Auditory agnosia with anosognosia

Klarendić, Maja; Gorišek, Veronika Rutar; Granda, Gal; Avsenik, Jernej; Zgonc, Vid; Kojović, Maja

doi:10.1016/j.cortex.2020.12.025

Cited by 4 publications

(6 citation statements)

References 109 publications

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“…A total of 103 manuscripts was selected for a complete text evaluation, after which 44 were included [ 2 , 4 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 ]. The total number of patients included was 46 ( Table 1 ).…”

Section: Resultsmentioning

confidence: 99%

“…The duration of the follow-up varied from 2 weeks to 15 years. A substantial part of the patients did not improve, and remained with a persistent inability to recognize any sound at all (16/34.8%) [ 2 , 8 , 12 , 15 , 16 , 17 , 18 , 19 , 29 , 33 , 34 , 35 , 37 , 43 , 46 , 48 ], while others evolved to pure word deafness (11/36.6%) [ 7 , 9 , 10 , 11 , 13 , 18 , 21 , 22 , 24 , 31 , 36 , 39 , 41 , 42 ]. Oral conversation was regained in six (20%) of the patients [ 7 , 20 , 25 , 27 , 28 , 32 , 42 , 43 ].…”

Section: Resultsmentioning

confidence: 99%

“…A substantial part of the patients did not improve, and remained with a persistent inability to recognize any sound at all (16/34.8%) [ 2 , 8 , 12 , 15 , 16 , 17 , 18 , 19 , 29 , 33 , 34 , 35 , 37 , 43 , 46 , 48 ], while others evolved to pure word deafness (11/36.6%) [ 7 , 9 , 10 , 11 , 13 , 18 , 21 , 22 , 24 , 31 , 36 , 39 , 41 , 42 ]. Oral conversation was regained in six (20%) of the patients [ 7 , 20 , 25 , 27 , 28 , 32 , 42 , 43 ]. In two patients, CD was transient, one following a bilateral middle cerebral vasospasm secondary to an aneurysm rupture [ 30 ] and the other two after a unilateral ischemic stroke [ 38 , 40 ].…”

Section: Resultsmentioning

confidence: 99%

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Stroke-Associated Cortical Deafness: A Systematic Review of Clinical and Radiological Characteristics

et al. 2021

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Background: Stroke is the leading cause of cortical deafness (CD), the most severe form of central hearing impairment. CD remains poorly characterized and perhaps underdiagnosed. We perform a systematic review to describe the clinical and radiological features of stroke-associated CD. Methods: PubMed and the Web of Science databases were used to identify relevant publications up to 30 June 2021 using the MeSH terms: “deafness” and “stroke”, or “hearing loss” and “stroke” or “auditory agnosia” and “stroke”. Results: We found 46 cases, caused by bilateral lesions within the central auditory pathway, mostly located within or surrounding the superior temporal lobe gyri and/or the Heschl’s gyri (30/81%). In five (13.51%) patients, CD was caused by the subcortical hemispheric and in two (0.05%) in brainstem lesions. Sensorineural hearing loss was universal. Occasionally, a misdiagnosis by peripheral or psychiatric disorders occurred. A few (20%) had clinical improvement, with a regained oral conversation or evolution to pure word deafness (36.6%). A persistent inability of oral communication occurred in 43.3%. A full recovery of conversation was restricted to patients with subcortical lesions. Conclusions: Stroke-associated CD is rare, severe and results from combinations of cortical and subcortical lesions within the central auditory pathway. The recovery of functional hearing occurs, essentially, when caused by subcortical lesions.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Stroke-Associated Cortical Deafness: A Systematic Review of Clinical and Radiological Characteristics

et al. 2021

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“…The patient displayed normal perception of man-made, natural, and music sounds. 1,2 Although hearing aids improved thresholds to 30 dB HL, single-syllable word recognition remained poor. The patient discontinued their use, suggesting difficulties in discrimination beyond the degree of HL.…”

Section: Case Reportmentioning

confidence: 99%

Pearls & Oy-sters: Familial Verbal Auditory Agnosia Due to C9orf72 Repeat Expansion

Kim,

Choung

et al. 2023

Neurology

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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. He had difficulty recognizing single-syllable spoken words beyond his hearing loss but had no problem understanding complex written language. He had a heterozygous pathogenic variant carrying 160 hexanucleotide repeats in the C9orf72 gene. His family history included his deceased mother with similar symptoms that had progressed over 30 years, as well as his older brother and youngest sister who experienced speech perception difficulty beginning in their early fifties. His asymptomatic younger brother had a heterozygous 2 repeat in the C9orf72 gene, while his symptomatic youngest sister had a heterozygous 159 repeat. The patient and his sister exhibited more pronounced cortical thinning in the frontotemporoparietal areas. The discrepancy observed between the distribution of atrophy and the presentation of symptoms in patients with C9orf72 pathogenic repeat expansion may be attributable to the slow progression of their clinical course over time. The variable symptom presentation of C9orf72 pathogenic repeat expansion highlights the importance of considering this pathogenic variant as a potential cause of autosomal dominant degenerative brain diseases beyond FTD and ALS.

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“…Although right hemispheric stroke is the most common disease state associated with anosognosia, 2 other neurologic disorders can be associated with anosognosia, including Anton's syndrome of cortical blindness, 3 traumatic brain injury, 4 Wernicke's aphasia, 5 mild cognitive impairment, 6 and Alzheimer's disease. 7 In addition to anosognosia, those disorders can be accompanied by indifference to the deficit, which is referred to as "anosodiaphoria.…”

Section: Anosognosia In Neurologic Brain Disordersmentioning

confidence: 99%

Is anosognosia a delusion, a negative symptom, or a cognitive deficit?

Nasrallah¹

2022

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Auditory agnosia with anosognosia

Cited by 4 publications

References 109 publications

Stroke-Associated Cortical Deafness: A Systematic Review of Clinical and Radiological Characteristics

Stroke-Associated Cortical Deafness: A Systematic Review of Clinical and Radiological Characteristics

Pearls & Oy-sters: Familial Verbal Auditory Agnosia Due to C9orf72 Repeat Expansion

Is anosognosia a delusion, a negative symptom, or a cognitive deficit?

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