Unusual presentation of more common disease/injury

Case report

Auditory agnosia caused by bilateral
putamen haemorrhage
Tomohito Sugiura, Tsuyoshi Torii
Department of Neurology,
National Hospital Organization
Kure Medical Center, 3-1
Aoyama, Kure city, Japan
Correspondence to
Dr Tomohito Sugiura,
​sugiurat@​kure-​nh.​go.​jp and Dr
Tsuyoshi Torii, ​toriit@​kure-​nh.​
go.​jp
Accepted 7 December 2017

Summary
A 55-year-old right-handed man with a history of
hypertension suddenly fell and developed right
hemiparesis. Neurological examination revealed that he
was alert, but did not appropriately respond to verbal
questions and commands. Detailed examination revealed
that he could correctly respond to written commands.
His speech was almost fluent, showing no paraphasia
and normal articulation. His written sentences were
legible. Pure tone audiometry showed that his auditory
acuity was relatively preserved. His brainstem auditory
evoked potential components from I to V were
recorded bilaterally with normal latency. Cerebral CT
demonstrated fresh bleeding in the left putamen and
an old haemorrhage on the opposite side. He was
treated by antihypertensive therapy and rehabilitation.
Although there remained mild sensory deficit on his right
extremities and he felt a slight noise during conversation,
he had little difficulty with verbal communication when
he was transferred to another hospital on day 38.

Auditory agnosia or cortical deafness generally refers to a condition that is characterised by
defective comprehension of both verbal and environmental sounds despite a preserved hearing
ability. Currently, auditory agnosia is divided into
four categories: pure word deafness, pure auditory
non-verbal agnosia, phonagnosia and pure amusia.1
There may be one or a mixture of these deficits.
There have been some reports and studies about
this interesting symptom,2 3 and the causative
lesion is now thought to be bilateral transverse
temporal gyri (Heschl’s gyri) or auditory radiations.
Cerebrovascular diseases are the most commonly
reported cause.4 5 Among patients who had general
stroke, however, auditory agnosia is a very rare
manifestation.6
Here, we report a patient with auditory agnosia
that involved word deafness, which was caused by
bilateral putamen haemorrhage.

Case presentation
Background

This report highlights auditory agnosia caused by
stroke. It is a rare manifestation and is easily misdiagnosed as sensory aphasia.

To cite: Sugiura T, Torii T. BMJ
Case Rep Published Online
First: [please include Day
Month Year]. doi:10.1136/
bcr-2017-222535

Figure 1 Brain CT image on admission. There was fresh
haemorrhage in the left putamen. The bleeding expanded
over the putamen slight posteriorly and laterally, but it
did not apparently involve the transverse temporal or
superior temporal gyri (A–D). An old haemorrhage in the
right putamen was also apparent (A–C).

A 55-year-old right-handed man with a history
of hypertension suddenly fell and developed
right hemiparesis, for which he was admitted to
our hospital. He also had an old haemorrhage in
the right putamen 7 years ago and had recovered
without any disabilities. On admission, the general
physical examination was normal except that his
blood pressure was 144/88 mm Hg. Neurological
examination revealed that he was alert, but did
not appropriately respond to verbal questions and
commands. Right mild hemiparesis and sensory
deficit were apparent. Detailed examination
revealed that he could correctly respond to written
commands. His speech was almost fluent, showing
no paraphasia and normal articulation. Repetition
was difficult, but naming and reading were normal.
He had messy writing because of right-sided weakness, although his written sentences were legible.
There was no other abnormality including apraxia,
visual agnosia and unilateral spatial neglect. He
spoke about his own symptom as, ‘I realise that
someone has told me something, but I cannot
understand what they said. It sounded like some
noise’. He also mentioned that the ambulance ran
without a siren during his transport.

Investigations
Auditory recognition tests showed that he was not
able to discriminate most vowels such as ‘a’ or ‘u’.
Discriminating polysyllabic words was slight better
than vowels because only one syllable of the word

Sugiura T, Torii T. BMJ Case Rep 2017. doi:10.1136/bcr-2017-222535

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Unusual presentation of more common disease/injury
Learning points
►► Auditory agnosia including word deafness may mimic sensory

aphasia at initial presentation. Because reading ability is
preserved, written commands help to distinguish these two
symptoms.
►► Agnosia of environmental sounds or music is not as apparent
as word deafness. Active assessment may be required in
these cases.
►► Most cases of auditory agnosia are caused by bilateral
temporal lobe lesion. However, bilateral putamen lesion may
also cause this symptom.
►► Prognosis of auditory agnosia which caused by stroke vary
in patients. Long-term rehabilitation may be required for
improvement.

Discussion

Figure 2 Brain MRI on day 9. There was subacute haemorrhage in
the left putamen on T2-weighted image (A–D). The size of lesion was
not so different from the CT image on admission. The mild surrounding
oedema remained.

which he was able to recognise might help his understanding.
In contrast to listening words, his auditory acuity seemed to be
relatively preserved, for example, he responded well to a noise
such as cracking a finger. His speech ability was almost spared.
He was capable of explaining scenes of photo cards. Laboratory
findings were almost normal. Cerebral CT demonstrated fresh
bleeding in the left putamen and an old haemorrhage on the
opposite side (figure 1).

Treatment
He was treated by antihypertensive therapy and rehabilitation.

Outcome and follow-up
His symptom gradually improved during the first week. A standard language test of aphasia (SLTA) on day 9 revealed that the
percentage of correct answers was 95% in hearing but some
repeats and cues were necessary for him to answer. Cerebral
MRI on the same day showed subacute left putamen haemorrhage with mild surrounding oedema on T2-weighted image
(figure 2). Subsequent otological examination revealed no
abnormality in his tympanic membrane. Pure tone audiometry
on day 10 showed a mild bilateral sensorineural hearing loss.
His speech reception threshold and speech discrimination ability
in the right and left ears were 37 dB and 50 dB, 60% and 20%,
respectively. These results improved to 20 dB and 20 dB, 95%
and 65%, respectively on day 30. His brainstem auditory evoked
potential (BAEP) components from I to V were recorded bilaterally with normal latency.
Although there remained mild sensory deficit on his right
extremities and he felt a slight noise during conversation, he had
little difficulty with verbal communication when he was transferred to another hospital on day 38.
2

The hearing problem in this patient was an impairment of
understanding verbal sounds, whereas auditory acuity and BAEP
response were relatively preserved. These findings indicated that
this symptom was word deafness and was attributed to a cerebral
auditory pathway, which involved primary auditory cortices,
auditory radiations and medial geniculate bodies. Most cases of
auditory agnosia were caused by bilateral temporal lobe lesion.7
However, the CT finding in this patient demonstrated bilateral
putamen haemorrhage. This indicated that auditory radiations
near putamen caused his symptom. There are few reports of
image analysis about basal ganglia lesion and auditory agnosia.8 9
According to one report, the white matter immediately ventral
and lateral to the posterior half of the putamen may be a causative lesion,9 and this patient seemed to have a similar condition
(figure 1). It is possible that this region might be important to
the neural network in auditory radiation and for word deafness.
His word deafness gradually improved and he was able to
communicate verbally within 1 month. There are a few case
reports which describe prognosis of auditory agnosia.5 6 Some
patients do improve partially and others do not within several
months. It is thought that the size of lesion and the surrounding
oedema are related to the duration of the disability, permanent
or non-permanent. However, the brain MRI in this case showed
the oedema still remained despite beginning improvement on
day 9. This implies the surrounding oedema is not so important
for causing symptom.
This patient did not recognise some environmental sounds in
the acute phase, for example, sirens of the ambulance or chimes
in the hospital. This symptom disappeared in the first week and
we could not test auditory recognition about environmental
sounds before the recovery. He did not complain of phonagnosia
or amusia. However, other than word deafness, patients are not
often aware of the symptom of auditory agnosia, so that active
assessment may be required.
In conclusion, bilateral putamen haemorrhage may cause
auditory agnosia including word deafness. Close attention to the
symptom and the brain image is important for appropriate diagnosis and treatment.
Contributors Both authors assessed and managed this case. TS conceived the idea
of writing the case, obtained informed consent from the patient and drafted the first
version of the manuscript. TT edited the article.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
Sugiura T, Torii T. BMJ Case Rep 2017. doi:10.1136/bcr-2017-222535

Unusual presentation of more common disease/injury
© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article)
2017. All rights reserved. No commercial use is permitted unless otherwise expressly
granted.

References
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Amsterdam: Elsevier, 2015.
2 Graham J, Greenwood R, Lecky B. Cortical deafness-a case report and review of the
literature. J Neurol Sci 1980;48:35–49.
3 Dick F, Saygin AP, Galati G, et al. What is involved and what is necessary for complex
linguistic and nonlinguistic auditory processing: evidence from functional magnetic
resonance imaging and lesion data. J Cogn Neurosci 2007;19:799–816.

4	Shindo M. Cortical deafness and auditory agnosia. Clin Neurosci 2006;24:518–20.
5 Ishii K, Ueda Y, Ohkoshi N, et al. Cortical auditory disorder caused by bilateral temporal
infarctions. Intern Med 1995;34:801–5.
6 Murayama H, Matsuo S, Nishimura N, et al. Two cases of cortical deafness and auditory
agnosia with bilateral temporal lesions. Jpn J Stroke 2010;32:190–6.
7 Kaga K, Takegoshi H, Hayashi A. Central auditory information processing in patients
with bilateral auditory cortex or auditory radiation – sounds of speech, music and
environment. Higher Brain Function Research 2008;28:224–30.
8 Kaga K, Kurauchi T, Nakamura M, et al. Magnetoencephalography and positron
emission tomography studies of a patient with auditory agnosia caused by bilateral
lesions confined to the auditory radiations. Acta Otolaryngol 2005;125:1351–5.
9 Tanaka Y, Kamo T, Yoshida M, et al. ’So-called’ cortical deafness. Clinical,
neurophysiological and radiological observations. Brain 1991;114:2385–401.

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