Search Results (12)

COVID-19 is an infectious disease caused by the SARS-CoV-2 virus. During and after COVID-19, audiovestibular symptoms and impairments have been reported. Objectives: This study aimed to investigate the impacts of COVID-19 on the peripheral and central auditory systems of children and adolescents following the acute COVID-19 phase based on behavioral, electroacoustic, and electrophysiological audiological assessments. Methods: This is a primary, prospective, observational, and cross-sectional study of 23 children aged 8 to 15 years who acquired confirmed COVID-19 and who, before infection, had not had any auditory complaints or school complications. The results were compared with pre-pandemic data collected from a similar group of 23 children who had normal peripheral and central hearing and good school performance. Each participant answered a questionnaire about child development, school, and health history and underwent tests including pure-tone audiometry and high-frequency audiometry, imitanciometry, transient evoked otoacoustic emissions, and distortion product otoacoustic emissions. They also received tests of Brainstem Auditory Evoked Potentials, Long Latency Auditory Evoked Potentials, Dichotic Digits Test, Sentence Identification Test, Dichotic Consonant–Vowel Test, Frequency Pattern Test, and Gaps-In-Noise Test. Results: Significant differences were observed between the groups, with the study group showing worse thresholds compared to the control group at both standard audiometric frequencies and at higher frequencies, although both groups were still within normal limits (p ≤ 0.05). In addition, the study group had a higher prevalence of absent responses, as identified by otoacoustic emissions and acoustic reflexes. In terms of central auditory performance, the study group showed ABRs with significantly longer latencies of waves I, III, and V compared to the control group. The study group also performed less well on the Dichotic Digits and Pediatric Speech Identification tests. Conclusions: COVID-19 appears to alter the auditory system, both peripherally at the level of the outer hair cells and more centrally. Full article

Background: Many studies have demonstrated the benefits of long-term music training (i.e., musicianship) on the neural processing of sound, including simple tones and speech. However, the effects of musicianship on the encoding of simultaneously presented pitches, in the form of complex musical chords, is less well established. Presumably, musicians’ stronger familiarity and active experience with tonal music might enhance harmonic pitch representations, perhaps in an attention-dependent manner. Additionally, attention might influence chordal encoding differently across the auditory system. To this end, we explored the effects of long-term music training and attention on the processing of musical chords at the brainstem and cortical levels. Method: Young adult participants were separated into musician and nonmusician groups based on the extent of formal music training. While recording EEG, listeners heard isolated musical triads that differed only in the chordal third: major, minor, and detuned (4% sharper third from major). Participants were asked to correctly identify chords via key press during active stimulus blocks and watched a silent movie during passive blocks. We logged behavioral identification accuracy and reaction times and calculated information transfer based on the behavioral chord confusion patterns. EEG data were analyzed separately to distinguish between cortical (event-related potential, ERP) and subcortical (frequency-following response, FFR) evoked responses. Results: We found musicians were (expectedly) more accurate, though not faster, than nonmusicians in chordal identification. For subcortical FFRs, responses showed stimulus chord effects but no group differences. However, for cortical ERPs, whereas musicians displayed P2 (~150 ms) responses that were invariant to attention, nonmusicians displayed reduced P2 during passive listening. Listeners’ degree of behavioral information transfer (i.e., success in distinguishing chords) was also better in musicians and correlated with their neural differentiation of chords in the ERPs (but not high-frequency FFRs). Conclusions: Our preliminary results suggest long-term music training strengthens even the passive cortical processing of musical sounds, supporting more automated brain processing of musical chords with less reliance on attention. Our results also suggest that the degree to which listeners can behaviorally distinguish chordal triads is directly related to their neural specificity to musical sounds primarily at cortical rather than subcortical levels. FFR attention effects were likely not observed due to the use of high-frequency stimuli (>220 Hz), which restrict FFRs to brainstem sources. Full article

Background: Auditory neuropathy (AN) is a hearing disorder that affects neural activity in the VIIIth cranial nerve and central auditory pathways. Progressive forms have been reported in a number of neurodegenerative diseases and may occur as a result of both the deafferentiation and desynchronisation of neuronal processes. The purpose of this study was to describe changes in auditory function over time in a patient with axonal neuropathy and to explore the effect of auditory intervention. Methods: We tracked auditory function in a child with progressive AN associated with Charcot–Marie–Tooth (Type 2C) disease, evaluating hearing levels, auditory-evoked potentials, and perceptual abilities over a 3-year period. Furthermore, we explored the effect of auditory intervention on everyday listening and neuroplastic development. Results: While sound detection thresholds remained constant throughout, both electrophysiologic and behavioural evidence suggested auditory neural degeneration over the course of the study. Auditory brainstem response amplitudes were reduced, and perception of auditory timing cues worsened over time. Functional hearing ability (speech perception in noise) also deteriorated through the first 1.5 years of study until the child was fitted with a “remote-microphone” listening device, which subsequently improved binaural processing and restored speech perception ability to normal levels. Conclusions: Despite the deterioration of auditory neural function consistent with peripheral axonopathy, sustained experience with the remote-microphone listening system appeared to produce neuroplastic changes, which improved the patient’s everyday listening ability—even when not wearing the device. Full article

Background: By using outcome prediction scores, it is possible to distinguish between good and poor performers with cochlear implants (CI) after CI implantation. The reasons for poor performance, despite good basic conditions, can be manifold. On the one hand, the postoperative fitting may be inadequate; on the other, neurophysiological disease processes may impair speech understanding with a CI. These disease processes are not yet fully understood. In acoustics, it is known that the auditory brainstem responses (ABR) and their latencies and amplitudes allow differential diagnosis based on reference values for normal-hearing individuals. The aim of this study was to provide reference values for electrically evoked brainstem responses (EABRs) in terms of rate-dependent latencies and amplitudes. Methods: 20 ears of 18 experienced adult CI recipients with a predicted and measured good postoperative word recognition score were recruited from the clinic’s patient pool. In the same stimulation mode and intensity we measured latencies and interpeak-latencies of EABRs and electrically evoked compound action potentials (ECAPs). With a defined supra-threshold stimulation intensity above the individual ECAP threshold, we applied stimulation at several rates between 11 and 91 stimuli per second. Results: We found rate dependences for EABR latency t3 and t5 in the order of 0.19 ms and 0.37 ms, respectively, while ECAP was not affected by rate. Correspondingly, the interpeak intervals’ rate dependences for $\mathrm{t}5-\mathrm{t}1$, $\mathrm{t}5-\mathrm{t}3$ and $\mathrm{t}3-\mathrm{t}1$ were of the order of 0.37 ms, 0.18 ms and 0.19 ms. Comparing the EABR amplitudes between the stimulation rates 11/s and 81/s, we found that at 81/s the amplitudes were significantly reduced down: to 73% for A3 and 81% for A5. These rate dependences of latency and amplitude in EABR have characteristics comparable to those of acoustic ABR. Conclusions: These data may serve to provide reference values for EABR and ECAP latencies, interpeak intervals and amplitudes with respect to stimulation rate. Altered response patterns of ECAPs and EABRs to normalised stimulation modes could be used in the future to describe and classify neuropathological processes in a better-differentiated way. Full article

Wolfram syndrome (WS) is a rare neurodegenerative disease usually of autosomal recessive origin. There is limited research about sensorineural hearing loss, despite it being a fundamental entity. It is important to broaden the study of this disease and specify a set of tests required for an adequate assessment of patients for efficient monitoring and control. The fundamental objective of this research is to understand WS from a biomedical perspective in order to help in its diagnosis, follow-up, and control. Pure tones audiometry, tympanometry, speech perception, the speech intelligibility index without aid, and testing at high frequencies were among the audiological measurements utilised since they were deemed suitable for standardised follow-up. Mixed linear models were used to examine the effects of age, time, or mean interaction in pure-tone (IPT), the average of high frequencies (HFA), auditory brainstem response (ABR), and brainstem auditory evoked potentials (BAEP). The genetic analysis allowed mutations to be classified into three phenotype-genotype groups, where the phenotype indicated the severity of the hearing loss. Patients with homozygous gene changes had a more severe neurosensory phenotype. The early discovery of sensorineural hearing loss and WS is crucial since it allows intensive follow-up and treatment of the person affected from the start. Full article

Recent studies involving guinea pigs have shown that noise can damage the synapses between the inner hair cells and spiral ganglion neurons, even with normal hearing thresholds—which makes it important to investigate this kind of impairment in humans. The aim was to investigate, with multiple audiological assessments, the auditory function of normal hearing workers exposed to occupational noise. Altogether, 60 workers were assessed (30 in the noise-exposure group [NEG], who were exposed to occupational noise, and 30 in the control group [CG], who were not exposed to occupational noise); the workers were matched according to age. The following procedures were used: complete audiological assessment; speech recognition threshold in noise (SRTN); speech in noise (SN) in an acoustic field; gaps-in-noise (GIN); transient evoked otoacoustic emissions (TEOAE) and inhibitory effect of the efferent auditory pathway; auditory brainstem response (ABR); and long-latency auditory evoked potentials (LLAEP). No significant difference was found between the groups in SRTN. In SN, the NEG performed worse than the CG in signal-to-noise ratio (SNR) 0 (p-value 0.023). In GIN, the NEG had a significantly lower percentage of correct answers (p-value 0.042). In TEOAE, the NEG had smaller amplitude values bilaterally (RE p-value 0.048; LE p-value 0.045) and a smaller inhibitory effect of the efferent pathway (p-value 0.009). In ABR, the NEG had greater latencies of wave V (p-value 0.017) and interpeak intervals III-V and I-V in the LE (respective p-values: 0.005 and 0.04). In LLAEP, the NEG had a smaller P3 amplitude bilaterally (RE p-value 0.001; LE p-value 0.002). The NEG performed worse than the CG in most of the assessments, suggesting that the auditory function in individuals exposed to occupational noise is impaired, even with normal audiometric thresholds. Full article

Background and objectives: Otoferlin is a multi-C2 domain protein implicated in neurotransmitter-containing vesicle release and replenishment of the cochlear inner hair cell (IHC) synapses. Mutations in the OTOF gene have been associated with two different clinical phenotypes: a prelingual severe-to-profound sensorineural hearing loss (ANSD-DFNB9); and the peculiar temperature-sensitive auditory neuropathy (TS-ANSD), characterized by a baseline mild-to-moderate hearing threshold that worsens to severe-to-profound when the body temperature rises that returns to a baseline a few hours after the temperature has fallen again. The latter clinical phenotype has been described only with a few OTOF variants with an autosomal recessive biallelic pattern of inheritance. Case report: A 7-year-old boy presented a picture compatible with TS-ANSD exacerbated by febrile states or physical exercise with mild-to-moderate hearing loss at low and medium frequencies and a decrease in speech discrimination that worsened with an unfavorable speech-to-noise ratio. Otoacoustic emissions (OAEs) were present whereas auditory brainstem responses (ABRs) evoked by a click or tone-burst were generally absent. No inner ear malformations were described from the CT scan or MRI. Next-generation sequencing (NGS) of the known deafness genes and multi-phasic bioinformatic analyses of the data detected in OTOF a c.2521G>A missense variant and the deletion of 7.4 Kb, which was confirmed by array-comparative genomic hybridization (array-CGH). The proband’s parents, who were asymptomatic, were tested by Sanger sequencing and the father presented the c.2521G>A missense variant. Conclusions: The picture presented by the patient was compatible with OTOF-induced TS-ANSD. OTOF has been generally associated with an autosomal recessive biallelic pattern of inheritance; in this clinical report, two pathogenic variants never previously associated with TS-ANSD were described. Full article

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids. Full article

Speech evoked auditory brainstem responses depicts the neural encoding of speech at the level of brainstem. This study was designed to evaluate the neural encoding of speech at the brainstem in younger population and middle-aged population at three different repetition rates (6.9, 10.9 and 15.4). Speech evoked auditory brainstem response was recorded from 84 participants (young participants=42, middle aged participants=42) with normal hearing sensitivity. The latency of wave V and amplitude of the fundamental frequency, first formant frequency and second formant frequency was calculated. Results showed that the latency of wave V was prolonged for middle-aged individuals for all three-repetition rates compared to the younger participants. The results of the present study also revealed that there was no difference in encoding of fundamental frequency between middle aged and younger individuals at any of the repetition rates. However, increase in repetition rate did affect the encoding of the fundamental frequency in middle-aged individuals. The above results suggest a differential effect of repetition rate on wave V latency and encoding of fundamental frequency. Further, it was noticed that repetition rate did not affect the amplitude of first formant frequency or second formant frequency in middle aged participants compared to the younger participants. Full article

The following objectives of the study were formulated: i) to investigate differences in measured signal to noise ratios while recording speech-evoked auditory brainstem response (cABR) and cortical late latency response (LLR) in low and high acceptable noise level (ANL) groups; and ii) to compare peak to peak amplitude of cABR (V-A) and LLR (N1-P2) in low and high ANL groups. A total of 23 normal hearing participants was included in the study. One shot replicative and partly exploratory research design was utilized to study the effect of signal to noise ratio in a recorded waveform on afferent mechanism, assessed by cABR and LLR on participants having values of ANL of ≤7 (low ANL group) and ≥13 (high ANL group). There were no differences in signal to noise ratio in the recorded waveforms of cABR and LLR between low and high ANL groups at both brainstem and cortical levels. However, the peak to peak amplitude of V-A of cABR and N1-P2 of LLR were both statistically larger in the high ANL group compared to their counterpart. The signal to noise ratio in recorded waveforms did not differentiated cABR (V-A) or LLR (N1-P2) in low and high ANL groups. However, Larger peak to peak amplitudes in the high ANL group suggests differences higher processing centers in the upper brainstem to the auditory cortex. The findings of the study may be useful in determining the patient acceptability of noise. Full article

The aim of the present study was to investigate the effect of stimulus polarity on speech evoked auditory brainstem response (ABR). In order to accomplish it, speech evoked ABR was recorded with various stimulus polarities from 17 normally hearing adults. The result of the study shows differential effect of stimulus polarity on components of speech evoked ABR. Latency of peaks for onset, sustained and offset responses of speech evoked ABR were found to be not significantly different across stimulus polarities. In contrast, the amplitude of first formant and high frequency components was found to be significantly reduced for alternating polarity compared to single polarity, while amplitude of fundamental frequency response was not affected by polarity of the stimuli. Thus speech evoked ABR may be recorded using single polarity rather than using alternating polarities. Full article

Speech auditory brainstem responses (speech ABR) reflect activity that is phase-locked to the harmonics of the fundamental frequency (F0) up to at least the first formant (F1). Recent evidence suggests that responses at F0 in the presence of noise are more robust than responses at F1, and are also dissociated in some learning-impaired children. Peripheral auditory processing can be broadly divided into resolved and unresolved harmonic regions. This study investigates the contribution of these two regions to the speech ABR, and their susceptibility to noise. We recorded, in quiet and in background white noise, evoked responses in twelve normal hearing adults in response to three variants of a synthetic vowel: i) Allformants, which contains all first three formants, ii) F1Only, which is dominated by resolved harmonics, and iii) F2&F3Only, which is dominated by unresolved harmonics. There were no statistically significant differences in the response at F0 due to the three variants of the stimulus in quiet, nor did the noise affect this response with the Allformants and F1Only variants. On the other hand, the response at F0 with the F2&F3Only variant was significantly weaker in noise than with the two other variants (p<0.001). With the response at F1, there was no difference with the Allformants and F1Only variants in quiet, but was expectedly weaker with the F2&F3Only variant (p<0.01). The addition of noise significantly weakened the response at F1 with the F1Only variant (p<0.05), but this weakening only tended towards significance with the Allformants variant (p=0.07). The results of this study indicate that resolved and unresolved harmonics are processed in different but interacting pathways that converge in the upper brainstem. The results also support earlier work on the differential susceptibility of responses at F0 and F1 to added noise. Full article