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Introduction Hearing impairment in neonates and infants is a critical concern due to its potential to impede language acquisition, cognitive development, and overall quality of life. Brainstem-evoked response audiometry (BERA) stands out as a valuable diagnostic tool. The early detection of hearing impairments is paramount in neonatal care. Hearing loss during infancy can impede speech and language development, social interaction, and academic achievement. High-risk neonates, including those born prematurely or with low birth weight, have a heightened susceptibility to hearing impairment due to various factors such as exposure to ototoxic medications, mechanical ventilation, and complications associated with prematurity. Methods A hospital-based prospective study was conducted in the department of otorhinolaryngology; the study focused on high-risk neonates and infants from the outpatient department and inpatient department. The study was conducted from October 2022 to March 2024. A sample size of 70 patients was taken, including high-risk neonates and infants. Healthy term neonates and healthy infants were excluded from the study. Results In the current study, there were 40 males and 30 females. Among the infants surveyed, prematurity was the most prevalent risk factor, followed by perinatal asphyxia. Low birth weight was observed in 43% of cases, while hyperbilirubinemia and neonatal sepsis were the next. Among the 70 infants assessed, 50% were found to have normal hearing. Mild hearing loss was observed in 23% of cases, while 14% had moderate hearing loss. Severe and profound hearing loss were less common. Conclusion Our study highlighted the importance of early and routine auditory screening using BERA in high-risk neonates and infants, revealing a significant prevalence of hearing loss linked to various risk factors such as premature babies, low birth weight, hyperbilirubinemia, neonatal intensive care unit stay, perinatal asphyxia, and ototoxic drugs during pregnancy. Prematurity is the most common risk factor. For language development, early diagnosis and intervention were crucial. If babies have profound sensorineural hearing loss, they can go for a cochlear implant.

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The frequency-following response (FFR) is an evoked potential that provides a neural index of complex sound encoding in the brain. FFRs have been widely used to characterize speech and music processing, experience-dependent neuroplasticity (e.g., learning and musicianship), and biomarkers for hearing and language-based disorders that distort receptive communication abilities. It is widely assumed that FFRs stem from a mixture of phase-locked neurogenic activity from the brainstem and cortical structures along the hearing neuraxis. In this study, we challenge this prevailing view by demonstrating that upwards of ~50% of the FFR can originate from an unexpected myogenic source: contamination from the postauricular muscle (PAM) vestigial startle reflex. We measured PAM, transient auditory brainstem responses (ABRs), and sustained frequency-following response (FFR) potentials reflecting myogenic (PAM) and neurogenic (ABR/FFR) responses in young, normal-hearing listeners with varying degrees of musical training. We first establish that PAM artifact is present in all ears, varies with electrode proximity to the muscle, and can be experimentally manipulated by directing listeners' eye gaze toward the ear of sound stimulation. We then show this muscular noise easily confounds auditory FFRs, spuriously amplifying responses 3-4-fold with tandem PAM contraction and even explaining putative FFR enhancements observed in highly skilled musicians. Our findings expose a new and unrecognized myogenic source to the FFR that drives its large inter-subject variability and cast doubt on whether changes in the response typically attributed to neuroplasticity/pathology are solely of brain origin.

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The present study attempted to understand the association between Auditory neuropathy spectrum disorder (ANSD) and Sickle cell anemia (SCA) and to recognize possible causative factors for the presence of ANSD in SCA individuals. Two cases, 24 years male and 17years female with a laboratory-confirmed diagnosis of Sickle cell anemia underwent detailed audiological evaluation i.e., pure tone audiometry, speech audiometry, immittance audiometry, otoacoustic emission, and auditory brainstem responses. Audiological evaluation revealed a bilateral moderate low-frequency sensorineural hearing loss in male and bilateral moderately severe sensorineural Hearing loss in female case with elevated Speech Recognition Threshold and poor Speech Identification Scores. 'A' type tympanogram with the absence of Acoustic reflexes and the presence of Otoacoustic emission with no distinct and reproducible peak V in Auditory Brainstem Response (ABR) at 90 dBnHL with the presence of ringing cochlear microphonics on polarity reversal collectively indicating bilateral ANSD in both cases. ANSD and SCA are reported to have a genetic basis of etiology. There might be possibilities that one genetic condition may be common in manifesting both conditions or one genetic condition can cause the presence of another genetic condition or can exaggerate the evolution of another genetic condition. Also, abnormal ABR findings indicate the possibility of neuropathological involvement in isolation or in combination with genetic abnormalities that need detailed investigation to understand non-genetic causative factors. Thus, paved the path for further research in this line and might provide better rehabilitative options.

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A 51-year-old man with a history of cisplatin treatment for a right testicular tumor underwent microvascular decompression for hemifacial spasm. At an early stage in the surgical procedure, the intraoperative auditory brainstem response (ABR) was diminished despite a relatively minimally invasive approach, resulting in irreversible hearing loss. Cisplatin is known to cause dose-dependent hearing impairment primarily affecting the cochlea, but it can also induce neurotoxicity. In the present case, prior cisplatin administration may have caused fragility of the cochlear nerve as well. Patients with a history of ototoxic and neurotoxic drugs such as cisplatin require more careful manipulation and thorough intraoperative auditory monitoring during neurosurgical procedures that may affect hearing, such as those for hemifacial spasms.

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Cisplatin is a widely used and highly effective anti-cancer drug with significant side effects including ototoxicity and nephrotoxicity. Macrophages, the major resident immune cells in the cochlea and kidney, are important drivers of both inflammatory and tissue repair responses. To investigate the roles of macrophages in cisplatin-induced ototoxicity and nephrotoxicity, we used PLX3397, an FDA-approved inhibitor of the colony-stimulating factor 1 receptor (CSF1R), to eliminate tissue-resident macrophages during the course of cisplatin administration. Mice treated with cisplatin alone (cisplatin/vehicle) had significant hearing loss (ototoxicity) as well as kidney injury (nephrotoxicity). Macrophage ablation using PLX3397 resulted in significantly reduced hearing loss measured by auditory brainstem responses (ABR) and distortion-product otoacoustic emissions (DPOAE). Sensory hair cells in the cochlea were protected against cisplatin-induced death in mice treated with PLX3397. Macrophage ablation also protected against cisplatin-induced nephrotoxicity, as evidenced by markedly reduced tubular injury and fibrosis as well as reduced plasma blood urea nitrogen (BUN) and neutrophil gelatinase-associated lipocalin (NGAL) levels. Mechanistically, our data suggest that the protective effect of macrophage ablation against cisplatin-induced ototoxicity and nephrotoxicity is mediated by reduced platinum accumulation in both the inner ear and the kidney. Together our data indicate that ablation of tissue-resident macrophages represents a novel strategy for mitigating cisplatin-induced ototoxicity and nephrotoxicity.

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Background: Vertigo and hearing loss are both prevalent in the elderly. This study retrospectively analyzed hearing test results from elderly patients experiencing vertigo and dizziness at ENT outpatient over a 10-year period, in order to study the patterns of hearing loss in this patient population. Methods: Nine thousand three hundred eighty four patients over 50 years old underwent retrospective collection and screening of outpatient diagnosis, pure tone audiometry, acoustic immittance measurement (tympanogram) and auditory brainstem response (ABR) test. The patient's audiograms are divided into 7 subtypes according to a set of fixed criteria. Meanwhile, K-Means clustering analysis method was used to classify the audiogram. Results: The Jerger classification of tympanogram in elderly patients with vertigo and dizziness showed the majority falling under type A. The leading audiogram shapes were flat (27.81% in right ear and 26.89% in left ear), high-frequency gently sloping (25.97% in right ear and 27.34% in left ear), and high-frequency steeply sloping (21.60% in right ear and 22.53% in left ear). Meniere's disease (MD; 30.87%), benign recurrent vertigo (BRV; 19.07%), and benign paroxysmal positional vertigo (BPPV; 15.66%) were the most common etiologies in elderly vestibular diseases. We observed statistically significant differences in hearing thresholds among these vestibular diseases (P < 0.001). K-Means clustering analysis suggested that the optimal number of clusters was three, with sample sizes for the three clusters being 2,747, 2,413, and 4,139, respectively. The ANOVA statistical results of each characteristic value showed P < 0.001. Conclusion: The elderly patients often have mild to moderate hearing loss as a concomitant symptom with vertigo. Female patients have better hearing thresholds than males. The dominant audiometric shapes in this patient population were flat, high-frequency gently sloping, and high-frequency steeply sloping according to a set of fixed criteria. This study highlights the need for tailored strategies in managing hearing loss in elderly patients with vertigo and dizziness.

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Evidence shows that females have increased supra-threshold peripheral auditory processing compared to males. This is indicated by larger auditory brainstem responses (ABR) wave I amplitude, which measures afferent spiral ganglion neuron (SGN)-auditory nerve synchrony. However, the underlying molecular mechanisms of this sex difference are mostly unknown. We sought to elucidate sex differences in ABR wave I amplitude by examining molecular markers known to affect synaptic transmission kinetics. Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors (AMPARs) mediate fast excitatory transmission in mature SGN afferent synapses. Each AMPAR channel is a tetramer composed of GluA2, 3, and 4 subunits (Gria2, 3, and 4 genes), and those lacking GluA2 subunits have larger currents, are calcium-permeable, and have faster gating kinetics. Moreover, alternatively spliced flip and flop isoforms of each AMPAR subunit affect channel kinetics, having faster kinetics those AMPARs containing Gria3 and Gria4 flop isoforms. We hypothesized that SGNs of females have more fast-gating AMPAR subunit mRNA than males, which could contribute to more temporally precise synaptic transmission and increased SGN synchrony. Our data show that the index of Gria3 relative to Gria2 transcripts on SGN was higher in females than males (females: 48%; males: 43%), suggesting that females have more SGNs with higher Gria3 mRNA relative to Gria2. Analysis of the relative abundance of the flip and flop alternatively spliced isoforms showed that females have a 2-fold increase in fast-gating Gria3 flop mRNA, while males have more slow-gating (2.5-fold) of the flip. We propose that Gria3 may in part mediate greater SGN synchrony in females. Significance Statement: Females of multiple vertebrate species, including fish and mammals, have been reported to have enhanced sound-evoked synchrony of afferents in the auditory nerve. However, the underlying molecular mediators of this physiologic sex difference are unknown. Elucidating potential molecular mechanisms related to sex differences in auditory processing is important for maintaining healthy ears and developing potential treatments for hearing loss in both sexes. This study found that females have a 2-fold increase in Gria3 flop mRNA, a fast-gating AMPA-type glutamate receptor subunit. This difference may contribute to greater neural synchrony in the auditory nerve of female mice compared to males, and this sex difference may be conserved in all vertebrates.

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BACKGROUND: Obesity is an independent risk factor for hearing loss. Although attention has focused on major obesity comorbidities such as cardiovascular disease, stroke, and type 2 diabetes, the impact of obesity on sensorineural organs, including the auditory system, is unclear. Using a high-fat diet (HFD)-induced obese mouse model, we investigated the impact of diet-induced obesity on sexual dimorphism in metabolic alterations and hearing sensitivity. METHODS: Male and female CBA/Ca mice were randomly assigned to three diet groups and fed, from weaning (at 28 days) to 14 weeks of age, a sucrose-matched control diet (10 kcal% fat content diet), or one of two HFDs (45 or 60 kcal% fat content diets). Auditory sensitivity was evaluated based on the auditory brainstem response (ABR), distortion product otoacoustic emission (DPOAE), and ABR wave 1 amplitude at 14 weeks of age, followed by biochemical analyses. RESULTS: We found significant sexual dimorphism in HFD-induced metabolic alterations and obesity-related hearing loss. Male mice exhibited greater weight gain, hyperglycemia, increased ABR thresholds at low frequencies, elevated DPOAE, and lower ABR wave 1 amplitude compared to female mice. The hair cell (HC) ribbon synapse (CtBP2) puncta showed significant sex differences. The serum concentration of adiponectin, an otoprotective adipokine, was significantly higher in female than in male mice; cochlear adiponectin levels were elevated by HFD in female but not male mice. Adiponectin receptor 1 (AdipoR1) was widely expressed in the inner ear, and cochlear AdipoR1 protein levels were increased by HFD, in female but not male mice. Stress granules (G3BP1) were significantly induced by the HFD in both sexes; conversely, inflammatory (IL-1ß) responses were observed only in the male liver and cochlea, consistent with phenotype HFD-induced obesity. CONCLUSIONS: Female mice are more resistant to the negative effects of an HFD on body weight, metabolism, and hearing. Females showed increased peripheral and intra-cochlear adiponectin and AdipoR1 levels, and HC ribbon synapses. These changes may mediate resistance to HFD-induced hearing loss seen in female mice.

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Auditory evoked potentials (AEPs) are brain-derived electrical signals, following an auditory stimulus, utilised to examine any obstructions along the brain neural-pathways and to diagnose hearing impairment. The clinical evaluation of AEPs is based on the measurements of the latencies and amplitudes of waves of interest; hence, their identification is a prerequisite for AEP analysis. This process has proven to be complex, as it requires relevant clinical experience, and the existing software for this purpose has little practical use. The aim of this study was the development of two automated annotation tools for ABR (auditory brainstem response)- and AMLR (auditory middle latency response)-tests. After the acquisition of 1046 raw waveforms, appropriate pre-processing and implementation of a four-stage development process were performed, to define the appropriate logical conditions and steps for each algorithm. The tools' detection and annotation results, regarding the waves of interest, were then compared to the clinicians' manual annotation, achieving match rates of at least 93.86%, 98.51%, and 91.51% respectively, for the three ABR-waves of interest, and 93.21%, 92.25%, 83.35%, and 79.27%, respectively, for the four AMLR-waves. The application of such tools in AEP analysis is expected to assist towards an easier interpretation of these signals.

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The primary aim of this study was to investigate whether auditory brainstem response (ABR) and speech perception in noise (SPiN) were associated with occupational noise exposure in normal hearing young factory workers. Forty young adults occupationally exposed to noise and 40 non-exposed young adults (control group) from Zhejiang province in China were selected. All participants presented with normal hearing thresholds and distortion product otoacoustic emissions. Participants were evaluated with the Mandarin Bamford-Kowal-Bench (BKB) test and ABR. The latter was obtained for click stimulus at 50, 60, 70, 80, and 90 dBnHL. Peak-to-trough amplitudes and latencies for waves I and V were obtained. The ABR wave I amplitude, the wave I/V amplitude ratio, the slope of the wave I amplitude growth as a function of stimulus intensity (AMP-ISlope), and the wave V latency shift with ipsilateral noise (LAT-VSlope) were used as ABR outcomes. Finally, equivalent continuous average sound pressure level normalized to 8 h (LAeq.8h) and cumulative noise exposure (CNE) were obtained for noise-exposed participants. No significant differences between groups were found for any ABR outcomes. Noise-exposed participants exhibited worse BKB scores than control group participants. A multivariate regression model showed that 23.3% of the variance in BKB scores was explained by group category (exposed vs. non-exposed) and hearing thresholds. However, since none of the ABR outcomes exploring cochlear synaptopathy were associated with noise exposure, we cannot conclude that cochlear synaptopathy was the contributing factor for the differences between groups for BKB scores. Factors that go beyond sensory processing may explain such results, especially given socio-economic differences between the noise-exposed and control groups. We conclude that in this sample of participants, occupational noise exposure was not associated with signs of cochlear synaptopathy as measured by ABR and BKB.

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Animal studies have shown that noise exposure and aging cause a reduction in the number of synapses between low and medium spontaneous rate auditory nerve fibers and inner hair cells before outer hair cell deterioration. This noise-induced and age-related cochlear synaptopathy (CS) is hypothesized to compromise speech recognition at moderate-to-high suprathreshold levels in humans. This paper evaluates the evidence on the relative and combined effects of noise exposure and aging on CS, in both animals and humans, using histopathological and proxy measures. In animal studies, noise exposure seems to result in a higher proportion of CS (up to 70% synapse loss) compared to aging (up to 48% synapse loss). Following noise exposure, older animals, depending on their species, seem to either exhibit significant or little further synapse loss compared to their younger counterparts. In humans, temporal bone studies suggest a possible age- and noise-related auditory nerve fiber loss. Based on the animal data obtained from different species, we predict that noise exposure may accelerate age-related CS to at least some extent in humans. In animals, noise-induced and age-related CS in separation have been consistently associated with a decreased amplitude of wave 1 of the auditory brainstem response, reduced middle ear muscle reflex strength, and degraded temporal processing as demonstrated by lower amplitudes of the envelope following response. In humans, the individual effects of noise exposure and aging do not seem to translate clearly into deficits in electrophysiological, middle ear muscle reflex, and behavioral measures of CS. Moreover, the evidence on the combined effects of noise exposure and aging on peripheral neural deafferentation in humans using electrophysiological and behavioral measures is even more sparse and inconclusive. Further research is necessary to establish the individual and combined effects of CS in humans using temporal bone, objective, and behavioral measures.

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Age-related hearing loss (ARHL, formerly presbycusis) is due to a variety of lifetime damages to the auditory system and is characterized by bilateral sensorineural hearing loss, impaired speech understanding in noise and central sound processing deficits. Despite its commonness, the pathogenesis has not been completely clarified yet; especially the existence of an independent central ARHL component still remains controversial. We present the results of a cross-sectional topodiagnostic test battery study which aimed at separating aging- and hearing loss-related effects on all parts of the auditory system by current test procedures. Three groups of 30 participants each underwent extensive topodiagnostic test procedures (otoscopy, tympanometry, questionnaires, pure-tone audiometry, DPOAE threshold measurements, auditory brainstem response, central auditory discrimination tests, and speech-in-noise test). By comparing the results of the normally hearing young (18-26 years) and healthy control group, the normally hearing elderly group (60-80 years) and the hearing-impaired elderly group (60-80 years), we deduced aging and hearing loss-related effects on auditory performance. All measurements indicated a significant deterioration of auditory performance in the elderly, partly associated with aging and partly with age-related hearing loss. Our study thereby contributes to a multifocal concept of ARHL. All parts of the auditory system are impaired by aging, age-related hearing loss, or a combination of both. Further evidence for an independent central ARHL component, not attributable to peripheral hearing loss, is provided by the results of the central auditory discrimination test.

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Functional hair cell regeneration in the adult mammalian inner ear remains challenging. This study aimed to study the function of new hair cells induced by a DNA demethylating agent 5-azacytidine. Adult mice were deafened chemically, followed by injection of 5-azacytidine or vehicle into the inner ear. Functionality of regenerated hair cells was evaluated by expression of hair cell proteins, auditory brainstem response (ABR), and distortion-product otoacoustic emission (DPOAE) tests for 6 weeks. In the vehicle-treated group, no cells expressed the hair cell-specific protein myosin VIIa in the cochlea, whereas numerous myosin VIIa-expressing cells were found in the 5-azacytidine-treated cochlea, suggesting the regeneration of auditory hair cells. Moreover, regenerated hair cells were co-labeled with functional proteins espin and prestin. Expression of ribbon synapse proteins suggested synapse formation between new hair cells and neurons. In hearing tests, progressive improvements in ABR [5-30 dB sound pressure level (SPL)] and DPOAE (5-20 dB) thresholds were observed in 5-azacytidine-treated mice. In vehicle-treated mice, there were <5 dB threshold changes in hearing tests. This study demonstrated the ability of 5-azacytidine to promote the functional regeneration of auditory hair cells in a mature mouse model via DNA demethylation, which may provide insights into hearing regeneration using an epigenetic approach.

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OBJECTIVE: Little is known about peripheral auditory function in young adults with HIV, who might be expected to show early evidence of hearing loss if HIV infection or treatment does affect peripheral function. The goal of this study was to compare peripheral auditory function in 2 age- and gender-matched groups of young adults with clinically normal hearing with and without HIV. STUDY DESIGN: Matched cohort study with repeated measures. SETTING: Infectious disease center in Dar es Salaam, Tanzania. METHODS: Participants included HIV-positive (n = 38) and HIV-negative (n = 38) adults aged 20 to 30 years who had clinically normal hearing, defined as type A tympanograms, air conduction thresholds ≤25 dB HL bilaterally from 0.5 to 8 kHz, and distortion product otoacoustic emissions (DPOAEs) >6 dB above the noise floor bilaterally from 1.5 to 8 kHz. Participants were tested multiple times over 6-month intervals (average, 2.7 sessions/participant) for a total of 208 observations. Primary outcome measures included tympanograms, air conduction audiograms, DPOAEs, and click-evoked auditory brainstem responses. RESULTS: HIV groups did not significantly differ in age, static immittance, or air conduction thresholds. HIV-positive status was independently associated with approximately 3.7-dB lower DPOAE amplitudes from 2 to 8 kHz (95% CI, 1.01-6.82) in both ears and 0.04-µV lower (95% CI, 0.003-0.076) auditory brainstem response wave I amplitudes in the right ear. CONCLUSION: Young adults living with HIV have slightly but reliably smaller DPOAEs and auditory brainstem response wave I amplitudes than matched HIV-negative controls. The magnitude of these differences is small, but these results support measuring peripheral auditory function in HIV-positive individuals as they age.

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In India, newborn hearing screening programs have been implemented as a part of research studies since early 1970s. Amongst the previously reported programs most are from the southern region and very few are from the west and the northern region of the country. There is a lack of evidence of such program in other region of the country. 1. To study the outcome, experience, and challenges faced during the implementation of the universal newborn hearing screening program in a medical college set up of Raipur, Chhattisgarh. 2. To determine the prevalence of hearing impairment with a two tier screening protocol with Otoacoustic emission and Auditory Brainstem Response. The Prospective Non randomised study was carried out between December 2017 and December 2019. A total of 1200 neonates delivered at the medical college, Raipur were screened using the two tier screening protocol. In our study, the prevalence of hearing loss was 2 per 1000 live births for bilateral hearing loss and 1 per 1000 live births for unilateral hearing loss. Implementing universal newborn screening in a vast country like India is a challenging task because of a high birth rate, diverse socio-economic and cultural background with limited resources. Though several hospitals and clinics have implemented the UNHS program, yet there is a dearth of literature regarding the program outcome, success, challenges, and lessons learnt. Therefore best practices of such evolved programs should be in public domain.

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In nonlinear systems, the inclusion of low-level noise can paradoxically improve signal detection, a phenomenon known as stochastic resonance (SR). SR has been observed in human hearing whereby sensory thresholds (e.g., signal detection and discrimination) are enhanced in the presence of noise. Here, we asked whether subcortical auditory processing (neural phase locking) shows evidence of SR. We recorded brainstem frequency-following-responses (FFRs) in young, normal-hearing listeners to near-electrophysiological-threshold (40 dB SPL) complex tones composed of 10 iso-amplitude harmonics of 150 Hz fundamental frequency (F0) presented concurrent with low-level noise (+20 to -20 dB SNRs). Though variable and weak across ears, some listeners showed improvement in auditory detection thresholds with subthreshold noise confirming SR psychophysically. At the neural level, low-level FFRs were initially eradicated by noise (expected masking effect) but were surprisingly reinvigorated at select masker levels (local maximum near âˆ¼ 35 dB SPL). These data suggest brainstem phase-locking to near threshold periodic stimuli is enhanced in optimal levels of noise, the hallmark of SR. Our findings provide novel evidence for stochastic resonance in the human auditory brainstem and suggest that under some circumstances, noise can actually benefit both the behavioral and neural encoding of complex sounds.

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The guinea pig is a commonly-used animal model in hearing research, as their audible frequency range is similar to that of humans, and they possess comparatively large cochleae among rodents. Numerous studies have investigated the ototoxic effects of cisplatin in guinea pigs, but these have been mostly limited to single high-dose bolus injections of cisplatin. This method of drug administration is not consistent with human treatment schedules, and therefore lacks translational value to clinical applications. We tested several different cisplatin dosing schedules in guinea pigs based on common research based and clinical regimens, measuring the resulting hearing loss and morbidity (weight loss). We propose a dosing paradigm of once-weekly 4 mg/kg cisplatin injections for three weeks to best mimic clinical treatment schedules. This method resulted in a configuration of hearing loss similar to what is observed in humans along with minimal changes in weight.

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Scalp-recorded frequency-following responses (FFRs) reflect a mixture of phase-locked activity across the auditory pathway. FFRs have been widely used as a neural barometer of complex listening skills, especially speech-in noise (SIN) perception. Applying individually optimized source reconstruction to speech-FFRs recorded via EEG (FFREEG), we assessed the relative contributions of subcortical [auditory nerve (AN), brainstem/midbrain (BS)] and cortical [bilateral primary auditory cortex, PAC] source generators with the aim of identifying which source(s) drive the brain-behavior relation between FFRs and SIN listening skills. We found FFR strength declined precipitously from AN to PAC, consistent with diminishing phase-locking along the ascending auditory neuroaxis. FFRs to the speech fundamental (F0) were robust to noise across sources, but were largest in subcortical sources (BS > AN > PAC). PAC FFRs were only weakly observed above the noise floor and only at the low pitch of speech (F0≈100 Hz). Brain-behavior regressions revealed (i) AN and BS FFRs were sufficient to describe listeners' QuickSIN scores and (ii) contrary to neuromagnetic (MEG) FFRs, neither left nor right PAC FFREEG related to SIN performance. Our findings suggest subcortical sources not only dominate the electrical FFR but also the link between speech-FFRs and SIN processing in normal-hearing adults as observed in previous EEG studies.

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Sensory hypersensitivity, especially in the auditory system, is a common symptom in Fragile X syndrome (FXS), the most common monogenic form of intellectual disability. However, linking phenotypes across genetic background strains of mouse models has been a challenge and could underly some of the issues with translatability of drug studies to the human condition. This study is the first to characterize the auditory brain stem response (ABR), a minimally invasive physiological readout of early auditory processing that is also used in humans, in a commonly used mouse background strain model of FXS, C57BL/6J. We measured morphological features of pinna and head and used ABR to measure the hearing range, and monaural and binaural auditory responses in hemizygous males, homozygous females, and heterozygous females compared with those in wild-type mice. Consistent with previous study, we showed no difference in morphological parameters across genotypes or sexes. There was no significant difference in hearing range between the sexes or genotypes, however there was a trend towards high frequency hearing loss in male FXS mice. In contrast, female mice with homozygous FXS had a decreased amplitude of wave IV of the monaural ABR, while there was no difference in males for amplitudes and no change in latency of ABR waveforms across sexes and genotypes. Finally, males with FXS had an increased latency of the binaural interaction component (BIC) at 0 interaural timing difference compared with that in wild-type males. These findings further clarify auditory brain stem processing in FXS by adding more information across genetic background strains allowing for a better understanding of shared phenotypes.

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OBJECTIVES: To explore the relationship between the frequency characteristics and response threshold of auditory steady-state response (ASSR), auditory brainstem response (ABR) and 40 Hz auditory event related potential (40 Hz AERP), and their application values in forensic medicine. METHODS: Thirty volunteers with normal hearing (60 ears) were selected to perform pure tone audiometry (PTA) threshold and ASSR, ABR and 40 Hz AERP response threshold tests in the standard sound insulation shielding room, and the results were statistically analyzed by SPSS 22.0 software. RESULTS: At 0.5 kHz and 1.0 kHz frequencies, the correlation between 40 Hz AERP response threshold and PTA threshold was good, which was better than that of ASSR and ABR response threshold. At 2.0 kHz and 4.0 kHz frequencies, the correlation between ASSR and ABR response thresholds and PTA threshold was good, which was better than that of 40 Hz AERP response threshold. CONCLUSIONS: To evaluate the hearing at 0.5 kHz and 1.0 kHz frequencies, it is recommended to use 40 Hz AERP and ASSR to comprehensively assess the PTA threshold of the subjects. To evaluate the hearing at 2.0 kHz and 4.0 kHz frequencies, ABR and ASSR are recommended to assess the PTA threshold of subjects comprehensively. The combination of ASSR, ABR and 40 Hz AERP can improve the accuracy of hearing function evaluation.

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