Subcortical auditory agnosia

A case of generalized auditory agnosia without aphasia secondary to cardiogenic cerebral embolism is reported. The infarcts in this patient were localized within the bitemporal subcortices as confirmed by computerized axial tomography and magnetic resonance imaging. The findings suggested that interruption of both auditory radiations by bilateral subcortical lesions may play an important role in the occurrence of "cerebral auditory disorders."     

Central deafness and subcortical motor aphasia

Clinical and autopsy studies were made on a right-handed man who had central deafness and subcortical motor aphasia, and the literature on central deafness and on subcortical motor aphasia was analyzed. Central deafness is due to bilateral destruction of the primary auditory cortex. It is sometimes difficult to distinguish from word deafness and from auditory agnosia, which are due to pathology in other parts of the temporal lobes. There is almost always some preserved hearing in central deafness, possibly from some auditory pathway other than the classical pathway. In this patient the subcortical motor aphasia was due to bilateral destruction of the motor cortex for the mouth and throat. In some other cases subcortical motor aphasia was due to the same pathology that usually causes Broca's aphasia; in these cases the unexpected preservation of writing was perhaps related to some difference in how language functions were organized in the brain.     

Self-monitoring behavior in a case of severe auditory agnosia with aphasia

This case report describes an unusual combination of speech and language deficits secondary to bilateral infarctions in a 62-year-old woman. The patient was administered an extensive series of speech, language, and audiologic tests and was found to exhibit a fluent aphasia in which reading and writing were extremely well preserved in comparison to auditory comprehension and oral expression, and a severe auditory agnosia. In spite of her auditory processing deficits, the patient exhibited unexpected self-monitoring ability and the capacity to form acoustic images on visual tasks. The manner in which she corrected and attempted to correct her phonemic errors, while ignoring semantic errors, suggests that different mechanisms may underlie the monitoring of these errors.     

Speech discrimination and lip reading in patients with word deafness or auditory agnosia

The purpose of this study was to assess the ability of four patients with word deafness or auditory agnosia to discriminate speech by reading lips. The patients were studied using nonsense monosyllables to test for speech discrimination, a lip reading test, the Token Test for auditory comprehension, and the Aphasia test. Our results show that patients with word deafness or auditory agnosia without aphasia can improve speech comprehension by reading lips in combination with listening, as compared with lip reading or listening alone. In conclusion, lip reading was shown to be useful for speech comprehension among these patients.     

Optic aphasia, optic apraxia, and loss of dreaming

A 47-year-old man with a left temporo-occipital infarct in the area of the posterior cerebral artery is presented. The neuropsychological examination did not reveal aphasia or gross mental deficits. The patient presented with alexia without agraphia, color agnosia, but few visual perceptual deficits. The main impairment was in confrontation naming; he was incapable of naming objects and pictures, not from lack of recognition (excluding visual agnosia) but from lack of access to the appropriate word (optic aphasia). The patient also exhibited a deficit in the evocation of gesture from the visual presentation of an object (optic apraxia) and a difficulty in "conjuring up" visual images of objects (impaired visual imagery) and loss of dreams. The fundamental deficit of this patient is tentatively explained in terms of visuoverbal and visuogestural disconnection and a deficit of mental imagery.     

Semantic aphasia: A neglected entity

Three cases of semantic aphasia are reported. Computerized brain tomography showed bilateral temporo-parieto-occipital junction hemorrhages in one patient, and left parieto-occipital junction infarctions in the other two patients. The auditory comprehension defect of the three patients was characterized by preserved understanding of single words and impaired understanding of grammatically complex constructions. It is suggested that this comprehension defect reflects an inability to fully grasp the meaning of words and grammatical constructions imbued with spatial or quasi-spatial significance. Each of the three patients showed a complex spatial disorder that included constructional apraxia, spatial agnosia, and elements of Gerstmann's syndrome. The aphasic as well as the spatial disorder of semantic aphasics may be manifestations of a common defect in the perception of spatial relationships produced by left temporo-parieto-occipital region damage.     

Prosopagnosia and visual object agnosia: A behavioral study

A left-handed woman developed visual object agnosia, prosopagnosia, and visual disorientation after resection of the right occipital lobe. Color agnosia and alexia were absent. When asked to identify objects presented visually, the patient's errors represented visually related objects (underspecifications) or perseverations. Identification was facilitated when she observed the object being used in a natural way. Identification was impaired by surrounding the object with unrelated objects, decreasing the background illumination, decreasing the duration of exposure of the object to the patient, and probably also by decreasing the visual angle subtended by the object. In addition, there were disturbances of visualization (i.e., imaging in the absence of a visual stimulus) that paralleled the perceptual difficulties. We conclude that: (1) A deficit in visual perception, characterized by insufficient feature analysis of visual stimuli, was the basis of the visual agnosia in this case. (2) The visual agnosia could not be explained by (a) a vision-language disconnection syndrome, (b) decay of visual memory traces, or (c) deficiencies in the visual fields (pathologic Funktionswandel). (3) The ability to visualize (visual imagery) probably utilizes some of the same neural pathways used in perception. (4) The results in this case probably can be generalized to some but not all cases of visual agnosia; in particular, the deficit in most previously reported patients with prosopagnosia is similar to that of our case. However, agnosic alexia and color agnosia usually have a different neuropsychological basis.     

Characteristics of auditory agnosia in a child with severe traumatic brain injury: a case report

We present a case that is unusual in many respects from other documented incidences of auditory agnosia, including the mechanism of injury, age of the individual, and location of neurological insult. The clinical presentation is one of disturbance in the perception of spoken language, music, pitch, emotional prosody, and temporal auditory processing in the absence of significant deficits in the comprehension of written language, expressive language production, or peripheral auditory function. Furthermore, the patient demonstrates relatively preserved function in other aspects of audition such as sound localization, voice recognition, and perception of animal noises and environmental sounds. This case study demonstrates that auditory agnosia is possible following traumatic brain injury in a child, and illustrates the necessity of assessment with a wide variety of auditory stimuli to fully characterize auditory agnosia in a single individual.     

Cortical auditory disorders: a case of non-verbal disturbances assessed with event-related brain potentials

In the auditory modality, there has been a considerable debate about some aspects of cortical disorders, especially about auditory forms of agnosia. Agnosia refers to an impaired comprehension of sensory information in the absence of deficits in primary sensory processes. In the non-verbal domain, sound agnosia and amusia have been reported but are frequently accompanied by language deficits whereas pure deficits are rare. Absolute pitch and musicians' musical abilities have been associated with left hemispheric functions. We report the case of a right handed sound engineer with the absolute pitch who developed sound agnosia and amusia in the absence of verbal deficits after a right perisylvian stroke. His disabilities were assessed with the Seashore Test of Musical Functions, the tests of Wertheim and Botez (Wertheim and Botez, Brain 84, 1961, 19-30) and by event-related potentials (ERP) recorded in a modified 'oddball paradigm'. Auditory ERP revealed a dissociation between the amplitudes of the P3a and P3b subcomponents with the P3b being reduced in amplitude while the P3a was undisturbed. This is interpreted as reflecting disturbances in target detection processes as indexed by the P3b. The findings that contradict some aspects of current knowledge about left/right hemispheric specialization in musical processing are discussed and related to the literature concerning cortical auditory disorders.     

Developmental auditory agnosia in the severely retarded: A further investigation

The hypotheses were investigated that (a) ability to recognize the auditory perceptual stimuli for familiar events is a developmental correlate to language acquisition and (b) the low functioning mentally handicapped suffer from auditory agnosia and are impaired in this ability. The subjects were 42 nonretarded children of ages 3 through 6 and 53 severely and moderately retarded, noninstitutionalized students. The retarded subjects were matched by mental age to the chronological age of the nonretarded children. The stimuli were 49 environmental sounds; the task consisted of sound-and-picture matching-to-sample. Group membership and developmental age were the factors in an analysis of variance design. The results revealed a strong effect of developmental age (p <. 0001). The effect of group was not significant, indicating that auditory agnosia may not be common among the lower functioning retarded. The assumption that agnosia may be a major factor underlying the language disability of the severely retarded was reexamined. It was suggested that the severely retarded achieve the requisite perceptual-semantic knowledge base for language too late, after the critical age for spontaneous and efficient language learning has passed.     

Correlation between EEG and auditory perceptual measures in auditory agnosia

We describe a child who acquired a gradual sustained speech deficit for which no specific etiology was found, and who had an associated epileptogenic abnormality on EEG. Assessment of auditory perceptual skills and receptive language confirmed that gradual parallel improvement occurred with the EEG after therapeutic anticonvulsant blood levels were obtained. Prospective trials of anticonvulsant drugs in conjunction with serial measures of central auditory abilities are necessary to establish their value in the management of the linguistic deficit in children with auditory agnosia.     

Some aspects of phonological impairment in aphasia

The relationships between expressive and receptive phonemic disorders and between disturbances of phoneme processing and general disorders of auditory comprehension were studied in 69 aphasic patients, by means of a phoneme discrimination test and of a standard aphasia battery. A significant but incomplete correlation between disorders of phonemic output and of phoneme analysis was found. The view is put forth that in some patients only phoneme encoding or decoding is disturbed, whereas in others a central level of phoneme processing is impaired. A significant but partial correlation was also found between disorders of phonological analysis and general disorders of auditory comprehension. This supports the view that, besides sequential phonological analysis, other processes operate in auditory comprehension.     

Hierarchical planning disability in the drawings and constructions of a special group of severely aphasic children

A small group of severely receptive aphasic children who appear to be suffering from a rare aphasic syndrome that has been referred to in the literature as "acquired aphasia with convulsive disorder in children" was tested on drawing and construction tasks which tap hierarchical planning abilities. Although they could draw and construct a complex figure when using a serial method, they could not do so when required to use hierarchical planning in building up the model in terms of its subunits. Their scores reflecting hierarchical ability were significantly lower than those of age-matched profoundly deaf and normal children. The nature of this disability is discussed and its effects noted on tasks in other modalities. It is argued that neither an auditory deficit theory nor a short-term memory deficit theory is adequate to account for the observed disabilities. Individual differences in the group were noted. It was also found that a smaller percentage of developmentally expressive aphasic children evidenced the hierarchical planning disability. It is concluded that a primary hierarchical planning disability in some aphasic children can account for some aspects of their linguistic disabilities and for disabilities on other nonlanguage and nonauditory tasks.     

Chronic Auditory Agnosia Following Landau-Kleffner Syndrome: A 23 Year Outcome Study

We report a 27-year-old woman with chronic auditory agnosia following Landau-Kleffner Syndrome (LKS) diagnosed at age 4 1/2 . She grew up in the hearing/speaking community with some exposure to manually coded English and American Sign Language (ASL). Manually coded (signed) English is her preferred mode of communication. Comprehension and production of spoken language remain severely compromised. Disruptions in auditory processing can be observed in tests of pitch and duration, suggesting that her disorder is not specific to language. Linguistic analysis of signed, spoken, and written English indicates her language system is intact but compromised because of impoverished input during the critical period for acquisition of spoken phonology. Specifically, although her sign language phonology is intact, spoken language phonology is markedly impaired. We argue that deprivation of auditory input during a period critical for the development of a phonological grammar and auditory–verbal short-term memory has limited her lexical and syntactic development in specific ways.     

A modern assessment of the basic forms of aphasia

A brief survey of the field of aphasia is presented. Many of the facts described in the neurological classics remain valid, but the interpretation of these facts must be altered. The basic principles of aphasia are reassessed in the light of neuropsychology. Neuropsychological analysis shows sensory (acoustico-gnostic) aphasia to be based upon an impairment in phonemic hearing. Motor aphasia breaks down into afferent (kinesthetic) and efferent (kinetic) aphasia. The former is based upon an impaired kinesthetic input into the speech functions, which results in a special type of oral apraxia. Efferent (kinetic) aphasia is based upon a breakdown in the kinetic organization of speech motor acts. Nominative (amnestic) aphasia consists of a number of forms, each based on either a weakness of visual or auditory analysis or difficulty in selection between evoked memory traces. “Transcortical motor aphasia” proves to be either perseverative aphasia, i.e., aphasia in which the dynamics of plasticity are impaired, or the result of the impairment of internal speech. The basic defect underlying “conduction” aphasia is beginning to be understood as a weakened acoustico-gnostic function or difficulties in retarding sideline associations.     

A case of acquired aphasia and convulsive disorder: some linguistic aspects of recovery and breakdown

Among the cases of acquired aphasia and convulsive disorder several children are reported to have experienced more than one aphasic period. We present such a case in more detail, with emphasis on some characteristics of spontaneous speech. The occurrence of paraphasias, and in particular of neologisms, appeared to be associated with language recovery and breakdown. At the beginning of the period of recovery, a rapid and total disappearance of neologisms was observed, while only literal paraphasias remained. A period of language breakdown was preceded by the return of neologisms. The process was not influenced by drug therapy.     

A case of cortical deafness: Clinical and electrophysiological data

A patient suffering from a primary myocardiopathy is suddenly no longer able to recognize speech and environmental sounds, in spite of good peripheral hearing; he remains able to speak, read, and write. The C.T. scan shows lesions involving both supratemporal planes. The cortical sensory deficit documented by the absence of late acoustic evoked potentials leads to a diagnosis of cortical deafness rather than of auditory agnosia.     

Defects of immediate memory in Broca's and conduction aphasia

It has been suggested that the repetition defect seen in conduction aphasia is caused by an auditory immediate memory defect. The purposes of this study were the following: to ascertain whether the finding of an auditory immediate memory defect in conduction aphasia could be replicated, to ascertain whether Broca's aphasics have a similar type of immediate memory defect, and to ascertain whether a relationship exists between immediate memory and comprehension of spoken language. Digits were either spoken or presented visually to Broca's and conduction aphasics. An analysis of span scores reveals that within each group there were no significant differences between the modes of presentation or the response modes. There were no significant differences between the aphasic groups. Comprehension of spoken language significantly correlated with digit span scores.     

Historical Perspectives on Ancient Greek Derived “a” Prefixed Nomenclature for Acquired Neurocognitive Impairment

Distinct forms of acquired neurocognitive impairment are often described by “a” prefixed terms that derive from ancient Greek (and in one case Latin). Two modern English language neurological and neuropsychological reference books were searched to identify 17 such terms in contemporary usage: amnesia, akinesia, ataxia, aphasia, agraphia, anosmia, apraxia, athetosis, ageusia, achromatopsia, agnosia, alexia, amusia, anomia, anarthria, anosognosia, and acalculia. These were traced to their initial association with acquired neurocognitive impairment in German, English, and French language medical publications from the late 18th, 19th, and early 20th centuries (1770 through 1920). Some of these terms (e.g., agnosia) were used in ancient Greek, although not associated with neurocognitive impairment. The remainder constitute novel semantically plausible (e.g., anosmia) and unclear (e.g., alexia) formulations. In the localizationist thinking of the time, neurocognition was conceived as being organized within specialized “centers” in specific locations connected by pathways within the brain.     

Does long term use of piracetam improve speech disturbances due to ischemic cerebrovascular diseases?

Aphasia causes significant disability and handicap among stroke survivors. Language therapy is recommended for aphasic patients, but not always available. Piracetam, an old drug with novel properties, has been shown to have mild beneficial effects on post-stroke aphasia. In the current study, we investigated the effects of 6 months treatment with piracetam on aphasia following stroke. Thirty patients with first-ever ischemic strokes and related aphasia were enrolled in the study. The scores for the National Institutes of Health Stroke Scale (NIHSS), Barthel Index (BI), modified Rankin Scale (mRS), and Gülhane Aphasia Test were recorded. The patients were scheduled randomly to receive either 4.8 g piracetam daily or placebo treatment for 6 months. At the end of 24 weeks, clinical assessments and aphasia tests were repeated. The level of improvement in the clinical parameters and aphasia scores was compared between the two groups. All patients had large lesions and severe aphasia. No significant difference was observed between the piracetam and placebo groups regarding the improvements in the NIHSS, BI and mRS scores at the end of the treatment. The improvements observed in spontaneous speech, reading fluency, auditory comprehension, reading comprehension, repetition, and naming were not significantly different in the piracetam and placebo groups, the difference reached significance only for auditory comprehension in favor of piracetam at the end of the treatment. Piracetam is well-tolerated in patients with post-stroke aphasia. Piracetam taken orally in a daily dose of 4.8 g for 6 months has no clear beneficial effect on post-stroke language disorders.