Auditory Agnosia for Environmental Sounds in Alzheimer's Disease: Not Hearing and Not Listening?

Auditory agnosia for environmental sounds (AES) is an example of central auditory dysfunction. It is presumed to be independent of language deficits and in presence of normal hearing. We undertook a detailed neuropsychological assessment including environmental sound naming and recognition in 34 clinically mild Alzheimer's disease (AD) patients and 29 age-matched healthy control subjects. In patients with AD, audiometry was performed to assess the impact on test performance, and in normal controls the Hearing Handicap Inventory for the Elderly - Screening Version to exclude more than mild hearing loss. We adapted a validated environmental sound battery and found near perfect scores in controls. We found that environmental sound agnosia is common in mild AD. We found a statistically significant difference in mean pure tone audiometry in the best ear between patients with and those patients without naming deficits of 11.3 dB (p = 0.010) and of 14.7 dB (p = 0.000) between those with and without recognition deficits. Statistical significance remained after correcting for age, aphasia, Mini-Mental State Examination score, and working memory. Slight and moderate peripheral hearing loss increases the odds ratio of recognition deficits by 13.75 (confidence interval 2.3-81.5) compared to normal hearing patients. We did not find evidence for different forms of AES. This work suggests that an interaction between peripheral hearing loss and AD pathology produces problems with environmental sound recognition. It confirms that the relationship between hearing and dementia is complex but also suggests that interventions to prevent and treat hearing loss could have an effect on AD in its clinical expression.

Metacognitive knowledge of olfactory dysfunction in Parkinson's disease.

It is well known that patients with Parkinson's Disease (PD) suffer from olfactory impairments, but it is not clear whether patients are aware of their level of deficit in olfactory functioning. Since PD is a neurodegenerative disorder and its progression may be correlated with olfactory loss (Ansari & Johnson, 1975; but see also Doty, Deems, & Stellar, 1988), it is possible that these patients would be subject to metacognitive errors of over-estimation of olfactory ability (White & Kurtz, 2003). Nineteen non-demented PD patients and 19 age-matched controls were each given an objective measure of olfactory identification (the UPSIT, Doty, Shaman, Kimmelman, & Dann, 1984) and a subjective measure involving a questionnaire that asked them to self-rate both their olfactory function generally and their ability to smell each of 20 odors, 12 of which were assessed on the UPSIT. All of the PD patients showed impaired olfactory ability, as did 7 of the controls, according to the UPSIT norms. Self-rated and performance-based olfactory ability scores were significantly correlated in controls (r=.49, p=.03) but not in patients with PD (r=.20, p=.39). When the 12 odors common to both the self-rated questionnaire and UPSIT were compared, PD patients were less accurate than controls (t(36)=-4.96, p<.01) at estimating their own ability and the number of over-estimation errors was significantly higher (tone-tailed(29)=1.80, p=.04) in PD patients than in the control group, showing less metacognitive awareness of their ability than controls. These results support the idea that olfactory metacognition is often impaired in PD, as well as in controls recruited for normosmic ability (Wehling, Nordin, Espeseth, Reinvang, & Lundervold, 2011), and indicate that people with PD generally exhibit over-estimation of their olfactory ability at a rate that is higher than controls. These findings imply that PD patients, unaware of their olfactory deficit, are at greater risk of harm normally detected through olfaction, such as smoke or spoiled foods.

Subgroup differences in the lexical tone mismatch negativity (MMN) among Mandarin speakers with congenital amusia.

The association/dissociation of pitch processing between music and language is a long lasting debate. We examined this music-language relationship by investigating to what extent pitch deficits in these two domains were dissociable. We focused on a special neurodevelopmental pitch disorder - congenital amusia, which primarily affects musical pitch processing. Recent research has also revealed lexical tone deficits in speech among amusics. Approximately one-third of Mandarin amusics exhibits behavioural difficulties in lexical tone perception, which is known as tone agnosia. Using mismatch negativities (MMNs), our current work probed lexical tone encoding at the pre-attentive level among the Mandarin amusics with (tone agnosics) and without (pure amusics) behavioural lexical tone deficits compared with age- and IQ-matched controls. Relative to the controls and the pure amusics, the tone agnosics exhibited reduced MMNs specifically in response to lexical tone changes. Their tone-consonant MMNs were intact and similar to those of the other two groups. Moreover, the tone MMN reduction over the left hemisphere was tightly linked to behavioural insensitivity to lexical tone changes. The current study thus provides the first psychophysiological evidence of subgroup differences in lexical tone processing among Mandarin amusics and links amusics' behavioural tone deficits to impaired pre-attentive tone processing. Despite the overall music pitch deficits, the subgroup differences in lexical tone processing in Mandarin-speaking amusics suggest dissociation of pitch deficits between music and speech.

Anosognosia and neglect respond differently to the same treatments.

Different techniques, such as optokinetic stimulation, adaptation to prismatic shift of the visual field to the right, or transcutaneous electrical nerve stimulation (TENS), have been shown to alleviate neglect, at least temporarily. We assessed the effect of these techniques on anosognosia and whether their therapeutic effect, if any, matches that on neglect. The effect of the three types of treatment on anosognosia and neglect was investigated in five patients presenting with both severe anosognosia and neglect. Patient 1 was treatment responsive to anosognosia but not to neglect, whereas patients 4 and 5 showed the reverse pattern, i.e., they were treatment responsive to neglect but not to anosognosia. This "treatment response bias" proved to be a valid means to investigate different effects of treatments in the same patient.

Mirror agnosia and the mirrored-self misidentification delusion: a hypnotic analogue.

INTRODUCTION: Mirrored-self misidentification is the delusional belief that one's reflection in the mirror is a stranger. Current theories suggest that one pathway to the delusion is mirror agnosia (a deficit in which patients are unable to use mirror knowledge when interacting with mirrors). This study examined whether a hypnotic suggestion for mirror agnosia can recreate features of the delusion. METHOD: Ten high hypnotisable participants were given either a suggestion to not understand mirrors or to see the mirror as a window. Participants were asked to look into a mirror and describe what they saw. Participants were tested on their understanding of mirrors and received a series of challenges. Participants then received a detailed postexperimental inquiry. RESULTS: Three of five participants given the suggestion to not understand mirrors reported seeing a stranger and maintained this belief when challenged. These participants also showed signs of mirror agnosia. No participants given the suggestion to see a window reported seeing a stranger. CONCLUSION: Results indicate that a hypnotic suggestion for mirror agnosia can be used to recreate the mirrored-self misidentification delusion. Factors influencing the effectiveness of hypnotic analogues of psychopathology, such as participants' expectations and interpretations, are discussed.

Impaired perception of harmonic complexity in congenital amusia: a case study.

This study investigates whether congenital amusia (an inability to perceive music from birth) also impairs the perception of musical qualities that do not rely on fine-grained pitch discrimination. We established that G.G. (64-year-old male, age-typical hearing) met the criteria of congenital amusia and demonstrated music-specific deficits (e.g., language processing, intonation, prosody, fine-grained pitch processing, pitch discrimination, identification of discrepant tones and direction of pitch for tones in a series, pitch discrimination within scale segments, predictability of tone sequences, recognition versus knowing memory for melodies, and short-term memory for melodies). Next, we conducted tests of tonal fusion, harmonic complexity, and affect perception: recognizing timbre, assessing consonance and dissonance, and recognizing musical affect from harmony. G.G. displayed relatively unimpaired perception and production of environmental sounds, prosody, and emotion conveyed by speech compared with impaired fine-grained pitch perception, tonal sequence discrimination, and melody recognition. Importantly, G.G. could not perform tests of tonal fusion that do not rely on pitch discrimination: He could not distinguish concurrent notes, timbre, consonance/dissonance, simultaneous notes, and musical affect. Results indicate at least three distinct problems-one with pitch discrimination, one with harmonic simultaneity, and one with musical affect-and each has distinct consequences for music perception.

Nonverbal auditory agnosia with lesion to Wernicke's area.

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). We verified that the patient exhibited persistent and severe auditory agnosia for nonverbal sounds in the absence of verbal comprehension deficits or peripheral hearing problems. Acoustical analyses suggested that his residual processing of a minority of environmental sounds might rely on his speech processing abilities. In the patient's brain, contralateral (right) temporal cortex as well as perilesional (left) anterior temporal cortex were strongly responsive to verbal, but not to nonverbal sounds, a pattern that stands in marked contrast to the controls' data. This substantial reorganization of auditory processing likely supported the recovery of M's speech processing.

A comparative case study of face recognition: the contribution of configural and part-based recognition systems, and their interaction.

Understanding the interaction between the configural and part-based systems in face recognition is the major aim of this study. Specifically, we established whether configural representation of faces contribute to aspects of face recognition that depend on part-based processes, such as identifying inverted or fractured faces. Using face recognition tasks that require part-based or configural processing, we compared the results of CK--a man who has object agnosia and alexia [Moscovitch, M., Winocur, G., & Behrmann, M. (1997). What is special about face recognition? Nineteen experiments on a person with visual object agnosia and dyslexia but normal face recognition. Journal of Cognitive Neuroscience, 9(5), 555-604] but normal upright face recognition, to those of DC--a man who has prosopagnosia but normal object recognition. CK was normal at recognizing faces if configural processing was sufficient, but poor at recognizing faces that were modified so as to alter their gestalt, and require part-based processing (Moscovitch et al.). DC was impaired at recognizing upright faces and his performance declined in all tasks involving recognition of modified faces, including those that depend on part-based and on configural processing. Nevertheless, DC was normal on tasks involving perception of generic faces and face imagery. These results show that although configural face perception can proceed without part-based processing, the reverse is not the case. Our results suggest that the configural system is always necessary for face recognition, and appears to support what remains of face identification even in prosopagnosic people who have an intact part-based system.

Memories are made of this: the effects of time on stored visual knowledge in a case of visual agnosia.

We report the effects of the passage of time on the longterm visual knowledge for objects in a patient with visual agnosia (H.J.A.). The naming of real objects was found to have improved, although this was not associated with any change in H.J.A.'s basic perceptual abilities which were stable over a 16-year period. The improvement in object naming was attributed to better use of non-contour-based visual information (such as surface detail and depth cues). In addition, we demonstrate a deterioration in H.J.A.'s long-term memory for the visual properties of objects, and argue that this has occurred as a result of his having impaired perceptual input. The deterioration was only apparent in drawing from memory and in the verbal descriptions of items; with forced-choice testing, H.J.A. operated at ceiling; we propose that current tests of visual imagery may not be sufficiently sensitive to detect subtle impairments of visual memory. Our findings can be taken to indicate that perceptual and memorial processes are not functionally independent, but are linked in an interactive manner.

One or several semantic system(s)? Maybe none: evidence from a case study of modality and category-specific "semantic" impairment.

Following cerebral anoxia, EC a 55-year-old patient, exhibited a severe and clear-cut pattern of semantic impairments without general intellectual deficit or perceptual difficulty. EC demonstrated a complex neuropsychological picture including a massive visual agnosia and a complete lack of imagery, both of which involved all categories of objects (living and non living) and a category-specific word comprehension deficit limited to animal names. Findings are discussed in the light of the theoretical frameworks currently available in the area of neuropsychology. It is argued that neither the single nor the multiple view of semantics fully succeed in providing a satisfactory account of the data and a tentative interpretation of the whole pattern of impairment is proposed in the general framework of non abstractive conceptions of meaning.

Copying without perceiving: motor imagery in visual form agnosia.

Patient DF has severely impaired visual contour perception, despite being able to use that same visual information to guide her motor actions. We report that DF has developed a strategy to overcome some of her perceptual deficits. DF was first asked to copy single lines set at different orientations. She performed surprisingly accurately, although her responses were slow. When questioned, DF reported imagining tracing the line with her finger before copying the line on paper, although she was still unable to discriminate perceptually between different line orientations. We found that time restraints, or the requirement to perform secondary concurrent tasks, severely disrupted DF's orientation copying ability. We conclude that DF can use pure motor imagery to compensate for some of her perceptual difficulties.

Congenital visual agnosia and prosopagnosia in a child: a case report.

We present an eight years old child, L.G. with congenital agnosia and prosopagnosia. This is a special case of perceptual deficits in a child which are discrete and exist in the context of a very high verbal intelligence. L.G. was administered an extensive battery of tests of cognitive functioning. He has intact basic visual skills, although his visual analysis is sometimes slow. L.G. can read, write and do math at age level or above. Four normal eight years old boys were used as controls on a selection of the perceptual tests, administered to L.G., which did not have normative data. L.G.'s object recognition skills bear the hallmarks of adult apperceptive agnosia. His visual memory and imagery are normal. Tests of face processing skills reveal, unlike adult prosopagnosics, severe deficits in addition to the familiar face recognition problem. L.G.'s agnosia and prosopagnosia are compared to the relevant literature.

Selective loss of verbal imagery.

This single case study of the ability to generate verbal and non-verbal imagery in a woman who sustained a gunshot wound to the brain reports a significant difficulty in generating images of word shapes but not a significant problem in generating object images. Further dissociation, however, was observed in her ability to generate images of living vs non-living material. She made more errors in imagery and factual information tasks for non-living items than for living items. This pattern contrasts with our previous report of the agnosic patient, M.S., who had severe difficulty in generating images of living material, whereas his ability to image the shape of words was comparable to that of normal control subjects. Furthermore, with regard to the generation of images of living compared with non-living material, M.S. shows more errors with living than nonliving items. In contrast, the present patient, S.M., made significantly more errors with non-living relative to living items. There appear to be two types of double dissociation which reinforce the growing evidence of dissociable impairments in the ability to generate images for different types of verbal and non-verbal material. Such dissociations, presumably related to sensory and cognitive processing demands, address the problem of the neural basis of imagery.

Preserved visual imagery in visual form agnosia.

We investigated the ability of a patient (D.F.) with profound visual form agnosia to perform a variety of tasks requiring visual imagery. Despite her inability to discriminate between objects and patterns of different shapes, sizes, and orientations, D.F. showed quite normal visual imagery involving these same 'visual' properties when the images were drawn from long-term memory. Thus, she was able both to scan mental images in search of particular features and to form new images by combining several known images. While there is growing evidence that perception and imagery share common neural substrates, the fact that D.F. shows intact visual imagery in the face of a massive perceptual deficit in form vision challenges recent suggestions that these two psychological processes share common input pathways in early vision. It is suggested that regions in the occipitotemporal pathway may be important for the generation of visual images while regions in the posterior parietal system might be involved in the manipulation of these images.

Differences in the visual control of pantomimed and natural grasping movements.

In a series of experiments, we studied the differences between natural target-directed grasping movements and 'pantomimed' movements directed towards remembered objects. Although subjects continued to scale their hand opening for object size when pantomiming, grip formation and other kinematic variables differed significantly from those seen in normal target-directed actions. This was true whether the subjects had just seen the target object 2 sec before (Experiments 1 and 2) or whether the target object was still present and they were simply required to pantomime the grasping movement beside it (Experiment 3). We argued that these pantomimed reaches were being driven by stored perceptual information about the object, and were not utilizing the normal visuomotor control systems that direct actions in real time. This interpretation received strong support from observations of a patient with visual form agnosia who was also tested. In an earlier report, we had shown that this patient showed anticipatory scaling of her grasp despite her inability to discriminate between objects perceptually on the basis of size. The present study showed, however, that the requirement to remember an object even briefly, or to pantomime an action beside it, was enough to completely disrupt her visuomotor scaling (Experiments 2 and 3). That this reflected a failure of perception rather than imagery or understanding was supported by the fact that she could convincingly pantomime actions to imagined, familiar objects, the sizes of which were known to her (Experiment 4). All these results suggest that the mechanisms underlying the formation of perceptual representations of objects are quite independent of those mediating on-line visuomotor control.

Reading of letters and words in a patient with Balint's syndrome.

A patient with bilateral parietal damage, and Balint's syndrome, named visual letters. These were presented individually or within four-letter strings. Solitary letters were identified very accurately. In the case of strings, more letters were correctly reported for words than for nonwords, and more for pronounceable than for unpronounceable nonwords. When required to read words as a whole, performance was better than predicted by letter-reports. These results extend the object-based limitation apparent in Balint's syndrome to the case of reading. The component letters of a string benefit when they form a familiar global object, rather than requiring representation as multiple separate objects. The patient occasionally made homophonic errors when listing the letters in a visual word. This suggests an attempt to bypass visual simultanagnosia by treating the string as a single object, deriving a holistic phonological code for it, and then decomposing this into component letters via spelling rules.

Apperceptive visual agnosia: a case study.

A man with an infarction of his inferior temporal and occipital association cortex bilaterally, which spared primary visual cortex, had impaired visual recognition of objects, faces, colors, words, and gestures. Analysis of visual function indicated that the recognition failures resulted from an agnosia, rather than elemental visual impairment. Whereas his impairment of gesture recognition appeared to be related to an associative agnosia, his inability to recognize objects was related to an apperceptive agnosia. There may be four subtypes of apperceptive agnosia: one where the internal object representations or structural descriptions are impaired, another where an adequate percept cannot be derived, a third where the internal referent and percept are dissociated, and a fourth where both levels are impaired. Our patient demonstrated a failure to relate individual elements to the whole, a failure to integrate multiple elements, and a reliance on global perception. He had normal object imagery. These results suggest that, whereas internal representations were intact, he was unable to form adequate perceptual representations.

Partial recovery from visual object agnosia: a 10 year follow-up study.

We describe a young woman, J.R., who sustained a very severe head injury in 1981 at the age of 17 years. She was assessed in 1982 and found to have visual agnosia. Since then J.R. has been assessed on several occasions over a period of ten years. Her agnosia for real objects has resolved and she has improved on the identification of other classes of stimuli. However, she still has some problems with the identification of line drawings, photographs and model animals. Her drawing from memory remains particularly poor and she has difficulty with visual imagery. We consider her residual deficits in the light of Farah's (1990) theoretical framework; this proposes that associative agnosia could be due to a disconnection syndrome, a loss of stored visual representations or to the loss of knowledge of how to perceive objects. J.R.'s residual impairments appear to be mainly due to a loss of access to visual representations in the absence of visual input.

Impaired drawing from memory in a visual agnosic patient.

A case is reported of an associative visual agnosic patient who could not draw from memory objects he could recognize, even though he could copy drawings flawlessly. His ability to generate mental visual images was found to be spared, as was his ability to operate upon mental images. These data suggest that the patient could generate mental images but could not draw from memory because he did not have access to stored knowledge about pictorial attributes of objects. A similar functional impairment can be found in some other visual agnosic patients and in patients affected by optic aphasia. The present case allows a discussion of relationships among drawing from memory, imagery, and copying procedures.