scholarly journals Assessing the integrity of auditory processing and sensory memory in adults with cystinosis (CTNS gene mutations)

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ana A. Francisco ◽  
Alaina S. Berruti ◽  
Frederick J. Kaskel ◽  
John J. Foxe ◽  
Sophie Molholm
Keyword(s):  
Auditory Processing ◽  
Early Adulthood ◽  
Gene Mutations ◽  
Cognitive Testing ◽  
Auditory Evoked Potential ◽  
Sensory Memory ◽  
Oddball Paradigm ◽  
Neural Function ◽  
Lysosomal Storage ◽  
Auditory Sensory Memory

Abstract Background Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues and organs, including the kidneys and brain. Its impact on neural function appears mild relative to its effects on other organs, but therapeutic advances have led to substantially increased life expectancy, necessitating deeper understanding of its impact on neurocognitive function in adulthood. We previously demonstrated intact auditory sensory processing, accompanied by mild sensory memory difficulties, in children and adolescents with cystinosis. Methods We investigated whether further progressive decrements in these processes would be observed in adults with cystinosis, comparing high-density auditory-evoked potential (AEP) recordings from adults with cystinosis (N = 15; ages: 19–38 years) to those of age-matched controls (N = 17). We employed a duration oddball paradigm with different stimulation rates, in which participants passively listened to regularly occurring standard tones interspersed with infrequently occurring deviant tones. Analyses focused on AEP components reflecting auditory sensory-perceptual processing (N1 and P2), sensory memory (mismatch negativity, MMN), and attentional orienting (P3a). Results Overall, adults with cystinosis produced highly similar sensory-perceptual AEP responses to those observed in controls suggesting intact early auditory cortical processing. However, significantly increased P2 and P3a amplitudes and reduced MMN at slower stimulation rates were observed, suggesting mild-to-moderate changes in auditory sensory memory and attentional processing. While cognitive testing revealed lower scores on verbal IQ and perceptual reasoning in cystinosis, these did not correlate with the AEP measures. Conclusions These neurophysiological data point to the emergence of subtle auditory processing deficits in early adulthood in cystinosis, warranting further investigation of memory and attentional processes in this population, and of their consequences for perceptual and cognitive function.

scholarly journals Assessing the integrity of auditory processing and sensory memory in adults with cystinosis (CTNS gene mutations)

2021 ◽  
Author(s):  
Ana A. Francisco ◽  
Alaina S. Berruti ◽  
Frederick J. Kaskel ◽  
John J. Foxe ◽  
Sophie Molholm
Keyword(s):  
Auditory Processing ◽  
Early Adulthood ◽  
Gene Mutations ◽  
Cognitive Testing ◽  
Auditory Evoked Potential ◽  
Sensory Memory ◽  
Oddball Paradigm ◽  
Neural Function ◽  
Lysosomal Storage ◽  
Auditory Sensory Memory

Abstract Background Cystinosis, a rare lysosomal storage disease, is characterized by cystine crystallization and accumulation within tissues and organs, including the kidneys and brain. Its impact on neural function appears mild relative to its effects on other organs, but therapeutic advances have led to substantially increased life expectancy, necessitating deeper understanding of its impact on neurocognitive function in adulthood. We previously demonstrated intact auditory sensory processing, accompanied by mild sensory memory difficulties, in children and adolescents with cystinosis. Methods We investigated whether further progressive decrements in these processes would be observed in adults with cystinosis, comparing high-density auditory-evoked potential (AEP) recordings from adults with cystinosis (N = 15; ages: 19–38 years) to those of age-matched controls (N = 17). We employed a duration oddball paradigm with different stimulation rates, in which participants passively listened to regularly occurring standard tones interspersed with infrequently occurring deviant tones. Analyses focused on AEP components reflecting auditory sensory-perceptual processing (N1 and P2), sensory memory (mismatch negativity, MMN), and attentional orienting (P3a). Results Overall, adults with cystinosis produced highly similar sensory-perceptual AEP responses to those observed in controls suggesting intact early auditory cortical processing. However, significantly increased P2 and P3a amplitudes and reduced MMN at slower stimulation rates were observed, suggesting mild-to-moderate changes in auditory sensory memory and attentional processing. While cognitive testing revealed lower scores on verbal IQ and perceptual reasoning in cystinosis, these did not correlate with the AEP measures. Conclusions These neurophysiological data point to the emergence of subtle auditory processing deficits in early adulthood in cystinosis, warranting further investigation of memory and attentional processes in this population, and of their consequences for perceptual and cognitive function.

scholarly journals Electrophysiological evidence for impaired auditory sensory memory in Cystinosis despite typical sensory processing: An MMN investigation

2019 ◽  
Author(s):  
Ana A. Francisco ◽  
John J. Foxe ◽  
Douwe J. Horsthuis ◽  
Sophie Molholm
Keyword(s):  
Children And Adolescents ◽  
Auditory Processing ◽  
Brain Function ◽  
Stimulus Onset ◽  
Sensory Memory ◽  
Oddball Paradigm ◽  
Order Processing ◽  
Auditory Sensory Memory ◽  
Age Range ◽  
Future Work

AbstractCystinosis, a genetic rare disease characterized by cystine accumulation and crystallization, results in significant damage in a multitude of tissues and organs, such as the kidney, thyroid, eye, and brain. While Cystinosis’ impact on brain function is relatively mild compared to its effects on other organs, the increased lifespan of this population and thus potential for productive societal contributions have led to increased interest on the effects on brain function. Nevertheless, and despite some evidence of structural brain differences, the neural impact of the mutation is still not well characterized.Here, using a passive duration oddball paradigm (with different stimulus onset asynchronies (SOAs), representing different levels of demand on memory) and high-density electrophysiology, we tested basic auditory processing in a group of 22 children and adolescents diagnosed with Cystinosis (age range: 6-17 years old) and in neurotypical age-matched controls (N=24). We examined whether the N1 and mismatch negativity (MMN) significantly differed between the groups and if those neural measures correlated with verbal and non-verbal IQ. Individuals diagnosed with Cystinosis presented similar N1 responses to their age-matched peers, indicating typical basic auditory processing in this population. However, whereas both groups showed similar MMN responses for the shortest (450ms) SOA, suggesting intact change detection and sensory memory, individuals diagnosed with Cystinosis presented clearly reduced responses for the longer (900ms and 1800ms) SOAs. This could indicate reduced duration auditory sensory memory traces, and thus sensory memory impairment, in children and adolescents diagnosed with Cystinosis. Future work addressing other aspects of sensory and working memory is needed to understand the underlying bases of the differences described here, and their implication for higher order processing.

scholarly journals Effects of Visual Attentional Load on the Tactile Sensory Memory Indexed by Somatosensory Mismatch Negativity

2020 ◽  
Vol 14 ◽  
Author(s):  
Xin He ◽  
Jian Zhang ◽  
Zhilin Zhang ◽  
Ritsu Go ◽  
Jinglong Wu ◽  
...  
Keyword(s):  
Sustained Attention ◽  
Mismatch Negativity ◽  
Memory Trace ◽  
Sensory Memory ◽  
Oddball Paradigm ◽  
Past Century ◽  
Dynamic Changes ◽  
Auditory Sensory Memory ◽  
Attentional Load ◽  
Tactile Stimuli

Auditory sensory memory indexed by mismatch negativity has been broadly studied over the past century, but far less attention has been directed to tactile sensory memory. To investigate whether tactile sensory memory is affected by attention, we recorded somatosensory mismatch negativity (sMMN) from 24 healthy adults in two experiments to distinguish sustained attention from non-sustained attention. Using the roving somatosensory oddball paradigm, we analyzed the average dynamic changes in the amplitude and latency of sMMN amplitude and found a clear sMMN component at the central region at a 100–300 ms interval. The sMMN amplitude, which indexes the early detection of tactile stimuli with the sensory memory trace, was larger in the tactile attentional task. Additionally, the sMMN latency increased with the increasing visual attentional load, which indicates a decay of tactile sensory memory. Our results indicate that the more attention resources are allocated for a tactile sensation, the more favorable it is to the generation of tactile sensory memory.

Feature conjunctions and auditory sensory memory

1998 ◽  
Vol 793 (1-2) ◽  
pp. 95-102 ◽  
Author(s):  
Elyse Sussman ◽  
Hilary Gomes ◽  
Jo Manette K Nousak ◽  
Walter Ritter ◽  
Herbert G Vaughan
Keyword(s):  
Sensory Memory ◽  
Auditory Sensory Memory

scholarly journals Evaluating the Precision of Auditory Sensory Memory as an Index of Intrusion in Tinnitus

2017 ◽  
Vol 38 (2) ◽  
pp. 262-265 ◽  
Author(s):  
Doug J. K. Barrett ◽  
Michael Pilling
Keyword(s):  
Sensory Memory ◽  
Auditory Sensory Memory

Informational Masking Effects of Speech Versus Nonspeech Noise on Cortical Auditory Evoked Potentials

2021 ◽  
Vol 64 (10) ◽  
pp. 4014-4029
Author(s):  
Kathy R. Vander Werff ◽  
Christopher E. Niemczak ◽  
Kenneth Morse
Keyword(s):  
Auditory Processing ◽  
Auditory Evoked Potentials ◽  
Spectral Characteristics ◽  
Auditory Evoked Potential ◽  
Informational Masking ◽  
Neural Encoding ◽  
Speech Stimuli ◽  
Typical Morphology ◽  
Cortical Auditory Evoked Potentials ◽  
Speech Information

Purpose Background noise has been categorized as energetic masking due to spectrotemporal overlap of the target and masker on the auditory periphery or informational masking due to cognitive-level interference from relevant content such as speech. The effects of masking on cortical and sensory auditory processing can be objectively studied with the cortical auditory evoked potential (CAEP). However, whether effects on neural response morphology are due to energetic spectrotemporal differences or informational content is not fully understood. The current multi-experiment series was designed to assess the effects of speech versus nonspeech maskers on the neural encoding of speech information in the central auditory system, specifically in terms of the effects of speech babble noise maskers varying by talker number. Method CAEPs were recorded from normal-hearing young adults in response to speech syllables in the presence of energetic maskers (white or speech-shaped noise) and varying amounts of informational maskers (speech babble maskers). The primary manipulation of informational masking was the number of talkers in speech babble, and results on CAEPs were compared to those of nonspeech maskers with different temporal and spectral characteristics. Results Even when nonspeech noise maskers were spectrally shaped and temporally modulated to speech babble maskers, notable changes in the typical morphology of the CAEP in response to speech stimuli were identified in the presence of primarily energetic maskers and speech babble maskers with varying numbers of talkers. Conclusions While differences in CAEP outcomes did not reach significance by number of talkers, neural components were significantly affected by speech babble maskers compared to nonspeech maskers. These results suggest an informational masking influence on neural encoding of speech information at the sensory cortical level of auditory processing, even without active participation on the part of the listener.

Information Theory Predicts the Magnitude of an MEG Response Related to the Operation of the Auditory Sensory Memory

Biomag 96 ◽  
2000 ◽  
pp. 872-873
Author(s):  
J. Sinkkonen ◽  
H. Tiitinen ◽  
P. May ◽  
R. J. Ilmoniemi ◽  
R. Näätänen
Keyword(s):  
Information Theory ◽  
Sensory Memory ◽  
Auditory Sensory Memory

scholarly journals Gaucher's Disease - A case report

2015 ◽  
Vol 2 (2) ◽  
pp. 130
Author(s):  
Preeti Bajaj ◽  
Jyoti Kasture ◽  
Balbir Singh Shah
Keyword(s):  
Autosomal Recessive ◽  
Genetic Disorder ◽  
Early Adulthood ◽  
Lysosomal Storage ◽  
Gaucher's Disease ◽  
Gaucher’S Disease ◽  
Type 3 ◽  
Storage Disorders

Gaucher's Disease (GD) is an autosomal recessive systemic lysosomal storage disorder which is characterized by glucocerebroside deposition in cells of the macrophage-monocyte system as a result of a deficiency in lysosomal P-glycosidase (glucocerebrosidase). GD is a rare genetic disorder. It is the most common amongst the lysosomal storage disorders. GD has been categorised into three types based on the presence of central nervous involvement1. Type 1 is a non-neuronopathic form that presents in childhood or early adulthood. Type 2 is acute neuronopathic form that presents in childhood. It progresses rapidly and is fatal. Type 3 is chronic non-neuronopathic form that presents in childhood but is slowly progressive. Here we describe a case of a three and a half year old male child in whom a diagnosis of Gaucher's disease was made based on bone marrow biopsy and later confirmed by glucocerebrosidase levels estimation.

scholarly journals Going beyond rote auditory learning: Neural patterns of generalized auditory learning

2021 ◽  
Author(s):  
Shannon L.M. Heald ◽  
Stephen C. Van Hedger ◽  
John Veillette ◽  
Katherine Reis ◽  
Joel S. Snyder ◽  
...  
Keyword(s):  
Speech Perception ◽  
Sensory Processing ◽  
Auditory Processing ◽  
Auditory Evoked Potentials ◽  
Brain Regions ◽  
Auditory Evoked Potential ◽  
Cortical Processing ◽  
Rote Learning ◽  
Neural Responses ◽  
Auditory Learning

AbstractThe ability to generalize rapidly across specific experiences is vital for robust recognition of new patterns, especially in speech perception considering acoustic-phonetic pattern variability. Behavioral research has demonstrated that listeners are rapidly able to generalize their experience with a talker’s speech and quickly improve understanding of a difficult-to-understand talker without prolonged practice, e.g., even after a single training session. Here, we examine the differences in neural responses to generalized versus rote learning in auditory cortical processing by training listeners to understand a novel synthetic talker using a Pretest-Posttest design with electroencephalography (EEG). Participants were trained using either (1) a large inventory of words where no words repeated across the experiment (generalized learning) or (2) a small inventory of words where words repeated (rote learning). Analysis of long-latency auditory evoked potentials at Pretest and Posttest revealed that while rote and generalized learning both produce rapid changes in auditory processing, the nature of these changes differed. In the context of adapting to a talker, generalized learning is marked by an amplitude reduction in the N1-P2 complex and by the presence of a late-negative (LN) wave in the auditory evoked potential following training. Rote learning, however, is marked only by temporally later source configuration changes. The early N1-P2 change, found only for generalized learning, suggests that generalized learning relies on the attentional system to reorganize the way acoustic features are selectively processed. This change in relatively early sensory processing (i.e. during the first 250ms) is consistent with an active processing account of speech perception, which proposes that the ability to rapidly adjust to the specific vocal characteristics of a new talker (for which rote learning is rare) relies on attentional mechanisms to adaptively tune early auditory processing sensitivity.Statement of SignificancePrevious research on perceptual learning has typically examined neural responses during rote learning: training and testing is carried out with the same stimuli. As a result, it is not clear that findings from these studies can explain learning that generalizes to novel patterns, which is critical in speech perception. Are neural responses to generalized learning in auditory processing different from neural responses to rote learning? Results indicate rote learning of a particular talker’s speech involves brain regions focused on the memory encoding and retrieving of specific learned patterns, whereas generalized learning involves brain regions involved in reorganizing attention during early sensory processing. In learning speech from a novel talker, only generalized learning is marked by changes in the N1-P2 complex (reflective of secondary auditory cortical processing). The results are consistent with the view that robust speech perception relies on the fast adjustment of attention mechanisms to adaptively tune auditory sensitivity to cope with acoustic variability.

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