ISSN 0971-7749

Vol 23 / Issue 1 / January - March 2017

Indian Journal of Otology

Indian Journal of Otology • Volume 23 • Issue 1 • January-March 2011 • Pages 1-00**

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 Case Report

Prelingual Auditory Verbal Agnosia, A Rare Condition

Violeta Necula, Cristina Mirela Stamate, Krishna Luchoo, Marcel Cosgarea
Department of Otorhinolaryngology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj‑Napoca, Romania

Abstract
Auditory agnosia, agnosia for speech is a rare condition, caused mostly by stroke, in adults, while in children it is often a sequel of herpes
simplex encephalitis or is associated with epilepsy, in Landau–Kleffner syndrome. This article presents a rare case of verbal auditory agnosia
as a result of meningoencephalitis, caused probably by herpes simplex virus. The patient’s developed encephalitis at the age of 1 year and
4 months. After her recovery, the family noticed the absence of language development. She was initially diagnosed with profound hearing
loss, and the hearing aids did not help her. The speech therapy developed her lip‑reading skills with a hard‑to‑understand pronunciation. Later,
audiometry showed a nearly normal peripheral auditory system and magnetic resonance imaging revealed the characteristic brain lesions in
both the temporal lobes, explaining the patient’s evolution. Assessment of children with delayed speech development must consider the central
hearing disorders as a differential diagnosis.

Key words: Auditory verbal agnosia, central deafness, herpes encephalitis

Introduction for bacteria or mycoplasma. After this episode, the family

Auditory agnosia is a rare auditory deficit due to bilateral
lesions of the primary auditory cortex or acoustic radiations.
It is divided into four categories: general auditory agnosia,
agnosia for speech, for environmental sounds and music.[1,2]
Auditory verbal agnosia is specific for speech sounds and
represent the inability to comprehend speech. Nonverbal
sounds are heard and comprehended.[1]
In adults, the most common causes of auditory agnosia are
cerebrovascular accident, dementia[3] or tumor within the third
ventricle, meanwhile, in children, possible causes include
herpes simplex encephalitis and Landau–Kleffner syndrome.[4]
Case Report
A 24‑year‑old female patient presented to our clinic for
audiological assessment with complaints of speech perception
impairment and difficulties in speech production. In childhood,
she was diagnosed with profound hearing loss, as a result of
which she started using hearing aids; however, she discontinued
them because they bothered her.
After a normal early development, at the age of 1 year and
4  months, she was diagnosed with meningoencephalitis of
unknown etiology. Cerebrospinal fluid cultures were negative
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noticed a decline in speech perception and production. She
was able to recognize some environmental sounds such as
doorbell, telephone, door knock, or the noise of fallen object.
She was trained intermittently by speech therapists developing
visual skills like lip‑reading and the language in some manner.
Clinical examination did not reveal any pathologiec aspect
except the delay in speech development and lack of speech
perception. Audiological assessment is presented in Figure 1.
Taking into considerations the audiological results with a
history of encephalitis and the absence of speech perception
the patient was suspected of central hearing loss of auditory
agnosia. The patient underwent a magnetic resonance
imaging  (MRI) examination, and bilateral temporal lobe
lesions were detected, predominantly on the right side
[Figures 2 and 3].
Address for correspondence: Dr. Violeta Necula,
Department of Otorhinolaryngology, “Iuliu Hatieganu” University of Medicine
and Pharmacy, 8th Babes Street, Cluj‑Napoca, Romania.
E‑Mail: neculav@yahoo.com
This is an open access article distributed under the terms of the Creative Commons
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For reprints contact: reprints@medknow.com

DOI: How to cite this article: Necula V, Stamate CM, Luchoo K,

10.4103/0971-7749.199499 Cosgarea M. Prelingual auditory verbal agnosia, A rare condition. Indian
J Otol 2017;23:46-8.

46 © 2017 Indian Journal of Otology | Published by Wolters Kluwer - Medknow

 Necula, et al.: Prelingual Auditory Verbal Agnosia
Figure 1: Puretone audiogram ‑ medium sensorineural hearing loss; auditory steady state response ‑ thresholds on 30–40 dB HL; auditory brainstem
response ‑ normal configurations and thresholds; Type A tympanogram; acoustic reflex present ipsi‑ and contralateral; distortion products otoacoustic
emissions with responses on both ears.
Neurological examination did not reveal any additional
impairment.
For a better communication, the patient continued to work
with a speech therapist, attempting to improve her lip‑reading
and pronunciation.
Discussion
This is a rare case of prelingual verbal auditory agnosia in a
young patient with severe speech production and perception
disorders, which occurred after an episode of encephalitis at
the age of 1 year and 4 months. The early onset of the disease
had severe repercussions in subsequent language evolution
because of lack of auditory feedback, necessary for speech
production. Unclear pronunciation, similar to a deaf person,
and childhood pure tone thresholds, which showed severe
to profound hearing impairment, enabled the patient to be
considered deaf–mute. The current audiological assessment
showed a medium hearing loss in contrast to the lack of
speech perception and a normal peripheral auditory system.
MRI confirmed brain lesions in both superior temporal gyri,
more pronounced on the right side.
In children, auditory agnosia is due to herpes encephalitis
or is associated with epilepsy in Landau–Kleffner
syndrome.[4] Herpes simplex is the most common cause of
acute sporadic encephalitis in children[5] and its incidence
Indian Journal of Otology  ¦  Volume 23  ¦  Issue 1 ¦ January-March 2017
is one case/250,000 population/year in the USA and
2.5/1,000,000 population/year in Sweden.[6] In the absence of
therapy, mortality exceeds 70%, and only 2.5% of surviving
individuals return to normal neurological function.[7] Herpes
simplex encephalitis is characterized by acute focal, necrotizing
brain lesions, particularly in the temporal and orbitofrontal
region. Our patient had bilateral lesions of temporal region,
more pronounced on the right, similar to the cases submitted
by Kaga et al.[8]
If the injury occurs before the language development, the lack
of auditory feedback prevents the speech development and
the communication mainly relies on visual cues. Word‑deaf
children are mute, and only few of them succeed to acquiring
adequate language.[9]
Clinical manifestations, in this case, were similar to
deaf‑mute and the voice quality was also affected. The
initial diagnosis was severe to profound hearing loss,
based on the child’s lack of response to sounds on puretone
audiogram; however, this was arrived without making a
complete objective assessment. Hearing aids did not help
the patient, which is understandable now when we found
that she could hear environmental sounds but could not
understand speech.
The patient was able to acquire only a poor language and
developed lip‑reading with the help of speech therapists. She
47
 Necula, et al.: Prelingual Auditory Verbal Agnosia
Figure 2: Fluid‑attenuated inversion recovery‑type acquisitions on
coronal plan: Sequelar pathological hypersignal on gyriform and
cortico‑subcortical junctional areas, in the posterosuperior temporal
gyrus, with an atrophic appearance (extremely thin cortex, linear), more
visible on the right side, secondary of inflammatory disease.
attended mainstream school, and she learnt to read and write;
however, her academic performances were poor.
Similar cases were presented by Kaga et al.[10] In one of the
reported cases, the onset was at 1  year and 2  months with
bilateral temporal lobe lesions, like in our case. Language
acquisition was difficult, communication was performed using
sign language, and the patient attended a special school for
deaf. Temporal lobe lesions were typical for agnosia.
Currently, children who do not cooperate for behavioral
testing are evaluated using objective electroacoustic tests.
A history suggestive of encephalitis or epilepsy can lead us to
think of a much rare condition which is the auditory agnosia.
In this situation, referring the patient to a neuropsychiatric
or a neurologic assessment and MRI of the brain could help
establish a diagnosis.
In this case, prelingual onset of the illness caused significant
communication disorder that limited language development.
The orientation of child to a special school would bring greater
benefits to developing communication‑based on sign language
and lip‑reading because hearing aids are useless.
Assessment of children with delayed speech development and
normal peripheral auditory system must consider auditory
agnosia as a differential diagnosis, a rare disease but with
devastating consequences.
48
Figure 3: The fluid‑attenuated inversion recovery‑type acquisition in
sagital plan reveals a peripheral hypersignal, gyriform but also junctional,
cortico‑subcortical, in posterosuperior temporal gyrus, more obvious
on the right side; quite obvious the atrophic, very thin appearance of
involved cortex.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Slevc  LR, Shell  AR. Auditory agnosia. Handb Clin Neurol
2015;129:573‑87.
2. Stefanatos  GA, Demarco  AT. Central auditoryprocessing disorders.
Encyclopedia of Human Behavior. Second ed. 2012. p. 441‑52.
3. Stefanatos  G. Changing perspectives on Landau‑Kleffner syndrome.
Clin Neuropsychol 2011;25:963‑88.
4. Shivashankar  N, Shashikala  HR, Nagaraja  D, Jayakumar  PN,
Ratnavalli  E. Pure word deafness in two patients with subcortical
lesions. Clin Neurol Neurosurg 2001;103:201‑5.
5. Baringer  JR. Herpes simplex virus encephalitis. In: Davis  LE,
Kennedy  PG, editors. Infectious Diseases of the Nervous System.
Oxford: Butterworth‑Heinemann; 2000. p. 139‑64.
6. Whitley  RJ, Roizman  B. Herpes simplex virus infections. Lancet
2001;357:1513‑8.
7. Tyler  KL. Herpes simplex virus infections of the central nervous
system: Encephalitis and meningitis, including Mollaret’s. Herpes
2004;11 Suppl 2:57A‑64A.
8. Kaga K, Kaga M, Tamai F, Shindo M. Auditory agnosia in children after
herpes encephalitis. Acta Otolaryngol 2003;123:232‑5.
9. Rapin I. Cortical deafness, auditory agnosia, and word‑deafness: How
distinct are they? Human Communication Canada1985;9:29‑37.
10. Kaga M, Shindo M, Kaga K. Long‑term follow‑up of auditory agnosia as
a sequel of herpes encephalitis in a child. J Child Neurol 2000;15:626‑9.
Indian Journal of Otology  ¦  Volume 23  ¦  Issue 1 ¦ January-March 2017