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1.
Intern Med ; 2024 Oct 04.
Article in English | MEDLINE | ID: mdl-39370257

ABSTRACT

A 54-year-old woman with persistent fatigue and a high fever presented with central pontine myelinolysis (CPM) on brain imaging, despite the absence of neurological symptoms. The patient had a slightly low serum sodium concentration. Further investigation led to a diagnosis of primary splenic diffuse large B-cell lymphoma (PS-DLBCL). Brainstem auditory-evoked potentials (BAEP) indicated minor abnormalities. CPM was successfully resolved after splenectomy and immunochemotherapy. This is the first reported case of CPM in a patient with PS-DLBCL confirmed by a pathological diagnosis following splenectomy and BAEP-detected abnormalities that improved with immunochemotherapy.

2.
Brain Lang ; 258: 105474, 2024 Sep 25.
Article in English | MEDLINE | ID: mdl-39326253

ABSTRACT

Infants quickly recognize the sounds of their mother language, perceiving the spectrotemporal acoustic features of speech. However, the underlying neural machinery remains unclear. We used an auditory evoked potential termed frequency-following response (FFR) to unravel the neural encoding maturation for two speech sound characteristics: voice pitch and temporal fine structure. 37 healthy-term neonates were tested at birth and retested at the ages of six and twelve months. Results revealed a reduction in neural phase-locking onset to the stimulus envelope from birth to six months, stabilizing by twelve months. While neural encoding of voice pitch remained consistent across ages, temporal fine structure encoding matured rapidly from birth to six months, without further improvement from six to twelve months. Results highlight the critical importance of the first six months of life in the maturation of neural encoding mechanisms that are crucial for phoneme discrimination during early language acquisition.

3.
Int J Audiol ; : 1-10, 2024 Aug 20.
Article in English | MEDLINE | ID: mdl-39162810

ABSTRACT

OBJECTIVE: We have shown that the acoustic change complex (ACC) can be elicited by changing the horizontal sound location in young individuals. In this study, we aimed to evaluate the application of ACC within the elderly and its relationship with behavioural results. DESIGN: The minimum audible angle (MAA), as well as onset cortical auditory evoked potentials (onset-CAEPs) and ACC elicited by the stimuli of location-change white noise (±45 to ±2 degrees) were recorded. Latencies and amplitudes were analysed using repeated-measures ANOVA. Pearson correlation analysis was conducted to examine the relationship between ACC and MAA. STUDY SAMPLE: Ten older adults with normal hearing (NH) and twenty with presbycusis. RESULTS: The ACC was effectively elicited with angular variations in elderly participants. The onset-CAEP N1 latency, ACC N1'-P2' amplitude, and N1' latency were all associated with the angle shifts, with the N1' latency being the most predictive factor for angle discrimination. The consistency between MAA and ACC made them complementary for the clinical evaluation of sound localisation. CONCLUSIONS: The utilisation of ACC, evoked by location-change sounds, presented a promising clinical objective measure for evaluating sound localisation abilities in the elderly.

4.
Article in English | MEDLINE | ID: mdl-39090303

ABSTRACT

INTRODUCTION: Although a broadband acoustic click is physically the shortest duration sound we can hear, its peripheral neural representation is not as short because of cochlear filtering. The traveling wave imposes frequency-dependent delays to the sound waveform so that in response to a click, apical nerve fibers, coding for low frequencies, are excited several milliseconds after basal fibers, coding for high frequencies. Nevertheless, a click sounds like a click and these across-fiber delays are not perceived. This suggests that they may be compensated by the central auditory system, rendering our perception consistent with the external world. This explanation is difficult to evaluate in normal-hearing listeners because the contributions of peripheral and central auditory processing cannot easily be disentangled. Here, we test this hypothesis in cochlear implant listeners for whom cochlear mechanics is bypassed. METHOD: Eight cochlear implant users ranked in perceived duration 12 electrical chirps of various physical durations and spanning the cochlea in the apex-to-base or base-to-apex direction (Exp. 1). Late-latency cortical potentials were also recorded in response to a subset of these chirps (Exp. 2). RESULTS: We show that an electrical chirp spanning the cochlea from base-to-apex is perceived as shorter than the same chirp spanning the cochlea in the opposite direction despite having the same physical duration. Cortical potentials also provide neural correlates of this asymmetry in perception. CONCLUSION: These results demonstrate that the central auditory system processes frequency sweeps differently depending on the direction of the frequency change and that this processing difference is not simply the result of peripheral filtering.

5.
Discov Med ; 36(187): 1672-1677, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39190382

ABSTRACT

BACKGROUND: Severe neonatal hyperbilirubinemia can cause hearing impairment. Bilirubin can be deposited in nerve cells, and the brainstem and the 8th nerve are especially sensitive to bilirubin toxicity. Abnormal changes in brainstem auditory evoked potential (BAEP) can be observed, and the BAEP test measures a nerve potential induced by short, high-frequency sound stimulation; thus, it is able to detect damage to the auditory conduction pathway in children. We aimed to identify relationships between clinical features and BAEP abnormalities in children with hyperbilirubinemia and to assess the predictive power of these risk factors for bilirubin-induced neurological damage. METHODS: Children with hyperbilirubinemia were evaluated with BAEP and retrospectively enrolled in the study between January 2012 and December 2018. Multivariate logistic regression was performed to identify independent predictors of BAEP abnormalities. RESULTS: Of the 561 children with hyperbilirubinemia enrolled, the BAEP anomaly group accounted for 198 (35.3%) cases. Except for body weight, there were no significant differences in the general data between the two groups with hyperbilirubinemia (p > 0.05). Univariate analysis showed that prematurity, abnormal umbilical cord, and gestational diabetes during pregnancy were significantly correlated with abnormal BAEP. Multivariate logistic regression analysis identified prematurity (p = 0.001), gestational diabetes (p = 0.03), Premature rupture of membranes (p = 0.013), total serum bilirubin (TSB), bilirubin/albumin (B/A) as independent risk factors for BAEP abnormalities. The prediction accuracy of TSB (Area Under Curve (AUC) = 0.557) and B/A (AUC = 0.566) was low, indicating that abnormal BAEP should be detected by multiple factors. CONCLUSIONS: Multivariate detection is beneficial for predicting the occurrence of auditory nerve injury in patients with hyperbilirubinemia.


Subject(s)
Evoked Potentials, Auditory, Brain Stem , Hyperbilirubinemia, Neonatal , Humans , Female , Infant, Newborn , Male , Retrospective Studies , Hyperbilirubinemia, Neonatal/blood , Hyperbilirubinemia, Neonatal/physiopathology , Hyperbilirubinemia, Neonatal/complications , Hyperbilirubinemia, Neonatal/diagnosis , Risk Factors , Bilirubin/blood , Pregnancy , Diabetes, Gestational/physiopathology , Diabetes, Gestational/blood
6.
Hear Res ; 451: 109090, 2024 09 15.
Article in English | MEDLINE | ID: mdl-39047579

ABSTRACT

OBJECTIVE: The acoustic change complex (ACC) is a cortical auditory evoked potential (CAEP) and can be elicited by a change in an otherwise continuous sound. The ACC has been highlighted as a promising tool in the assessment of sound and speech discrimination capacity, and particularly for difficult-to-test populations such as infants with hearing loss, due to the objective nature of ACC measurements. Indeed, there is a pressing need to develop further means to accurately and thoroughly establish the hearing status of children with hearing loss, to help guide hearing interventions in a timely manner. Despite the potential of the ACC method, ACC measurements remain relatively rare in a standard clinical settings. The objective of this study was to perform an up-to-date systematic review on ACC measurements in children, to provide greater clarity and consensus on the possible methodologies, applications, and performance of this technique, and to facilitate its uptake in relevant clinical settings. DESIGN: Original peer-reviewed articles conducting ACC measurements in children (< 18 years). Data were extracted and summarised for: (1) participant characteristics; (2) ACC methods and auditory stimuli; (3) information related to the performance of the ACC technique; (4) ACC measurement outcomes, advantages, and challenges. The systematic review was conducted using PRISMA guidelines for reporting and the methodological quality of included articles was assessed. RESULTS: A total of 28 studies were identified (9 infant studies). Review results show that ACC responses can be measured in infants (from < 3 months), and there is evidence of age-dependency, including increased robustness of the ACC response with increasing childhood age. Clinical applications include the measurement of the neural capacity for speech and non-speech sound discrimination in children with hearing loss, auditory neuropathy spectrum disorder (ANSD) and central auditory processing disorder (CAPD). Additionally, ACCs can be recorded in children with hearing aids, auditory brainstem implants, and cochlear implants, and ACC results may guide hearing intervention/rehabilitation strategies. The review identified that the time taken to perform ACC measurements was often lengthy; the development of more efficient ACC test procedures for children would be beneficial. Comparisons between objective ACC measurements and behavioural measures of sound discrimination showed significant correlations for some, but not all, included studies. CONCLUSIONS: ACC measurements of the neural capacity to discriminate between speech and non-speech sounds are feasible in infants and children, and a wide range of possible clinical applications exist, although more time-efficient procedures would be advantageous for clinical uptake. A consideration of age and maturational effects is recommended, and further research is required to investigate the relationship between objective ACC measures and behavioural measures of sound and speech perception for effective clinical implementation.


Subject(s)
Acoustic Stimulation , Auditory Perception , Evoked Potentials, Auditory , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Age Factors , Auditory Cortex/physiology , Auditory Cortex/physiopathology , Auditory Pathways/physiopathology , Auditory Pathways/physiology , Hearing , Hearing Loss/physiopathology , Hearing Loss/diagnosis , Hearing Loss/rehabilitation , Persons With Hearing Impairments/psychology , Persons With Hearing Impairments/rehabilitation , Predictive Value of Tests , Reproducibility of Results , Speech Discrimination Tests
7.
Audiol Neurootol ; : 1-12, 2024 Jul 31.
Article in English | MEDLINE | ID: mdl-39084205

ABSTRACT

INTRODUCTION: Electrically evoked cortical auditory evoked potentials (ECAEPs) are central brain responses to auditory stimuli that correlate with postoperative cochlear implant (CI) hearing outcomes. They differ from electrically evoked compound action potentials (ECAPs) which are peripheral responses that can be elicited intraoperatively and may also predict CI hearing outcomes. It is not known to what degree ECAP and ECAEP responses are associated with each other. Such a correlation, if present, may allow for an earlier and more accurate prediction of postoperative hearing outcomes. METHODS: This retrospective study involved 42 adult CI users. Threshold levels and amplitude growth function slopes of intraoperative ECAPs were compared to the latencies and peak-to-peak amplitudes of postoperative ECAEP responses at three different cochlear electrode array sites (apical, medial, and basal). RESULTS: A weak positive relationship was found between intraoperative ECAP thresholds and ECAEP N1-P2 peak-to-peak amplitude (r = 0.301, p = 0.005). Time between ECAP and ECAEP measurements was weakly correlated with P1-N1 peak-to-peak amplitude (r = 0.321, p = 0.002) and ECAEP N1-P2 peak-to-peak amplitude (r = 0.340, p = 0.001). ECAP amplitude growth function slopes varied by electrode location (χ2 = 26.701, df = 2, p = 0. 000002). CONCLUSION: These results suggest that intraoperative ECAP responses do not robustly predict postoperative ECAEP responses, providing caution against the use of ECAPs as a predictive tool for CI hearing outcomes.

8.
Front Neuroergon ; 5: 1358660, 2024.
Article in English | MEDLINE | ID: mdl-38989056

ABSTRACT

Introduction: To understand brain function in natural real-world settings, it is crucial to acquire brain activity data in noisy environments with diverse artifacts. Electroencephalography (EEG), while susceptible to environmental and physiological artifacts, can be cleaned using advanced signal processing techniques like Artifact Subspace Reconstruction (ASR) and Independent Component Analysis (ICA). This study aims to demonstrate that ASR and ICA can effectively extract brain activity from the substantial artifacts occurring while skateboarding on a half-pipe ramp. Methods: A dual-task paradigm was used, where subjects were presented with auditory stimuli during skateboarding and rest conditions. The effectiveness of ASR and ICA in cleaning artifacts was evaluated using a support vector machine to classify the presence or absence of a sound stimulus in single-trial EEG data. The study evaluated the effectiveness of ASR and ICA in artifact cleaning using five different pipelines: (1) Minimal cleaning (bandpass filtering), (2) ASR only, (3) ICA only, (4) ICA followed by ASR (ICAASR), and (5) ASR preceding ICA (ASRICA). Three skateboarders participated in the experiment. Results: Results showed that all ICA-containing pipelines, especially ASRICA (69%, 68%, 63%), outperformed minimal cleaning (55%, 52%, 50%) in single-trial classification during skateboarding. The ASRICA pipeline performed significantly better than other pipelines containing ICA for two of the three subjects, with no other pipeline performing better than ASRICA. The superior performance of ASRICA likely results from ASR removing non-stationary artifacts, enhancing ICA decomposition. Evidenced by ASRICA identifying more brain components via ICLabel than ICA alone or ICAASR for all subjects. For the rest condition, with fewer artifacts, the ASRICA pipeline (71%, 82%, 75%) showed slight improvement over minimal cleaning (73%, 70%, 72%), performing significantly better for two subjects. Discussion: This study demonstrates that ASRICA can effectively clean artifacts to extract single-trial brain activity during skateboarding. These findings affirm the feasibility of recording brain activity during physically demanding tasks involving substantial body movement, laying the groundwork for future research into the neural processes governing complex and coordinated body movements.

9.
J Neurodev Disord ; 16(1): 28, 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38831410

ABSTRACT

BACKGROUND: In the search for objective tools to quantify neural function in Rett Syndrome (RTT), which are crucial in the evaluation of therapeutic efficacy in clinical trials, recordings of sensory-perceptual functioning using event-related potential (ERP) approaches have emerged as potentially powerful tools. Considerable work points to highly anomalous auditory evoked potentials (AEPs) in RTT. However, an assumption of the typical signal-averaging method used to derive these measures is "stationarity" of the underlying responses - i.e. neural responses to each input are highly stereotyped. An alternate possibility is that responses to repeated stimuli are highly variable in RTT. If so, this will significantly impact the validity of assumptions about underlying neural dysfunction, and likely lead to overestimation of underlying neuropathology. To assess this possibility, analyses at the single-trial level assessing signal-to-noise ratios (SNR), inter-trial variability (ITV) and inter-trial phase coherence (ITPC) are necessary. METHODS: AEPs were recorded to simple 100 Hz tones from 18 RTT and 27 age-matched controls (Ages: 6-22 years). We applied standard AEP averaging, as well as measures of neuronal reliability at the single-trial level (i.e. SNR, ITV, ITPC). To separate signal-carrying components from non-neural noise sources, we also applied a denoising source separation (DSS) algorithm and then repeated the reliability measures. RESULTS: Substantially increased ITV, lower SNRs, and reduced ITPC were observed in auditory responses of RTT participants, supporting a "neural unreliability" account. Application of the DSS technique made it clear that non-neural noise sources contribute to overestimation of the extent of processing deficits in RTT. Post-DSS, ITV measures were substantially reduced, so much so that pre-DSS ITV differences between RTT and TD populations were no longer detected. In the case of SNR and ITPC, DSS substantially improved these estimates in the RTT population, but robust differences between RTT and TD were still fully evident. CONCLUSIONS: To accurately represent the degree of neural dysfunction in RTT using the ERP technique, a consideration of response reliability at the single-trial level is highly advised. Non-neural sources of noise lead to overestimation of the degree of pathological processing in RTT, and denoising source separation techniques during signal processing substantially ameliorate this issue.


Subject(s)
Electroencephalography , Evoked Potentials, Auditory , Rett Syndrome , Humans , Rett Syndrome/physiopathology , Rett Syndrome/complications , Adolescent , Female , Evoked Potentials, Auditory/physiology , Child , Young Adult , Auditory Perception/physiology , Reproducibility of Results , Acoustic Stimulation , Male , Signal-To-Noise Ratio , Adult
10.
J Neurosurg ; : 1-10, 2024 Jun 28.
Article in English | MEDLINE | ID: mdl-38941638

ABSTRACT

OBJECTIVE: Advancements in microsurgical technique and technology continue to improve outcomes in patients with skull base tumor. The primary cranial nerve eight monitoring systems used in hearing preservation surgery for vestibular schwannomas (VSs) are direct cranial nerve eight monitoring (DCNEM) and auditory brainstem response (ABR), although current guidelines are unable to definitively recommend one over the other due to limited literature on the topic. Thus, further research is needed to determine the utility of DCNEM and ABR. The authors performed a retrospective cohort study and created an interactive model that compares hearing preservation outcomes based on tumor size in patients receiving ABR+DCNEM and ABR-only monitoring. METHODS: Twenty-eight patients received ABR+DCNEM and 72 patients received ABR-only monitoring during VS hearing preservation surgery at a single tertiary academic medical center between January 2008 and November 2022. Inclusion criteria consisted of adult patients with a preoperative American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification of A or B. Tumor size was measured as the maximal medial to lateral length, including the internal auditory canal component. RESULTS: Overall hearing preservation (word recognition score [WRS] > 0%) was achieved in 31 patients with ABR-only monitoring (43.1%) and in 18 patients with ABR+DCNEM (64.3%). Serviceable hearing preservation (AAO-HNS class A or B) was attained in 19 patients with ABR-only monitoring (26.4%) and in 11 patients with ABR+DCNEM (39.3%). There was no difference in overall hearing preservation between the two groups (p = 0.13). Change in tumor size was not associated with the odds of serviceable hearing preservation for the ABR-only group (p = 0.89); however, for ABR+DCNEM, there was some indication of an interaction between tumor size and the association of ABR+DCNEM versus ABR-only monitoring, with the odds of serviceable hearing preservation at p = 0.089. Furthermore, with ABR+DCNEM, every 0.5-cm increase in tumor size was associated with a decreased odds of serviceable hearing preservation on multivariable analysis (p = 0.05). For both overall and serviceable hearing preservation, a worse preoperative AAO-HNS classification was associated with a decreased odds of preservation (OR 0.43, 95% CI 0.19-0.97, p = 0.042; OR 0.17, 95% CI 0.053-0.55, p = 0.0031, respectively). CONCLUSIONS: The result of this interactive model study proposes that there may be a higher chance of hearing preservation when using ABR+DCNEM rather than ABR alone for smaller tumors, with that relationship reversing as tumor size increases.

11.
J Neurosci Res ; 102(6): e25362, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38895852

ABSTRACT

Sudden infant death syndrome (SIDS)-the sudden and unexplained death of a seemingly healthy infant, <1 year old-may be associated with abnormalities in the brain regions that underlie breathing and arousal during sleep. While post-mortem studies suggest abnormalities in SIDS infants' brainstems, there are no studies of these infants' brainstem function before death. One way to assess the function of the brainstem is with auditory brainstem response (ABR), a routine hearing-screening method that noninvasively measures the brainstem's response to sound. We hypothesize that anomalies in newborns' ABR measures may predict SIDS. Indeed, previous studies identified abnormalities in ABR characteristics in small samples of near-miss SIDS infants hospitalized for infant apnea syndrome. However, there is a need to examine the ABRs of infants who died of SIDS. Therefore, in the current study, we propose integrating two secondary datasets to examine newborns' ABRs (N = 156,972), including those who later died of SIDS (n = ~42; .27 out of every 1000 infants), using existing archived records of neonatal ABR results from a sample of newborns born in Florida. We hypothesize that infants who die from SIDS are more likely than non-SIDS infants to have abnormal ABRs as newborns. Understanding the association between SIDS and ABR may facilitate more accurate identification of an infant's risk for SIDS at birth, enabling increased monitoring, which may facilitate interventions and improve survivorship.


Subject(s)
Evoked Potentials, Auditory, Brain Stem , Sudden Infant Death , Humans , Evoked Potentials, Auditory, Brain Stem/physiology , Infant, Newborn , Male , Female , Brain Stem/physiopathology , Infant
13.
Cureus ; 16(4): e57993, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38738130

ABSTRACT

Bickerstaff brainstem encephalitis (BBE) is a rare disorder that is characterized by ophthalmoplegia, ataxia, and disturbance in consciousness. Definite diagnosis is made primarily through clinical presentation and serology testing with anti-GQ1b antibody. However, in a country where access to serologic testing is scarce, electrophysiologic tests such as brainstem auditory evoked response (BAER) may contribute to the diagnosis. Due to its rarity and generally good prognosis, there is no established consensus for the treatment of BBE. Immunomodulatory treatments such as intravenous immunoglobulin (IVIG), plasma exchange, steroids, or a combination of these therapies are often used with good response. However, there are severe cases that respond poorly to these conventional treatments. We report the case of a 26-year-old Filipino man who came in for sudden onset of diplopia, with a one-week history of upper respiratory tract infection. Subsequently, he developed paresthesias, quadriparesis, and an altered level of consciousness. On initial examination, he only had partial third nerve palsy, but eventually became quadriparetic and obtunded during admission. Initial electromyography and nerve conduction velocity (EMG-NCV) study showed a reduced recruitment pattern of the right rectus femoris, absent H reflexes of bilateral posterior tibial nerves, and no abnormal increase in temporal dispersion. Cranial MRI with contrast was unremarkable. Video electroencephalogram (video-EEG) showed intermittent generalized 5-6 Hz and 6-7 Hz theta slowing of the background activity in the stimulated state. BAER was done revealing bilateral partial dysfunction of the auditory pathways to support brainstem involvement of the disease. He received IVIG and methylprednisolone pulse therapy with no significant clinical improvement. Hence, he was given a rituximab infusion. One week post-rituximab, he had sustained wakefulness and was able to move his extremities.

14.
Clin Psychopharmacol Neurosci ; 22(2): 383-386, 2024 May 31.
Article in English | MEDLINE | ID: mdl-38627086

ABSTRACT

This study compares the changes in Quantitative electroencephalography (QEEG), loudness dependence of auditory evoked potentials (LDAEP), and mismatch negativity (MMN) in the case of bipolar depression, mania, and euthymia in a single patient. the characteristic of QEEG in this patient with mixed depression was an increase in alpha; in mixed mania, there was little increase in alpha, and the decrease in delta, theta, and beta was noticeable. LDAEP increased more in the manic phase than in the depressive phase. In contrast, MMN decreased more in the manic than in the depressive phase. After remission of mania, QEEG, LDAEP, and MMN were re-measured. Compared with the manic phase, the decrease in delta, theta, and beta bands in the occipital, temporal, and parietal lobes improved significantly. The LDAEP decreased from LDAEP 1.67 to 0.97. However, in spite of the euthymic phase, MMN amplitude showed a further decrease, from -1.7 to -0.9. In conclusion, using QEEG, LDAEP, and MMN can help clinicians predict a patient's bipolar state and evaluate serotonin intensity and cognitive function, enabling customized treatment. However, there are still few consistent research results; therefore, there is a need to utilize a larger sample size.

15.
Front Hum Neurosci ; 18: 1342931, 2024.
Article in English | MEDLINE | ID: mdl-38681742

ABSTRACT

Objectives: The auditory spatial processing abilities mature throughout childhood and degenerate in older adults. This study aimed to compare the differences in onset cortical auditory evoked potentials (CAEPs) and location-evoked acoustic change complex (ACC) responses among children, adults, and the elderly and to investigate the impact of aging and development on ACC responses. Design: One hundred and seventeen people were recruited in the study, including 57 typically-developed children, 30 adults, and 30 elderlies. The onset-CAEP evoked by white noise and ACC by sequential changes in azimuths were recorded. Latencies and amplitudes as a function of azimuths were analyzed using the analysis of variance, Pearson correlation analysis, and multiple linear regression model. Results: The ACC N1'-P2' amplitudes and latencies in adults, P1'-N1' amplitudes in children, and N1' amplitudes and latencies in the elderly were correlated with angles of shifts. The N1'-P2' and P2' amplitudes decreased in the elderly compared to adults. In Children, the ACC P1'-N1' responses gradually differentiated into the P1'-N1'-P2' complex. Multiple regression analysis showed that N1'-P2' amplitudes (R2 = 0.33) and P2' latencies (R2 = 0.18) were the two most variable predictors in adults, while in the elderly, N1' latencies (R2 = 0.26) explained most variances. Although the amplitudes of onset-CAEP differed at some angles, it could not predict angle changes as effectively as ACC responses. Conclusion: The location-evoked ACC responses varied among children, adults, and the elderly. The N1'-P2' amplitudes and P2' latencies in adults and N1' latencies in the elderly explained most variances of changes in spatial position. The differentiation of the N1' waveform was observed in children. Further research should be conducted across all age groups, along with behavioral assessments, to confirm the relationship between aging and immaturity in objective ACC responses and poorer subjective spatial performance. Significance: ACCs evoked by location changes were assessed in adults, children, and the elderly to explore the impact of aging and development on these differences.

16.
BMC Genomics ; 25(1): 391, 2024 Apr 22.
Article in English | MEDLINE | ID: mdl-38649797

ABSTRACT

Developmental delay (DD), or intellectual disability (ID) is a very large group of early onset disorders that affects 1-2% of children worldwide, which have diverse genetic causes that should be identified. Genetic studies can elucidate the pathogenesis underlying DD/ID. In this study, whole-exome sequencing (WES) was performed on 225 Chinese DD/ID children (208 cases were sequenced as proband-parent trio) who were classified into seven phenotype subgroups. The phenotype and genomic data of patients with DD/ID were further retrospectively analyzed. There were 96/225 (42.67%; 95% confidence interval [CI] 36.15-49.18%) patients were found to have causative single nucleotide variants (SNVs) and small insertions/deletions (Indels) associated with DD/ID based on WES data. The diagnostic yields among the seven subgroups ranged from 31.25 to 71.43%. Three specific clinical features, hearing loss, visual loss, and facial dysmorphism, can significantly increase the diagnostic yield of WES in patients with DD/ID (P = 0.005, P = 0.005, and P = 0.039, respectively). Of note, hearing loss (odds ratio [OR] = 1.86%; 95% CI = 1.00-3.46, P = 0.046) or abnormal brainstem auditory evoked potential (BAEP) (OR = 1.91, 95% CI = 1.02-3.50, P = 0.042) was independently associated with causative genetic variants in DD/ID children. Our findings enrich the variation spectrums of SNVs/Indels associated with DD/ID, highlight the value genetic testing for DD/ID children, stress the importance of BAEP screen in DD/ID children, and help to facilitate early diagnose, clinical management and reproductive decisions, improve therapeutic response to medical treatment.


Subject(s)
Developmental Disabilities , Exome Sequencing , Intellectual Disability , Child , Child, Preschool , Female , Humans , Infant , Male , Developmental Disabilities/genetics , Developmental Disabilities/diagnosis , East Asian People/genetics , INDEL Mutation , Intellectual Disability/genetics , Phenotype , Polymorphism, Single Nucleotide
17.
PeerJ ; 12: e17104, 2024.
Article in English | MEDLINE | ID: mdl-38680894

ABSTRACT

Advancements in cochlear implants (CIs) have led to a significant increase in bilateral CI users, especially among children. Yet, most bilateral CI users do not fully achieve the intended binaural benefit due to potential limitations in signal processing and/or surgical implant positioning. One crucial auditory cue that normal hearing (NH) listeners can benefit from is the interaural time difference (ITD), i.e., the time difference between the arrival of a sound at two ears. The ITD sensitivity is thought to be heavily relying on the effective utilization of temporal fine structure (very rapid oscillations in sound). Unfortunately, most current CIs do not transmit such true fine structure. Nevertheless, bilateral CI users have demonstrated sensitivity to ITD cues delivered through envelope or interaural pulse time differences, i.e., the time gap between the pulses delivered to the two implants. However, their ITD sensitivity is significantly poorer compared to NH individuals, and it further degrades at higher CI stimulation rates, especially when the rate exceeds 300 pulse per second. The overall purpose of this research thread is to improve spatial hearing abilities in bilateral CI users. This study aims to develop electroencephalography (EEG) paradigms that can be used with clinical settings to assess and optimize the delivery of ITD cues, which are crucial for spatial hearing in everyday life. The research objective of this article was to determine the effect of CI stimulation pulse rate on the ITD sensitivity, and to characterize the rate-dependent degradation in ITD perception using EEG measures. To develop protocols for bilateral CI studies, EEG responses were obtained from NH listeners using sinusoidal-amplitude-modulated (SAM) tones and filtered clicks with changes in either fine structure ITD (ITDFS) or envelope ITD (ITDENV). Multiple EEG responses were analyzed, which included the subcortical auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs) elicited by stimuli onset, offset, and changes. Results indicated that acoustic change complex (ACC) responses elicited by ITDENV changes were significantly smaller or absent compared to those elicited by ITDFS changes. The ACC morphologies evoked by ITDFS changes were similar to onset and offset CAEPs, although the peak latencies were longest for ACC responses and shortest for offset CAEPs. The high-frequency stimuli clearly elicited subcortical ASSRs, but smaller than those evoked by lower carrier frequency SAM tones. The 40-Hz ASSRs decreased with increasing carrier frequencies. Filtered clicks elicited larger ASSRs compared to high-frequency SAM tones, with the order being 40 > 160 > 80> 320 Hz ASSR for both stimulus types. Wavelet analysis revealed a clear interaction between detectable transient CAEPs and 40-Hz ASSRs in the time-frequency domain for SAM tones with a low carrier frequency.


Subject(s)
Cochlear Implants , Cues , Electroencephalography , Humans , Electroencephalography/methods , Acoustic Stimulation/methods , Sound Localization/physiology , Auditory Perception/physiology , Evoked Potentials, Auditory/physiology , Time Factors
18.
World Neurosurg ; 185: e1153-e1159, 2024 05.
Article in English | MEDLINE | ID: mdl-38493889

ABSTRACT

BACKGROUND: We performed this study to investigate the effect of intraoperative brainstem auditory evoked potential (IBAEP) changes on the development of postoperative nausea and vomiting (PONV) after microvascular decompression (MVD) for neurovascular cross compression. METHODS: A total of 373 consecutive cases were treated with MVD. The use of rescue antiemetics after surgery was used as an objective indicator of PONV. IBAEP monitoring was routinely performed in all. RESULTS: The use of rescue antiemetics was significantly associated with female sex (OR = 3.427; 95% CI, 2.077-5.654; P < 0.001), PCA use (OR = 3.333; 95% CI, 1.861-5.104; P < 0.001), and operation time (OR = 1.017; 95% CI, 1.008-1.026; P < 0.001). A Wave V peak delay of more than 1.0 milliseconds showed a significant relation with the use of rescue antiemetics (OR = 1.787; 95% CI, 1.114-2.867; P = 0.016) and a strong significant relation with the use of rescue antiemetics more than 5 times (OR = 2.426; 95% CI, 1.372-4.290; P = 0.002). CONCLUSIONS: A wave V peak delay of more than 1.0 milliseconds might have value as a predictor of PONV after MVD. More detailed neurophysiological studies will identify the exact pathophysiology underlying PONV after MVD.


Subject(s)
Evoked Potentials, Auditory, Brain Stem , Microvascular Decompression Surgery , Postoperative Nausea and Vomiting , Humans , Microvascular Decompression Surgery/methods , Female , Male , Middle Aged , Evoked Potentials, Auditory, Brain Stem/physiology , Postoperative Nausea and Vomiting/epidemiology , Adult , Aged , Antiemetics/therapeutic use , Intraoperative Neurophysiological Monitoring/methods , Retrospective Studies
19.
Article in English | MEDLINE | ID: mdl-38367051

ABSTRACT

The matched filter hypothesis proposes a close match between senders and receivers and is supported by several studies on variation in signal properties and sensory-processing mechanisms among species and populations. Importantly, within populations, individual variation in sensory processing may affect how receivers perceive signals. Our main goals were to characterize hearing sensitivity of Pacific treefrogs (Pseudacris regilla), assess patterns of individual variation in hearing sensitivity, and evaluate how among-individual variation in hearing sensitivity and call frequency content affect auditory processing of communication signals. Overall, males and females are most sensitive to frequencies between 2.0 and 2.5 kHz, which matches the dominant frequency of the call, and have a second region of high sensitivity between 400 and 800 Hz that does not match the fundamental frequency of the call. We found high levels of among-individual variation in hearing sensitivity, primarily driven by subject size. Importantly, patterns of among-individual variation in hearing differ between males and females. Cross-correlation analyses reveal that among-individual variation in hearing sensitivity may lead to differences on how receivers, particularly females, perceive male calls. Our results suggest that individual variation in sensory processing may affect signal perception and influence the evolution of sexually selected traits.


Subject(s)
Anura , Hearing , Animals , Male , Female , Hearing/physiology , Anura/physiology , Sex Characteristics , Vocalization, Animal/physiology , Body Size , Auditory Perception/physiology , Acoustic Stimulation , Sexual Selection , Auditory Threshold/physiology
20.
medRxiv ; 2024 Jan 26.
Article in English | MEDLINE | ID: mdl-38343802

ABSTRACT

Background: In the search for objective tools to quantify neural function in Rett Syndrome (RTT), which are crucial in the evaluation of therapeutic efficacy in clinical trials, recordings of sensory-perceptual functioning using event-related potential (ERP) approaches have emerged as potentially powerful tools. Considerable work points to highly anomalous auditory evoked potentials (AEPs) in RTT. However, an assumption of the typical signal-averaging method used to derive these measures is "stationarity" of the underlying responses - i.e. neural responses to each input are highly stereotyped. An alternate possibility is that responses to repeated stimuli are highly variable in RTT. If so, this will significantly impact the validity of assumptions about underlying neural dysfunction, and likely lead to overestimation of underlying neuropathology. To assess this possibility, analyses at the single-trial level assessing signal-to-noise ratios (SNR), inter-trial variability (ITV) and inter-trial phase coherence (ITPC) are necessary. Methods: AEPs were recorded to simple 100Hz tones from 18 RTT and 27 age-matched controls (Ages: 6-22 years). We applied standard AEP averaging, as well as measures of neuronal reliability at the single-trial level (i.e. SNR, ITV, ITPC). To separate signal-carrying components from non-neural noise sources, we also applied a denoising source separation (DSS) algorithm and then repeated the reliability measures. Results: Substantially increased ITV, lower SNRs, and reduced ITPC were observed in auditory responses of RTT participants, supporting a "neural unreliability" account. Application of the DSS technique made it clear that non-neural noise sources contribute to overestimation of the extent of processing deficits in RTT. Post-DSS, ITV measures were substantially reduced, so much so that pre-DSS ITV differences between RTT and TD populations were no longer detected. In the case of SNR and ITPC, DSS substantially improved these estimates in the RTT population, but robust differences between RTT and TD were still fully evident. Conclusions: To accurately represent the degree of neural dysfunction in RTT using the ERP technique, a consideration of response reliability at the single-trial level is highly advised. Non-neural sources of noise lead to overestimation of the degree of pathological processing in RTT, and denoising source separation techniques during signal processing substantially ameliorate this issue.

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