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IEEE Transactions on Bio-medical... Jun 2024In cochlear implant users with residual acoustic hearing, compound action potentials (CAPs) can be evoked by acoustic (aCAP) or electric (eCAP) stimulation and recorded...
OBJECTIVE
In cochlear implant users with residual acoustic hearing, compound action potentials (CAPs) can be evoked by acoustic (aCAP) or electric (eCAP) stimulation and recorded through the electrodes of the implant. We propose a novel computational model to simulate aCAPs and eCAPs in humans, considering the interaction between combined electric-acoustic stimulation that occurs in the auditory nerve.
METHODS
The model consists of three components: a 3D finite element method model of an implanted cochlea, a phenomenological single-neuron spiking model for electric-acoustic stimulation, and a physiological multi-compartment neuron model to simulate the individual nerve fiber contributions to the CAP.
RESULTS
The CAP morphologies closely resembled those known from humans. The spread of excitation derived from eCAPs by varying the recording electrode along the cochlear implant electrode array was consistent with published human data. The predicted CAP amplitude growth functions largely resembled human data, with deviations in absolute CAP amplitudes for acoustic stimulation. The model reproduced the suppression of eCAPs by simultaneously presented acoustic tone bursts for different masker frequencies and probe stimulation electrodes.
CONCLUSION
The proposed model can simulate CAP responses to electric, acoustic, or combined electric-acoustic stimulation. It considers the dependence on stimulation and recording sites in the cochlea, as well as the interaction between electric and acoustic stimulation in the auditory nerve.
SIGNIFICANCE
The model enhances comprehension of CAPs and peripheral electric-acoustic interaction. It can be used in the future to investigate objective methods, such as hearing threshold assessment or estimation of neural health through aCAPs or eCAPs.
PubMed: 38843064
DOI: 10.1109/TBME.2024.3410686 -
Brain and Behavior Jun 2024This study aims to control all hearing thresholds, including extended high frequencies (EHFs), presents stimuli of varying difficulty levels, and measures...
OBJECTIVE
This study aims to control all hearing thresholds, including extended high frequencies (EHFs), presents stimuli of varying difficulty levels, and measures electroencephalography (EEG) and pupillometry responses to determine whether listening difficulty in tinnitus patients is effort or fatigue-related.
METHODS
Twenty-one chronic tinnitus patients and 26 matched healthy controls having normal pure-tone averages with symmetrical hearing thresholds were included. Subjects were evaluated with 0.125-20 kHz pure-tone audiometry, Montreal Cognitive Assessment Test (MoCA), Tinnitus Handicap Inventory (THI), EEG, and pupillometry.
RESULTS
Pupil dilatation and EEG alpha power during the "encoding" phase of the presented sentence in tinnitus patients were less in all listening conditions (p < .05). Also, there was no statistically significant relationship between EEG and pupillometry components for all listening conditions and THI or MoCA (p > .05).
CONCLUSION
EEG and pupillometry results under various listening conditions indicate potential listening effort in tinnitus patients even if all frequencies, including EHFs, are controlled. Also, we suggest that pupillometry should be interpreted with caution in autonomic nervous system-related conditions such as tinnitus.
Topics: Humans; Tinnitus; Male; Female; Electroencephalography; Adult; Middle Aged; Pupil; Audiometry, Pure-Tone; Auditory Perception; Auditory Threshold
PubMed: 38841736
DOI: 10.1002/brb3.3571 -
Nature Medicine Jun 2024Gene therapy is a promising approach for hereditary deafness. We recently showed that unilateral AAV1-hOTOF gene therapy with dual adeno-associated virus (AAV) serotype...
Gene therapy is a promising approach for hereditary deafness. We recently showed that unilateral AAV1-hOTOF gene therapy with dual adeno-associated virus (AAV) serotype 1 carrying human OTOF transgene is safe and associated with functional improvements in patients with autosomal recessive deafness 9 (DFNB9). The protocol was subsequently amended and approved to allow bilateral gene therapy administration. Here we report an interim analysis of the single-arm trial investigating the safety and efficacy of binaural therapy in five pediatric patients with DFNB9. The primary endpoint was dose-limiting toxicity at 6 weeks, and the secondary endpoint included safety (adverse events) and efficacy (auditory function and speech perception). No dose-limiting toxicity or serious adverse event occurred. A total of 36 adverse events occurred. The most common adverse events were increased lymphocyte counts (6 out of 36) and increased cholesterol levels (6 out of 36). All patients had bilateral hearing restoration. The average auditory brainstem response threshold in the right (left) ear was >95 dB (>95 dB) in all patients at baseline, and the average auditory brainstem response threshold in the right (left) ear was restored to 58 dB (58 dB) in patient 1, 75 dB (85 dB) in patient 2, 55 dB (50 dB) in patient 3 at 26 weeks, and 75 dB (78 dB) in patient 4 and 63 dB (63 dB) in patient 5 at 13 weeks. The speech perception and the capability of sound source localization were restored in all five patients. These results provide preliminary insights on the safety and efficacy of binaural AAV gene therapy for hereditary deafness. The trial is ongoing with longer follow-up to confirm the safety and efficacy findings. Chinese Clinical Trial Registry registration: ChiCTR2200063181 .
PubMed: 38839897
DOI: 10.1038/s41591-024-03023-5 -
CoDAS 2024To propose an instrument for assessing speech recognition in the presence of competing noise. To define its application strategy for use in clinical practice. To obtain...
PURPOSE
To propose an instrument for assessing speech recognition in the presence of competing noise. To define its application strategy for use in clinical practice. To obtain evidence of criterion validity and present reference values.
METHODS
The study was conducted in three stages: Organization of the material comprising the Word-with-Noise Test (Stage 1); Definition of the instrument's application strategy (Stage 2); Investigation of criterion validity and definition of reference values for the test (Stage 3) through the evaluation of 50 normal-hearing adult subjects and 12 subjects with hearing loss.
RESULTS
The Word-with-Noise Test consists of lists of monosyllabic and disyllabic words and speech spectrum noise (Stage 1). The application strategy for the test was defined as the determination of the Speech Recognition Threshold with a fixed noise level at 55 dBHL (Stage 2). Regarding criterion validity, the instrument demonstrated adequate ability to distinguish between normal-hearing subjects and subjects with hearing loss (Stage 3). Reference values for the test were established as cut-off points expressed in terms of signal-to-noise ratio: 1.47 dB for the monosyllabic stimulus and -2.02 dB for the disyllabic stimulus. Conclusion: The Word-with-Noise Test proved to be quick to administer and interpret, making it a useful tool in audiological clinical practice. Furthermore, it showed satisfactory evidence of criterion validity, with established reference values.
Topics: Humans; Reference Values; Adult; Female; Male; Noise; Young Adult; Reproducibility of Results; Middle Aged; Speech Perception; Signal-To-Noise Ratio; Auditory Threshold; Case-Control Studies; Hearing Loss; Speech Reception Threshold Test; Aged; Adolescent
PubMed: 38836822
DOI: 10.1590/2317-1782/20242023091pt -
Trends in Hearing 2024The use of in-situ audiometry for hearing aid fitting is appealing due to its reduced resource and equipment requirements compared to standard approaches employing... (Comparative Study)
Comparative Study
The use of in-situ audiometry for hearing aid fitting is appealing due to its reduced resource and equipment requirements compared to standard approaches employing conventional audiometry alongside real-ear measures. However, its validity has been a subject of debate, as previous studies noted differences between hearing thresholds measured using conventional and in-situ audiometry. The differences were particularly notable for open-fit hearing aids, attributed to low-frequency leakage caused by the vent. Here, in-situ audiometry was investigated for six receiver-in-canal hearing aids from different manufacturers through three experiments. In Experiment I, the hearing aid gain was measured to investigate whether corrections were implemented to the prescribed target gain. In Experiment II, the in-situ stimuli were recorded to investigate if corrections were directly incorporated to the delivered in-situ stimulus. Finally, in Experiment III, hearing thresholds using in-situ and conventional audiometry were measured with real patients wearing open-fit hearing aids. Results indicated that (1) the hearing aid gain remained unaffected when measured with in-situ or conventional audiometry for all open-fit measurements, (2) the in-situ stimuli were adjusted for up to 30 dB at frequencies below 1000 Hz for all open-fit hearing aids except one, which also recommends the use of closed domes for all in-situ measurements, and (3) the mean interparticipant threshold difference fell within 5 dB for frequencies between 250 and 6000 Hz. The results clearly indicated that modern measured in-situ thresholds align (within 5 dB) with conventional thresholds measured, indicating the potential of in-situ audiometry for remote hearing care.
Topics: Humans; Hearing Aids; Auditory Threshold; Acoustic Stimulation; Prosthesis Fitting; Reproducibility of Results; Audiometry; Audiometry, Pure-Tone; Hearing Loss; Hearing; Predictive Value of Tests; Persons With Hearing Impairments; Equipment Design; Male; Female
PubMed: 38835268
DOI: 10.1177/23312165241259704 -
Revue Neurologique Jun 2024Correcting of the lack of regularity in steps is a key component of gait rehabilitation in Parkinson's disease. We proposed to introduce adaptive spatial auditory cueing...
BACKGROUND
Correcting of the lack of regularity in steps is a key component of gait rehabilitation in Parkinson's disease. We proposed to introduce adaptive spatial auditory cueing (ASAC) based on verbal instruction "lengthen the step" automatically delivered when the stride length decreased below a predetermined threshold.
OBJECTIVES
The present study compared the effect of usual rhythmic auditory cueing versus ASAC used during a walking training in Parkinson's disease.
METHODS
Fifteen patients with Parkinson's disease performed both interventions in randomized order, one week apart: a 20-minute walking training with rhythmic auditory cueing, in form of a metronome adjusted on 110% of the patient's own cadence, or ASAC delivered when the stride length is less than 110% of the patient's own stride length. Assessment criteria were walking distance covered during the intervention, speed, step length, cadence, coefficients of variation of step length and step duration, and indexes of spatial and temporal asymmetry during a walking test before and just after the intervention.
RESULTS
The walking distance is higher with ASAC compared with rhythmic auditory cueing (rhythmic auditory cueing, 905 (203) m, mean (standard deviation); ASAC, 1043 (212) m; P=0.002). Between-intervention comparison showed some similar effects on walking after the intervention including free speed and step length increases (P<0.05).
CONCLUSION
The distance covered during 20-minute walking with ASAC increases by 15% compared to the use of classical rhythmic auditory cueing, while the immediate therapeutic effects show similar spatial-temporal benefits on short-distance walking. Auditory biofeedback cueing promoting the increase in step length might improve gait relearning in Parkinson's disease.
PubMed: 38834484
DOI: 10.1016/j.neurol.2024.05.003 -
BioRxiv : the Preprint Server For... May 2024Prestin's voltage-driven motor activity confers sound-elicited somatic electromotility in auditory outer hair cells (OHCs) and is essential for the exquisite sensitivity...
UNLABELLED
Prestin's voltage-driven motor activity confers sound-elicited somatic electromotility in auditory outer hair cells (OHCs) and is essential for the exquisite sensitivity and frequency selectivity of mammalian hearing. Lack of prestin results in hearing threshold shifts across frequency, supporting the causal association of variants in the prestin-coding gene, , with human hearing loss, DFNB61. However, cochlear function can tolerate reductions in prestin-mediated OHC electromotility. We found that two deafness-associated prestin variants, p.A100T and p.P119S, do not deprive prestin of its fast motor function but significantly reduce membrane expression, leading to large reductions in OHC electromotility that were only ∼30% of wildtype (WT). Mice harboring these missense variants suffered congenital hearing loss that was worse at high frequencies; however, they retained WT-like auditory brainstem response thresholds at 8 kHz, which is processed at the apex of the mouse cochlea. This observation suggests the increasing importance of prestin-driven cochlear amplification at higher frequencies relevant to mammalian hearing. The observation also suggests the promising clinical possibility that small enhancements of OHC electromotility could significantly ameliorate DFNB61 hearing loss in human patients.
SIGNIFICANCE
Prestin is abundantly expressed in the auditory outer hair cells and is essential for normal cochlear operation. Hence, reduction of prestin expression is often taken as indicative of reduced cochlear function in diseased or aged ears. However, this assumption overlooks the fact that cochlear function can tolerate large reductions in prestin motor activity. DFNB61 mouse models generated and characterized in this study provide an opportunity to gauge the amount of prestin motor activity needed to sustain normal hearing sensitivity. This knowledge is crucial not only for understanding the pathogenic roles of deafness-associated variants that impair OHC electromotility but also for unraveling how prestin contributes to cochlear amplification.
PubMed: 38826260
DOI: 10.1101/2024.05.22.595389 -
Ear and Hearing Jun 2024Objective estimation of minimum hearing levels using auditory brainstem responses (ABRs) elicited by single frequency tone-bursts presented monaurally is currently...
OBJECTIVES
Objective estimation of minimum hearing levels using auditory brainstem responses (ABRs) elicited by single frequency tone-bursts presented monaurally is currently considered the gold standard. However, the data acquisition time to estimate thresholds (for both ears across four audiometric frequencies) using this method usually exceeds the sleep time (ranging between 35 and 49 minutes) in infants below 4 months, thus providing incomplete information of hearing status which in turn delays timely clinical intervention. Alternate approaches using faster rate, or tone-burst trains have not been readily accepted due to additional hardware and software requirements. We propose here a novel binaural multifrequency stimulation paradigm wherein several stimuli of different frequencies are presented binaurally in an interleaved manner. The rationale here is that the proposed paradigm will increase acquisition efficiency, significantly reduce test time, and improve accuracy by incorporating an automatic wave V detection algorithm. It is important to note that this paradigm can be easily implemented in most commercial ABR systems currently used by most clinicians.
DESIGN
Using this binaural multifrequency paradigm, ear specific ABRs were recorded in 30 normal-hearing young adults to both tone-bursts, and narrow-band (NB) iChirps at 500, 1000, 2000, and 4000 Hz. Comparison of ABRs elicited by tone-bursts and narrow-band chirps allowed us to determine if NB iChirps elicited a more robust wave V component compared with the tone-bursts. ABR data were characterized by measures of minimum hearing levels; wave V amplitude; and response detectability for two electrode configurations (high forehead-C7; and high forehead-linked mastoids).
RESULTS
Consistent with the research literature, wave V response amplitudes were relatively more robust for NB iChirp stimuli compared with tone-burst stimuli. The easier identification and better detectability of wave V for the NB iChirps at lower stimulus levels contributed to their better thresholds compared with tone-burst elicited responses. It is important to note that binaural multifrequency hearing levels close to minimum hearing levels were determined in approximately 22 minutes using this paradigm-appreciably quicker than the 45 to 60 minutes or longer time required for threshold determination using the conventional single frequency method.
CONCLUSIONS
Our novel and simple paradigm using either NB iChirps or tone-bursts provides a reliable method to rapidly estimate the minimum hearing levels across audiometric frequencies for both ears. Incorporation of an automatic wave V detection algorithm increases objectivity and further reduce test time and facilitate early hearing identification and intervention.
PubMed: 38825740
DOI: 10.1097/AUD.0000000000001535 -
Ear and Hearing May 2024Electrocochleography (ECochG) appears to offer the most accurate prediction of post-cochlear implant hearing outcomes. This may be related to its capacity to interrogate...
OBJECTIVES
Electrocochleography (ECochG) appears to offer the most accurate prediction of post-cochlear implant hearing outcomes. This may be related to its capacity to interrogate the health of underlying cochlear tissue. The four major components of ECochG (cochlear microphonic [CM], summating potential [SP], compound action potential [CAP], and auditory nerve neurophonic [ANN]) are generated by different cochlear tissue components. Analyzing characteristics of these components can reveal the state of hair and neural cell in a cochlea. There is limited evidence on the characteristics of intracochlear (IC) ECochG recordings measured across the array postinsertion but compared with extracochlear recordings has better signal to noise ratio and spatial specificity. The present study aimed to examine the relationship between ECochG components recorded from an IC approach and postoperative speech perception or audiometric thresholds.
DESIGN
In 113 human subjects, responses to 500 Hz tone bursts were recorded at 11 IC electrodes across a 22-electrode cochlear implant array immediately following insertion. Responses to condensation and rarefaction stimuli were then subtracted from one another to emphasize the CM and added to one another to emphasize the SP, ANN, and CAP. Maximum amplitudes and extracochlear electrode locations were recorded for each of these ECochG components. These were added stepwise to a multi-factor generalized additive model to develop a best-fit model predictive model for pure-tone audiometric thresholds (PTA) and speech perception scores (speech recognition threshold [SRT] and consonant-vowel-consonant phoneme [CVC-P]) at 3- and 12-month postoperative timepoints. This best-fit model was tested against a generalized additive model using clinical factors alone (preoperative score, age, and gender) as a null model proxy.
RESULTS
ECochG-factor models were superior to clinical factor models in predicting postoperative PTA, CVC-P, and SRT outcomes at both timepoints. Clinical factor models explained a moderate amount of PTA variance ( r2 = 45.9% at 3-month, 31.8% at 12-month, both p < 0.001) and smaller variances of CVC-P and SRT ( r2 range = 6 to 13.7%, p = 0.008 to 0.113). Age was not a significant predictive factor. ECochG models explained more variance at the 12-month timepoint ( r2 for PTA = 52.9%, CVC-P = 39.6%, SRT = 36.4%) compared with the 3-month one timepoint ( r2 for PTA = 49.4%, CVC-P = 26.5%, SRT = 22.3%). The ECochG model was based on three factors: maximum SP deflection amplitude, and electrode position of CM and SP peaks. Adding neural (ANN and/or CAP) factors to the model did not improve variance explanation. Large negative SP deflection was associated with poorer outcomes and a large positive SP deflection with better postoperative outcomes. Mid-array peaks of SP and CM were both associated with poorer outcomes.
CONCLUSIONS
Postinsertion IC-ECochG recordings across the array can explain a moderate amount of postoperative speech perception and audiometric thresholds. Maximum SP deflection and its location across the array appear to have a significant predictive value which may reflect the underlying state of cochlear health.
PubMed: 38816899
DOI: 10.1097/AUD.0000000000001506 -
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke... May 2024This study aimed to compare the audiological characteristics between children with unilateral auditory neuropathy (UAN) and single-sided deafness (SSD) to establish a...
This study aimed to compare the audiological characteristics between children with unilateral auditory neuropathy (UAN) and single-sided deafness (SSD) to establish a valid basis for the differential diagnosis of children with UAN. A retrospective analysis was conducted on audiological and imaging evaluations of children with UAN and SSD who were treated at Beijing Children's Hospital of Capital Medical University between May 2015 and June 2023. There were 17 children with UAN, comprising 10 males and 7 females, with an average age of 4.7 years. Additionally, there were 43 children with SSD, consisting of 27 males and 16 females, with an average age of 6.5 years. Audiological assessments included Auditory brainstem response (ABR), Steady-state auditory evoked potential (ASSR), Behavioural audiometry, Cochlear microphonic potential (CM), Distortino-product otoacoustic emission (DPOAE), and acoustic immittance test. The results of the audiological assessment and imaging phenotypic between the two groups of children were compared and analyzed by applying SPSS 27.0 statistical software. (1) The UAN group (77.8%) had a significantly higher rate of ABR wave III than the SSD group (20.9%) (<0.01). The PA thresholds at 500 Hz and 1 000 Hz of children with SSD were higher than those of children with UAN, while the ASSR thresholds at 500 Hz, 1000 Hz, 2 000 Hz, and 4 000 Hz of children with SSD were significantly higher than those of children with UAN (<0.05). (2) The degree of hearing loss in both UAN and SSD children was predominantly complete hearing loss. The percentage of complete hearing loss was significantly higher (χ²=4.353, =0.037) in the SSD group (93.0%, 40/43) than in the UAN group (63.6%, 7/11). However, the percentage of profound hearing loss was significantly higher in the UAN group (27.3%, 3/11) than in the SSD group (2.3%, 1/43) (0.023). In terms of hearing curve configuration, the percentage of flat type was significantly higher in the SSD group (76.7%, 33/43) than in the UAN group (36.4%, 4/11). The proportion of the UAN group (27.3%, 3/11) was significantly higher than that in the SSD group (2.3%, 1/43) in ascending type (<0.05). There were no statistically significant differences in the hearing curves of the declining type and other types between the two groups (>0.05). (3) The proportion of imaging assessment without abnormality was significantly more common in the UAN group (81.8%) than in the SSD group (37.1%) (χ²=6.695, =0.015). Compared to children with SSD, the occurrence of wave III on the ABR test was significantly more common in children with UAN. The percentage of ascending hearing curves was significantly higher in children with UAN than in children with SSD. ASSR thresholds were significantly lower in children with UAN. The normal imaging phenotype was significantly more common in children with UAN than in children with SSD.
Topics: Humans; Female; Male; Retrospective Studies; Child, Preschool; Child; Evoked Potentials, Auditory, Brain Stem; Hearing Loss, Central; Hearing Loss, Unilateral; Auditory Threshold; Audiometry; Diagnosis, Differential
PubMed: 38811175
DOI: 10.3760/cma.j.cn115330-20231129-00248