of
stroke with appropriate antithrombotic therapy.1
The patient described in this report developed
bilateral temporal lobe infarcts after defaulting on
standard antiatherosclerotic medications including
low-dose aspirin. Although he attained only limited
recovery of hearing loss, rapid institution of antiplatelet and oral anticoagulation therapy prevented
further embolic events.
Isolated deafness is an uncommon manifestation
of cardioembolic stroke; a meticulous neurological
examination as well as a high index of suspicion is
essential for diagnosis, especially in persons with a
history of cardiac disease.

examination showed difficulty in comprehending
spoken words, and distinguishing musical notes
from environmental sounds. His speech was intact
and comprehension of written words was unimpaired. There were no other focal neurological deficits. The cardiovascular examination was essentially
normal.

INVESTIGATIONS
Routine laboratory tests revealed well-controlled
glycaemia (fasting blood glucose 135 mg/dL; glycoslylated haemoglobin 6%). Complete blood counts,
erythrocyte sedimentation rate and liver and renal
function tests were within normal limits.
Electrocardiography showed pathological q waves
in V1–3 with loss of R waves, and T wave inversion,
consistent with an old anterior wall myocardial
infarction. The patient was in sinus rhythm.
Transthoracic echocardiography demonstrated akinesia of the apical segment of the left ventricle with
hypokinesia of the interventricular septum and
anterior wall. A left ventricular apical clot was also
visualised. Mild left ventricular systolic dysfunction
was noted (ejection fraction 47%).
A cranial MRI showed a chronic infarct with
encephalomalacia and gliosis involving the right
temporal lobe, and a subacute infarct involving the
left temporal lobe with mild effacement of adjacent
sulci (figure 1). Intracranial flow voids were preserved. Carotid artery Doppler studies showed
normal flow velocities and spectral wave forms;
intimal plaques were not seen.
Audiometry revealed mild bilateral sensorineural
hearing loss inconsistent with the degree of hearing
impairment reported by the patient.

DIFFERENTIAL DIAGNOSIS
▸ Partial cortical deafness with auditory agnosia
secondary to cardioembolic infarction of bilateral temporal lobes.
▸ Sensorineural hearing loss.

TREATMENT
CASE PRESENTATION

To cite: Naha K, Vivek G,
Shetty RK, et al. BMJ Case
Rep Published online:
[please include Day Month
Year] doi:10.1136/bcr-2013201364

A 49-year-old Asian-Indian diabetic man presented
with the sudden onset of deafness in both ears for
2 weeks. There were no associated aural symptoms
such as tinnitus, giddiness or ear pain or discharge
and no other neurological symptoms. He had suffered a myocardial infarction 13 years before, but
had since defaulted on antiatherosclerotic medications. He claimed to be obedient on antidiabetic
drugs. He denied any history of smoking, alcohol
consumption or other forms of substance misuse.
General physical examination was unremarkable.
He was haemodynamically stable. Neurological

Naha K, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201364

Oral anticoagulation with warfarin was initiated for
cardioembolic stroke, with a target international
normalised ratio (INR) of 2.5. In addition, the
patient was restarted on antiplatelet therapy, statins,
ACE inhibitors and β-blockers for coronary artery
disease. Speech therapy was administered for his
hearing impairment.

OUTCOME AND FOLLOW-UP
Minimal subjective improvement in hearing loss
was reported by the patient, following speech and
pharmacotherapy. He was counselled regarding the
nature of his illness and the need for compliance to
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Rare disease

Figure 1 Cranial MRI showing a chronic infarct with encephalomalacia and gliosis involving the right temporal lobe, and a subacute infarct
involving the left temporal lobe with mild effacement of adjacent sulci ((A) T1 sequence, axial section; (B) T2 sequence, axial section).
therapy, and was subsequently discharged. He continues to
remain on follow-up at our hospital, with no further episodes of
neurological deterioration.

DISCUSSION
Syndromes of central auditory disorders include cortical deafness, pure word deafness, auditory agnosia for environmental
sounds and amusia.3 Of these syndromes, cortical deafness constitutes the most severe form, presenting with complete deafness
and preserved startle reflexes to loud sounds. Conversely, auditory agnosias are characterised by impaired perception of
certain classes of sounds. Examples of auditory agnosia thus
include pure word deafness, wherein the patient cannot distinguish speech sounds but can lip-read and can comprehend
written words, and agnosia for non-verbal environmental
sounds, with preserved comprehension of spoken speech. Other
specific forms of auditory agnosia include phonagnosia referring
to an inability to recognise individuals from the sound of their
voice4 and amusia indicating an impaired perception of music.5
The latter form of auditory agnosia was seen in our patient,
who had difficulty in distinguishing musical notes from environmental sounds. Pertinently, amusia is frequently associated with
other forms of auditory agnosia.5 Such was the case in this
instance, as our patient combined word deafness with amusia.
Auditory agnosia typically results from damage to bilateral
temporal lobes6 or specifically to the supratemporal gyri containing the primary auditory cortex, although there are isolated
reports of auditory agnosia in patients with unilateral temporal
2

lobe disease and even subcortical lesions.7 Some reports have
also demonstrated primarily apperceptive agnosia in patients
with right hemispherical lesions as opposed to associative
agnosia in those with left hemispherical lesions.8 Ischaemic
insult resulting from stroke is a major cause of temporal lobe
injury; other aetiologies include head trauma, congenital malformations, tumours, encephalitides and neurodegenerative
disorders.9
By their very nature, auditory agnosias respond poorly to
treatment. Therapeutic options including speech therapy and
training in lip-reading can produce limited improvement in
communication skills. Nevertheless, as this case report demonstrates, such agnosias can, albeit rarely, indicate an underlying
source of emboli with definite therapeutic options.

Learning points
▸ Embolic stroke should be considered in patients with
infarction of discrete bilateral cerebral arterial territories,
especially on a background of cardiac disease.
▸ Bilateral temporal lobe infarction can present with cortical
deafness, although unilateral temporal lobe involvement may
be clinically silent.
▸ Careful neurological evaluation for auditory agnosia can
provide critical clues for diagnosing cortical deafness.
Naha K, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201364

Rare disease
Contributors KN, LSD, GV and RKS were involved in the concept, design,
definition of intellectual content and literature search. KN, LKD and GV were in
addition involved in the preparation, editing and review of the manuscript. GV and
RKS were involved in clinical care of the patient.
Competing interests None.

3
4
5

Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.

6
7

REFERENCES
1
2

Freeman WD, Aguilar MI. Prevention of cardioembolic stroke. Neurotherapeutics
2011;8:488–502.
Arboix A, Alió J. Cardioembolic stroke: clinical features, specific cardiac disorders and
prognosis. Curr Cardiol Rev 2010;6:150–61.

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9

Levine RA, Hausler R. Auditory disorders in stroke. In: Bogousslavsky J, Caplan LR.
eds Stroke syndromes. 2nd edn. Cambridge University Press, 2001:144–61.
Hailstone JC, Crutch SJ, Vestergaard MD, et al. Progressive associative phonagnosia:
a neuropsychological analysis. Neuropsychologia 2010;48:1104–14.
Särkämö T, Tervaniemi M, Soinila S, et al. Cognitive deficits associated with acquired
amusia after stroke: a neuropsychological follow-up study. Neuropsychologia
2009;47:2642–51.
Satoh M, Takeda K, Murakami Y, et al. A case of amusia caused by the infarction of
anterior portion of bilateral temporal lobes. Cortex 2005;41:77–83.
Suh H, Shin YI, Kim SY, et al. A case of generalized auditory agnosia with unilateral
subcortical brain lesion. Ann Rehabil Med 2012;36:866–70.
Ayotte J, Peretz I, Rousseau I, et al. Patterns of music agnosia associated with
middle cerebral artery infarcts. Brain 2000;123:1926–38.
Boattini M, Arnone G, Procaccianti G. Auditory agnosia in a patient with nephrotic
syndrome. BMJ Case Rep. Published online: 14 Jan 2014. doi:10.1136/bcr-2012-008164

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