Auditory verbal agnosia

Auditory agnosia refers to impairments in sound perception and identification despite intact hearing, cognitive functioning, and language abilities (reading, writing, and speaking). Auditory agnosia can be general, affecting all types of sound perception, or can be (relatively) specific to a particular domain. Verbal auditory agnosia (also known as (pure) word deafness) refers to deficits specific to speech processing, environmental sound agnosia refers to difficulties confined to non-speech environmental sounds, and amusia refers to deficits confined to music...

OBJECTIVE: To investigate detailed auditory features in patients with auditory impairment as the first clinical symptoms of childhood adrenoleukodystrophy (CSALD). SUBJECTS AND METHODS: Three patients who had hearing difficulty as the first clinical signs and/or symptoms of ALD. Precise examination of the clinical characteristics of hearing and auditory function was performed, including assessments of pure tone audiometry, verbal sound discrimination, otoacoustic emission (OAE), and auditory brainstem response (ABR), as well as an environmental sound discrimination test, a sound lateralization test, and a dichotic listening test (DLT)...

PURPOSE: To retrospectively analyze the electroclinical characteristics, etiology, treatment, and prognosis of patients with epileptic encephalopathy with status epilepticus during sleep (ESES) with unusual EEG features and to corroborate if this series of patients is part of the ESES syndrome. METHOD: Charts of 17 patients with typical clinical manifestations of the ESES syndrome with focal ESES of non-REM sleep at onset and during the focal ESES phase, or bilateral synchronic and asynchronic ESES with a symmetric or asymmetric morphology, continuous or subcontinuous and sometimes multifocal paroxysms with or without slow-wave activity during slow sleep seen between 2000 and 2012 were analyzed...

We report 2 pediatric patients who presented initially with seizures followed by subacute language regression characterized by a verbal auditory agnosia. These previously normal children had no evidence of expressive aphasia during their symptomatic periods. Further, in both cases, auditory agnosia was associated with sleep-activated electroencephalographic (EEG) epileptiform activity, consistent with Landau-Kleffner syndrome. However, both cases are unique since the episodic auditory agnosia and sleep-activated EEG epileptiform activity rapidly responded to combination therapy with pulse benzodiazepine and corticosteroids...

PURPOSE: The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau-Kleffner syndrome (LKS) with a long-term follow-up. METHODS: Inclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures. RESULTS: Mean follow-up was 12 years...

This paper presents the first systematic review and meta-analysis of neuropsychological and brain morphometry studies comparing posterior cortical atrophy (PCA) to typical Alzheimer's disease (tAD). Literature searches were conducted for brain morphometry and neuropsychological studies including a PCA and a tAD group. Compared to healthy controls (HC), PCA patients exhibited significant decreases in temporal, occipital and parietal gray matter (GM) volumes, whereas tAD patients showed extensive left temporal atrophy...

In the search for 'pure autism', non-verbal children labeled aloof, Severely Autistic with Developmental Disabilities (ASA/DD), are routinely excluded from psychological research. This exclusion is predicated on the claim that they are indistinguishable from those with SLD/PMLD, which is refuted through a discussion of the extant literature. A novel, falsifiable, speech aversion hypothesis is proposed: "aloof, non-verbal young children (<7 years), with severe autism (CARS≥37), but without significant dysmorphic features, will show aversive reactions to complex speech (>2-3 words), but not to a silent interlocutor, or one imitating their vocalizations, in proximal encounters...

Landau-Kleffner syndrome (LKS) is a childhood disorder characterized by an acquired aphasia that emerges in association with epileptiform electroencephalographic abnormalities. The language loss is often characterized by a severe disturbance of auditory language comprehension (verbal auditory agnosia) combined with a substantial disruption of expressive language. Comorbid behavioral disturbances commonly involve hyperactivity and attentional problems but sometimes encompass a more pervasive pattern of difficulties resembling an autism spectrum disorder...

In this study, we describe three patients who each had two different forms of idiopathic focal epilepsiy. Two of these patients had electroclinical features compatible with Panayiotopoulos syndrome and benign childhood epilepsy with centro-temporal spikes (BCECTS), one of whom developed a particular electroclinical picture of atypical benign focal epilepsy and the other an atypical evolution characterized by verbal auditory agnosia and aphasia. The third patient had clinical and electroencephalographic features of BCECTS and of idiopathic childhood occipital epilepsy (Gastaut type) which evolved into electroclinical features of continuous spikes and waves during slow sleep (CSWS)...

A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear...

Extrapontine myelinolysis and pure word deafness are very uncommon disorders. Here, we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness. As a consequence of rapid correction of hyponatremia, the patient demonstrated an initial onset of cortical deafness, and then progressed to generalized auditory agnosia, which eventually developed into confined verbal auditory agnosia (pure word deafness). Bilateral extrapontine myelinolysis was confirmed using brain magnetic resonance imaging...

We report a new case of visual associative agnosia. Our patient (DJ) was impaired in several tasks assessing visual processing of real objects, colour pictures, and line drawings. The deficit was present both with naming and gesturing responses. Object processing in other modalities (verbal, auditory nonverbal, and tactile) was intact. Semantic processing was impaired in the visual but not in the verbal modality. Picture-word matching was better than single picture identification. DJ's visual perceptual processing, was intact in several tasks such as visual attributes discrimination, shape discrimination, illusory contours perception, segmentation, embedded figures processing and matching objects under different viewpoints...

We report the case of patient M, who suffered unilateral left posterior temporal and parietal damage, brain regions typically associated with language processing. Language function largely recovered since the infarct, with no measurable speech comprehension impairments. However, the patient exhibited a severe impairment in nonverbal auditory comprehension. We carried out extensive audiological and behavioral testing in order to characterize M's unusual neuropsychological profile. We also examined the patient's and controls' neural responses to verbal and nonverbal auditory stimuli using functional magnetic resonance imaging (fMRI)...

In Landau-Kleffner syndrome (LKS), the prominent and often first symptom is auditory verbal agnosia, which may affect nonverbal sounds. It was early suggested that the subsequent decline of speech expression might result from defective auditory analysis of the patient's own speech. Indeed, despite normal hearing levels, the children behave as if they were deaf, and very rapidly speech expression deteriorates and leads to the receptive aphasia typical of LKS. The association of auditory agnosia more or less restricted to speech with severe language decay prompted numerous studies aimed at specifying the defect in auditory processing and its pathophysiology...

PURPOSE: Landau-Kleffner syndrome (LKS) is a rare entity characterized by epilepsy and aphasia. It occurs in previously normal children, usually between three and seven years of age. The long-term outcome of LKS is not completely clear. The aim of this study is to verify the long-term follow-up of a group of patients with LKS, focusing on clinical and electroencephalographic (EEG) aspects, and quality of life. METHODS: This was a transversal study. Between November 2006 and April 2007 seven patients with previous diagnosis of LKS were interviewed...

A 7-year-old, right-handed girl started to have seizures at age 1 year 4 months. She developed normally until age 4 when she had worsening of seizures with auditory verbal agnosia, complete aphasia, and a behavioral disorder fulfilling the diagnostic criteria of autism. Medical therapy failed. MRI revealed a right temporal tumor. Video/EEG monitoring at age 7 showed contralateral electrical status epilepticus in wakefulness and sleep and ipsilateral onset of seizures. Resection (ganglioglioma with excessive inflammation) resulted in seizure freedom and marked reduction of the autistic features...

To be a neurologist, training in cognitive/behavioral neuroscience is essential. In this review article, the author tries to emphasize the localization relationship between focal brain lesions and language dysfunction, by discussing from split-brain syndromes, hemispheric asymmetry, to pure word dumbness, pure word deafness (verbal auditory agnosia), pure agraphia, and pure word blindness (pure alexia; visual word agnosia), and finally to aphasia and aprosodia in general. Hopefully, after getting familiar with all the terms, the readers will feel free exploring language disturbances and behavioral neurology for their own interests...

OBJECTIVE: To investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS). METHODS: The clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up. RESULTS: The age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia...

Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia disorder in which children, usually 3-8 years of age who have developed age-appropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. There are no controlled clinical trials investigating the therapeutic options for LKS. Only open-label data are available. Early diagnosis and initiation of prompt medical treatment appear to be important to achieving better long-term prognosis...

A 60-year-old right-handed man showed dysprosody and agnosia for environmental sounds. His mother tongue was Japanese, and he could not speak foreign languages. He gradually developed difficulty in speaking from the age of 57 years, speaking non-native Japanese. In addition, he often complained of difficulty in hearing sounds, but audiometry showed no abnormalities. At the age of 60 years, the standard language test of aphasia showed no abnormalities in repetition, verbal comprehension, or reading, suggesting the absence of aphasia...