Auditory verbal agnosia

PURPOSE OF REVIEW: To investigate the neurofunctional correlates of pure auditory agnosia and its varieties (global, verbal, and nonverbal), based on 116 anatomoclinical reports published between 1893 and 2022, with emphasis on hemispheric lateralization, intrahemispheric lesion site, underlying cognitive impairments. RECENT FINDINGS: Pure auditory agnosia is rare, and observations accumulate slowly. Recent patient reports and neuroimaging studies on neurotypical subjects offer insights into the putative mechanisms underlying auditory agnosia, while challenging traditional accounts...

Chromosome 9 open reading frame 72 ( C9orf72 ) gene pathogenic variants have been typically associated with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), but recent studies suggest their involvement in other disorders. This report describes a family with an autosomal dominant pattern of inheritance of progressive verbal auditory agnosia due to GGGGCC repeat expansion in C9orf72. A 60-year-old right-handed male truck driver presented with slowly progressive poor speech perception for 8 years, which became most troublesome when receiving verbal orders over the phone...

Following prolonged neglect during the formative decades of behavioural neurology, the temporopolar region has become a site of vibrant research on the neurobiology of cognition and conduct. This turnaround can be attributed to increasing recognition of neurodegenerative diseases that target temporopolar regions for peak destruction. The resultant syndromes include behavioural dementia, associative agnosia, semantic forms of primary progressive aphasia and semantic dementia. Clinicopathological correlations show that object naming and word comprehension are critically dependent on the language-dominant (usually left) temporopolar region, whereas behavioural control and non-verbal object recognition display a more bilateral representation with a rightward bias...

Functional neurological symptom disorder (FNSD), otherwise known as conversion disorder (CD), is a condition in which neurological deficits cannot solely be explained by medical pathology. Auditory verbal agnosia (AVA) is the inability to understand speech. While these two conditions are well-documented independently, a case of FNSD manifesting as AVA has not been previously reported. We present a 19-year-old patient, with a history complicated by congenital cardiomyopathy resulting in chronic heart failure with reduced ejection fraction and alpha-thalassemia, who demonstrated these symptoms...

Some patients with primary progressive aphasia (PPA) present with various types of hearing deficits. Research on the auditory function and speech sounds in PPA, including temporal, phonemic, and prosodic processing, revealed impairment in some of these auditory processes. Many patients with PPA who present with impaired word recognition subsequently developed non-fluent variant PPA. Herein, we present a patient with semantic variant PPA (svPPA) who demonstrated impaired verbal word discrimination. Audiological examinations revealed normal auditory brainstem responses and slightly impaired pure-tone perception...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Introduction: Verbal auditory agnosia is rarely caused by mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Lactate acidosis, which is the adverse effect of metformin, has proposed links to mitochondrial dysfunction and may trigger clinical features of mitochondrial diseases. Case Presentation: A 43-year-old right-handed man presented to our emergency department with acute onset fever and headache accompanied by impaired hearing comprehension...

No abstract text is available yet for this article.

We report a case study of cognitive linguistic treatment in a teenager with chronic severe Landau Kleffner Syndrome. The effect of speech and language therapy in LKS is rarely examined and our case is unique in that we use an effective approach in adult aphasia to treat language deficits in aphasia in LKS. The results show successful acquisition of a considerable amount of new words as well as improved communication in daily life. However, auditory verbal agnosia, the most prominent feature in LKS, persisted...

A 66-year-old right-handed female medical doctor suffered two consecutive cardioembolic strokes, initially affecting the right frontal lobe and the right insula, followed by a lesion in the left temporal lobe. The patient presented with distinctive phenomenology of general auditory agnosia with anosognosia for the deficit. She did not understand verbal requests and her answers to oral questions were fluent but unrelated to the topic. However, she was able to correctly answer written questions, name objects, and fluently describe their purpose, which is characteristic for verbal auditory agnosia...

Cortical neurodegeneration-induced non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) is a clinical syndrome characterized by non-fluent speech, such as apraxia of speech or agrammatism. We describe the case of an 80-year-old right-handed woman who exhibited nfvPPA. Atypically, our patient also presented with generalized auditory agnosia. Brain magnetic resonance imaging revealed left-sided predominant atrophy of the bilateral perisylvian area, including the inferior frontal and superior temporal lobes...

Verbal-auditory agnosia and aphasia are the most prominent symptoms in Landau-Kleffner syndrome (LKS), a childhood epilepsy that can have sustained long-term effects on language processing. The present study provides the first objective investigation of music perception skills in four adult patients with a diagnosis of LKS during childhood, covering the spectrum of severity of the syndrome from mild to severe. Pitch discrimination, short-term memory for melodic, rhythmic and verbal information, as well as emotion recognition in music and speech prosody were assessed with listening tests, and subjective attitude to music with a questionnaire...

Cortical deafness is an extremely rare clinical manifestation that originates mainly from bilateral cortical lesions in the primary auditory cortex. Its main clinical manifestation is the bilateral sudden loss of hearing. Diagnosis is difficulty due to its rarity and similarity with other language and communication disorders, such as Wernicke's aphasia, auditory agnosia or verbal deafness. Herein, we present a case report of a young woman with a sudden bilateral loss of auditory comprehension. Initially, a psychiatric nature of the disorder was considered, but the persistence of the symptoms, lead to the diagnosis of cortical deafness secondary to bilateral ischemic lesions in both temporal lobes...

Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulfate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently, a greater neurocognitive deterioration may happen. Herein, we report a 9-yr-old boy referred to Loghman Hospital, Tehran, Iran, in 2018...

Aphasia typically is associated with comparable difficulties in written and spoken modalities of language expression and comprehension. In contrast, auditory verbal agnosia is the disproportionate difficulty comprehending spoken compared to written language, also typically greater than difficulties with spoken and written language expression, in the absence of a primary sensory deficit. The terms pure word deafness and auditory verbal agnosia are often used synonymously. However, the broader term of auditory agnosia more accurately reflects difficulty processing both speech and non-speech sounds whereas individuals with auditory verbal agnosia (pure word deafness) have preserved processing of environmental sounds...

The cognitive organisation of nonverbal auditory knowledge remains poorly defined. Deficits of environmental sound as well as word and visual object knowledge are well-recognised in semantic dementia. However, it is unclear how auditory cognition breaks down in this disorder and how this relates to deficits in other knowledge modalities. We had the opportunity to study a patient with a typical syndrome of semantic dementia who had extensive premorbid knowledge of birds, allowing us to assess the impact of the disease on the processing of auditory in relation to visual and verbal attributes of this specific knowledge category...

RATIONALE: Verbal auditory agnosia is the selective inability to recognize verbal sounds. Patients with this disorder lose the ability to understand language, write from dictation, and repeat words with reserved ability to identify nonverbal sounds. However, to the best of our knowledge, there was no report about verbal auditory agnosia in adult patient with traumatic brain injury. PATIENT CONCERNS: He was able to clearly distinguish between language and nonverbal sounds, and he did not have any difficulty in identifying the environmental sounds...

Stroke-like episodes are one of the cardinal features of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), and occur in 84-99% of the patients. The affected areas detected on neuroimaging do not have classical vascular distribution, and involve predominantly the temporal, parietal and occipital lobes. Thus, the neurological symptoms including higher brain dysfunction correlate with this topographical distribution. In association with the occipital lobe involvement, the most frequent symptom is cortical blindness...