scholarly journals Auditory Phenotypic Variability in Friedreich’s Ataxia Patients

2021 ◽  
Author(s):  
Nehzat Koohi ◽  
Gilbert Thomas-Black ◽  
Paola Giunti ◽  
Doris-Eva Bamiou

AbstractAuditory neural impairment is a key clinical feature of Friedreich’s Ataxia (FRDA). We aimed to characterize the phenotypical spectrum of the auditory impairment in FRDA in order to facilitate early identification and timely management of auditory impairment in FRDA patients and to explore the relationship between the severity of auditory impairment with genetic variables (the expansion size of GAA trinucleotide repeats, GAA1 and GAA2), when controlled for variables such as disease duration, severity of the disease and cognitive status. Twenty-seven patients with genetically confirmed FRDA underwent baseline audiological assessment (pure-tone audiometry, otoacoustic emissions, auditory brainstem response). Twenty of these patients had additional psychophysical auditory processing evaluation including an auditory temporal processing test (gaps in noise test) and a binaural speech perception test that assesses spatial processing (Listening in Spatialized Noise-Sentences Test). Auditory spatial and auditory temporal processing ability were significantly associated with the repeat length of GAA1. Patients with GAA1 greater than 500 repeats had more severe auditory temporal and spatial processing deficits, leading to poorer speech perception. Furthermore, the spatial processing ability was strongly correlated with the Montreal Cognitive Assessment (MoCA) score. To our knowledge, this is the first study to demonstrate an association between genotype and auditory spatial processing phenotype in patients with FRDA. Auditory temporal processing, neural sound conduction, spatial processing and speech perception were more severely affected in patients with GAA1 greater than 500 repeats. The results of our study may indicate that auditory deprivation plays a role in the development of mild cognitive impairment in FRDA patients.

1999 ◽  
Vol 8 (S3) ◽  
pp. S28-S34 ◽  
Author(s):  
G. Schulte-Körne ◽  
W. Deimel ◽  
J. Bartling ◽  
H. Remschmidt

2020 ◽  
Vol 24 ◽  
pp. 233121652093616
Author(s):  
Maureen J. Shader ◽  
Sandra Gordon-Salant ◽  
Matthew J. Goupell

Although cochlear implants (CIs) are a viable treatment option for severe hearing loss in adults of any age, older adults may be at a disadvantage compared with younger adults. CIs deliver signals that contain limited spectral information, requiring CI users to attend to the temporal information within the signal to recognize speech. Older adults are susceptible to acquiring auditory temporal processing deficits, presenting a potential age-related limitation for recognizing speech signals delivered by CIs. The goal of this study was to measure auditory temporal processing ability via amplitude-modulation (AM) detection as a function of age in CI users. The contribution of the electrode-to-neural interface, in addition to age, was estimated using electrically evoked compound action potential (ECAP) amplitude growth functions. Within each participant, two electrodes were selected: one with the steepest ECAP slope and one with the shallowest ECAP slope, in order to represent electrodes with varied estimates of the electrode-to-neural interface. Single-electrode AM detection thresholds were measured using direct stimulation at these two electrode locations. Results revealed that AM detection ability significantly declined as a function of chronological age. ECAP slope did not significantly impact AM detection, but ECAP slope decreased (became shallower) with increasing age, suggesting that factors influencing the electrode-to-neural interface change with age. Results demonstrated a significant negative impact of chronological age on auditory temporal processing. The locus of the age-related limitation (peripheral vs. central origin), however, is difficult to evaluate because the peripheral influence (ECAPs) was correlated with the central factor (age).


2020 ◽  
Vol 24 ◽  
pp. 233121652095656
Author(s):  
Maureen J. Shader ◽  
Sandra Gordon-Salant ◽  
Matthew J. Goupell

Accurate processing of temporal information is critical to understanding speech through a cochlear implant (CI). This has potential implications for the growing population of CI users who are ≥65 years of age because of age-related auditory temporal processing deficits. The goal of this study was to measure temporal processing ability in a gap detection task in younger, middle-aged, and older CI users and to determine the relative contributions of chronological age and peripheral neural survival to performance. Single-electrode gap detection thresholds (GDTs) were measured using direct stimulation at five electrode locations and three electrical stimulation rates. The relationship between peripheral status (e.g., electrode-to-neural interface) and GDTs was assessed by the slope of the electrically evoked compound action potential (ECAP) amplitude growth function. Results showed that ECAP slope was the strongest subject-level predictor of GDTs. Steeper ECAP slopes, which are partially indicative of better peripheral function, were associated with better GDTs in younger participants. However, ECAP slope significantly interacted with stimulation rate and age, suggesting that ECAP slopes were not predictive of GDTs in middle-aged and older participants at some stimulation rates. ECAP slope was also related to age, with middle-aged and older participants exhibiting relatively shallow slopes and smaller ranges of slopes compared with younger participants. This pattern of ECAP results limited the evaluation of the independent effects of aging per se and peripheral status on temporal processing ability.


1983 ◽  
Vol 92 (3) ◽  
pp. 276-280 ◽  
Author(s):  
A. T. Cacace ◽  
S. Satya-Murti ◽  
C. T. Grimes

We performed psychoacoustical tuning curves and measured temporal summation in two patients with Friedreich's ataxia to study aspects of frequency selectivity and temporal processing in the auditory system. In the majority of frequencies tested, normal psychoacoustical tuning curves and unimpaired temporal summation functions were obtained. These findings suggest that the processing mechanism responsible for these phenomena is intact.


1995 ◽  
Vol 18 (1) ◽  
pp. 189-189 ◽  
Author(s):  
Roslyn Holly Fitch ◽  
Paula Tallal

AbstractWilkins & Wakefield suggest that changes in the hominid brain made it uniquely “preadaptive” for language, yet no precursor functions served as adaptive substrates to the emergence of language. We present contrary evidence that the ability to discriminate and process rapid and complex auditory information is a cross-species function subserving communication processes including, but not limited to, human speech perception. We suggest that auditory temporal processing served as an evolutionary precursor to speech processing and consequent language development in humans.


2021 ◽  
Author(s):  
Shauni Van Herck ◽  
Femke Vanden Bempt ◽  
Maria Economou ◽  
Jolijn Vanderauwera ◽  
Toivo Glatz ◽  
...  

Dyslexia has frequently been related to atypical auditory temporal processing and speech perception. Results of studies emphasizing speech onset cues and reinforcing the temporal structure of the speech envelope, i.e. envelope enhancement, demonstrated reduced speech perception deficits in individuals with dyslexia. The use of this strategy as an auditory intervention might thus reduce some of the deficits related to dyslexia. Importantly, interventions are most effective when they are provided during kindergarten and first grade. Hence, we provided a tablet-based 12-week preventive auditory and phonics-based intervention to pre-readers at cognitive risk for dyslexia and investigated the effect on auditory temporal processing with a rise time discrimination task. Ninety-one pre-readers at cognitive risk for dyslexia (aged 5-6) were assigned to two groups receiving a phonics-based intervention and playing a story listening game either with (n = 31) or without (n = 31) envelope enhancement or a third group playing control games and listening to non-enhanced stories (n = 29). Rise time discrimination was measured directly before, directly after and one year after the intervention. While the groups listening to non-enhanced stories mainly improved after the intervention during first grade, the group listening to enhanced stories improved during the intervention in kindergarten and subsequently remained stable during first grade. Hence, an envelope enhancement intervention improves auditory processing skills important for the development of phonological skills. This occurred before the onset of reading instruction, preceding the maturational improvement of these skills, hence giving at risk children a head start when learning to read.


2020 ◽  
Vol 63 (4) ◽  
pp. 1270-1281
Author(s):  
Leah Fostick ◽  
Riki Taitelbaum-Swead ◽  
Shulamith Kreitler ◽  
Shelly Zokraut ◽  
Miriam Billig

Purpose Difficulty in understanding spoken speech is a common complaint among aging adults, even when hearing impairment is absent. Correlational studies point to a relationship between age, auditory temporal processing (ATP), and speech perception but cannot demonstrate causality unlike training studies. In the current study, we test (a) the causal relationship between a spatial–temporal ATP task (temporal order judgment [TOJ]) and speech perception among aging adults using a training design and (b) whether improvement in aging adult speech perception is accompanied by improved self-efficacy. Method Eighty-two participants aged 60–83 years were randomly assigned to a group receiving (a) ATP training (TOJ) over 14 days, (b) non-ATP training (intensity discrimination) over 14 days, or (c) no training. Results The data showed that TOJ training elicited improvement in all speech perception tests, which was accompanied by increased self-efficacy. Neither improvement in speech perception nor self-efficacy was evident following non-ATP training or no training. Conclusions There was no generalization of the improvement resulting from TOJ training to intensity discrimination or generalization of improvement resulting from intensity discrimination training to speech perception. These findings imply that the effect of TOJ training on speech perception is specific and such improvement is not simply the product of generally improved auditory perception. It provides support for the idea that temporal properties of speech are indeed crucial for speech perception. Clinically, the findings suggest that aging adults can be trained to improve their speech perception, specifically through computer-based auditory training, and this may improve perceived self-efficacy.


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