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1.
Trends Hear ; 28: 23312165241260029, 2024.
Article in English | MEDLINE | ID: mdl-38831646

ABSTRACT

The extent to which active noise cancelation (ANC), when combined with hearing assistance, can improve speech intelligibility in noise is not well understood. One possible source of benefit is ANC's ability to reduce the sound level of the direct (i.e., vent-transmitted) path. This reduction lowers the "floor" imposed by the direct path, thereby allowing any increases to the signal-to-noise ratio (SNR) created in the amplified path to be "realized" at the eardrum. Here we used a modeling approach to estimate this benefit. We compared pairs of simulated hearing aids that differ only in terms of their ability to provide ANC and computed intelligibility metrics on their outputs. The difference in metric scores between simulated devices is termed the "ANC Benefit." These simulations show that ANC Benefit increases as (1) the environmental sound level increases, (2) the ability of the hearing aid to improve SNR increases, (3) the strength of the ANC increases, and (4) the hearing loss severity decreases. The predicted size of the ANC Benefit can be substantial. For a moderate hearing loss, the model predicts improvement in intelligibility metrics of >30% when environments are moderately loud (>70 dB SPL) and devices are moderately capable of increasing SNR (by >4 dB). It appears that ANC can be a critical ingredient in hearing devices that attempt to improve SNR in loud environments. ANC will become more and more important as advanced SNR-improving algorithms (e.g., artificial intelligence speech enhancement) are included in hearing devices.


Subject(s)
Hearing Aids , Noise , Perceptual Masking , Signal-To-Noise Ratio , Speech Intelligibility , Speech Perception , Humans , Noise/adverse effects , Computer Simulation , Acoustic Stimulation , Correction of Hearing Impairment/instrumentation , Persons With Hearing Impairments/rehabilitation , Persons With Hearing Impairments/psychology , Hearing Loss/diagnosis , Hearing Loss/rehabilitation , Hearing Loss/physiopathology , Equipment Design , Signal Processing, Computer-Assisted
2.
Proc Natl Acad Sci U S A ; 121(24): e2311570121, 2024 Jun 11.
Article in English | MEDLINE | ID: mdl-38830095

ABSTRACT

Even a transient period of hearing loss during the developmental critical period can induce long-lasting deficits in temporal and spectral perception. These perceptual deficits correlate with speech perception in humans. In gerbils, these hearing loss-induced perceptual deficits are correlated with a reduction of both ionotropic GABAA and metabotropic GABAB receptor-mediated synaptic inhibition in auditory cortex, but most research on critical period plasticity has focused on GABAA receptors. Therefore, we developed viral vectors to express proteins that would upregulate gerbil postsynaptic inhibitory receptor subunits (GABAA, Gabra1; GABAB, Gabbr1b) in pyramidal neurons, and an enzyme that mediates GABA synthesis (GAD65) presynaptically in parvalbumin-expressing interneurons. A transient period of developmental hearing loss during the auditory critical period significantly impaired perceptual performance on two auditory tasks: amplitude modulation depth detection and spectral modulation depth detection. We then tested the capacity of each vector to restore perceptual performance on these auditory tasks. While both GABA receptor vectors increased the amplitude of cortical inhibitory postsynaptic potentials, only viral expression of postsynaptic GABAB receptors improved perceptual thresholds to control levels. Similarly, presynaptic GAD65 expression improved perceptual performance on spectral modulation detection. These findings suggest that recovering performance on auditory perceptual tasks depends on GABAB receptor-dependent transmission at the auditory cortex parvalbumin to pyramidal synapse and point to potential therapeutic targets for developmental sensory disorders.


Subject(s)
Auditory Cortex , Gerbillinae , Hearing Loss , Animals , Auditory Cortex/metabolism , Auditory Cortex/physiopathology , Hearing Loss/genetics , Hearing Loss/physiopathology , Receptors, GABA-B/metabolism , Receptors, GABA-B/genetics , Glutamate Decarboxylase/metabolism , Glutamate Decarboxylase/genetics , Receptors, GABA-A/metabolism , Receptors, GABA-A/genetics , Parvalbumins/metabolism , Parvalbumins/genetics , Auditory Perception/physiology , Pyramidal Cells/metabolism , Pyramidal Cells/physiology , Genetic Vectors/genetics
3.
Trends Hear ; 28: 23312165241252240, 2024.
Article in English | MEDLINE | ID: mdl-38715410

ABSTRACT

In recent years, tools for early detection of irreversible trauma to the basilar membrane during hearing preservation cochlear implant (CI) surgery were established in several clinics. A link with the degree of postoperative hearing preservation in patients was investigated, but patient populations were usually small. Therefore, this study's aim was to analyze data from intraoperative extracochlear electrocochleography (ECochG) recordings for a larger group.During hearing preservation CI surgery, extracochlear recordings were made before, during, and after CI electrode insertion using a cotton wick electrode placed at the promontory. Before and after insertion, amplitudes and stimulus response thresholds were recorded at 250, 500, and 1000 Hz. During insertion, response amplitudes were recorded at one frequency and one stimulus level. Data from 121 patient ears were analyzed.The key benefit of extracochlear recordings is that they can be performed before, during, and after CI electrode insertion. However, extracochlear ECochG threshold changes before and after CI insertion were relatively small and did not independently correlate well with hearing preservation, although at 250 Hz they added some significant information. Some tendencies-although no significant relationships-were detected between amplitude behavior and hearing preservation. Rising amplitudes seem favorable and falling amplitudes disadvantageous, but constant amplitudes do not appear to allow stringent predictions.Extracochlear ECochG measurements seem to only partially realize expected benefits. The questions now are: do gains justify the effort, and do other procedures or possible combinations lead to greater benefits for patients?


Subject(s)
Audiometry, Evoked Response , Auditory Threshold , Cochlea , Cochlear Implantation , Cochlear Implants , Hearing , Humans , Audiometry, Evoked Response/methods , Retrospective Studies , Cochlear Implantation/instrumentation , Female , Middle Aged , Male , Aged , Adult , Hearing/physiology , Cochlea/surgery , Cochlea/physiopathology , Treatment Outcome , Adolescent , Predictive Value of Tests , Young Adult , Child , Audiometry, Pure-Tone , Aged, 80 and over , Child, Preschool , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Hearing Loss/surgery , Hearing Loss/rehabilitation
4.
J Neural Eng ; 21(3)2024 May 22.
Article in English | MEDLINE | ID: mdl-38729132

ABSTRACT

Objective.This study develops a deep learning (DL) method for fast auditory attention decoding (AAD) using electroencephalography (EEG) from listeners with hearing impairment (HI). It addresses three classification tasks: differentiating noise from speech-in-noise, classifying the direction of attended speech (left vs. right) and identifying the activation status of hearing aid noise reduction algorithms (OFF vs. ON). These tasks contribute to our understanding of how hearing technology influences auditory processing in the hearing-impaired population.Approach.Deep convolutional neural network (DCNN) models were designed for each task. Two training strategies were employed to clarify the impact of data splitting on AAD tasks: inter-trial, where the testing set used classification windows from trials that the training set had not seen, and intra-trial, where the testing set used unseen classification windows from trials where other segments were seen during training. The models were evaluated on EEG data from 31 participants with HI, listening to competing talkers amidst background noise.Main results.Using 1 s classification windows, DCNN models achieve accuracy (ACC) of 69.8%, 73.3% and 82.9% and area-under-curve (AUC) of 77.2%, 80.6% and 92.1% for the three tasks respectively on inter-trial strategy. In the intra-trial strategy, they achieved ACC of 87.9%, 80.1% and 97.5%, along with AUC of 94.6%, 89.1%, and 99.8%. Our DCNN models show good performance on short 1 s EEG samples, making them suitable for real-world applications. Conclusion: Our DCNN models successfully addressed three tasks with short 1 s EEG windows from participants with HI, showcasing their potential. While the inter-trial strategy demonstrated promise for assessing AAD, the intra-trial approach yielded inflated results, underscoring the important role of proper data splitting in EEG-based AAD tasks.Significance.Our findings showcase the promising potential of EEG-based tools for assessing auditory attention in clinical contexts and advancing hearing technology, while also promoting further exploration of alternative DL architectures and their potential constraints.


Subject(s)
Attention , Auditory Perception , Deep Learning , Electroencephalography , Hearing Loss , Humans , Attention/physiology , Female , Electroencephalography/methods , Male , Middle Aged , Hearing Loss/physiopathology , Hearing Loss/rehabilitation , Hearing Loss/diagnosis , Aged , Auditory Perception/physiology , Noise , Adult , Hearing Aids , Speech Perception/physiology , Neural Networks, Computer
5.
Article in Chinese | MEDLINE | ID: mdl-38811174

ABSTRACT

Objective: The purpose of this study was to analyze the clinical characteristics of auditory neuropathy (AN) patients with normal hearing or mild hearing loss. Methods: Data from Multicenter Study on Clinical Diagnosis and Intervention of Acoustic Neuropathy (registration number: ChiCTR2100050125). According to the Chinese clinical practice guideline of auditory neuropathy (version 2022), these patients divided into two groups: the normal hearing group (PTA Normal, PTAN group, the average hearing threshold<20 dB HL) and the mild hearing loss group (PTA Mild hearing loss, PTAM group, the average hearing threshold between 20-35 dBHL). The audiology characteristics, clinical features, and follow-up were analyzed. Data analysis was conducted using GraphPad Prism 8 and SPSS 20.0 software. Results: A total of 75 AN with normal hearing or mild hearing loss were included in this study. The PTAN group consisted of 19 patients (38 ears), including 12 males and 7 females. The average onset age was (16.9±4.5) years old, while the test age was (22.1±5.8) years old for PTAN group. The PTAM group consisted of 56 patients (112 ears), including 29 males and 27 females. The average onset age was (16.2±7.9) years old, while the test age was (23.9±9.0) yeas old for PTAM group. The average hearing threshold of low frequency (0.125-0.5 kHz) was significantly decreased. ABR disappeared in 86.00% (126/150) of the patients. The speech recognition rate was 71.80±22.44% in the PTAN group and 58.08±29.28% in the PTAM group.-SP/AP was 0.98±0.47 in the PTAN and 1.07±0.63 in PTAM group; 40 (53.33%) patients had tinnitus. 29 patients (58 ears) were followed up, including 10 patients (20 ears) in the PTAN group and 19 patients (38 ears) in the PTAM group. There was no significant change in hearing threshold in short-term follow-up (<3 years). With the extension of the disease duration (>3 years), the PTAN group tended to decrease at low frequency, and the PTAM group decreased at high frequency first. The hearing threshold at 0.25 kHz in the PTAN group and 4 kHz in the PTAM group decreased significantly. Conclusions: AN patients with normal hearing or mild hearing loss exhibit abnormal results in audiological examination results, including ABR, electrocochleography and speech discrimination score. A combination of audiological tests should be used to make the diagnosis of AN. With the progression of the disease, AN with normal hearing or mild hearing loss tends to decrease.


Subject(s)
Audiometry, Pure-Tone , Auditory Threshold , Hearing Loss, Central , Humans , Hearing Loss, Central/diagnosis , Hearing Loss, Central/physiopathology , Male , Female , Adult , Young Adult , Adolescent , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Child , Middle Aged
6.
Hear Res ; 447: 109024, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38735179

ABSTRACT

Delayed loss of residual acoustic hearing after cochlear implantation is a common but poorly understood phenomenon due to the scarcity of relevant temporal bone tissues. Prior histopathological analysis of one case of post-implantation hearing loss suggested there were no interaural differences in hair cell or neural degeneration to explain the profound loss of low-frequency hearing on the implanted side (Quesnel et al., 2016) and attributed the threshold elevation to neo-ossification and fibrosis around the implant. Here we re-evaluated the histopathology in this case, applying immunostaining and improved microscopic techniques for differentiating surviving hair cells from supporting cells. The new analysis revealed dramatic interaural differences, with a > 80 % loss of inner hair cells in the cochlear apex on the implanted side, which can account for the post-implantation loss of residual hearing. Apical degeneration of the stria further contributed to threshold elevation on the implanted side. In contrast, spiral ganglion cell survival was reduced in the region of the electrode on the implanted side, but apical counts in the two ears were similar to that seen in age-matched unimplanted control ears. Almost none of the surviving auditory neurons retained peripheral axons throughout the basal half of the cochlea. Relevance to cochlear implant performance is discussed.


Subject(s)
Auditory Threshold , Cochlear Implantation , Cochlear Implants , Spiral Ganglion , Cochlear Implantation/instrumentation , Cochlear Implantation/adverse effects , Humans , Spiral Ganglion/pathology , Spiral Ganglion/physiopathology , Hair Cells, Auditory, Inner/pathology , Time Factors , Cell Survival , Male , Hearing , Hearing Loss/physiopathology , Hearing Loss/pathology , Hearing Loss/surgery , Hearing Loss/etiology , Female , Hair Cells, Auditory/pathology , Aged , Nerve Degeneration , Middle Aged , Temporal Bone/pathology , Temporal Bone/surgery
7.
Hear Res ; 447: 109021, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38703432

ABSTRACT

Understanding the complex pathologies associated with hearing loss is a significant motivation for conducting inner ear research. Lifelong exposure to loud noise, ototoxic drugs, genetic diversity, sex, and aging collectively contribute to human hearing loss. Replicating this pathology in research animals is challenging because hearing impairment has varied causes and different manifestations. A central aspect, however, is the loss of sensory hair cells and the inability of the mammalian cochlea to replace them. Researching therapeutic strategies to rekindle regenerative cochlear capacity, therefore, requires the generation of animal models in which cochlear hair cells are eliminated. This review discusses different approaches to ablate cochlear hair cells in adult mice. We inventoried the cochlear cyto- and histo-pathology caused by acoustic overstimulation, systemic and locally applied drugs, and various genetic tools. The focus is not to prescribe a perfect damage model but to highlight the limitations and advantages of existing approaches and identify areas for further refinement of damage models for use in regenerative studies.


Subject(s)
Cochlea , Disease Models, Animal , Hair Cells, Auditory , Regeneration , Animals , Hair Cells, Auditory/pathology , Hair Cells, Auditory/metabolism , Mice , Cochlea/pathology , Cochlea/physiopathology , Humans , Hearing , Hearing Loss, Noise-Induced/physiopathology , Hearing Loss, Noise-Induced/pathology , Hearing Loss/pathology , Hearing Loss/physiopathology , Acoustic Stimulation
8.
Hear Res ; 447: 109012, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38703433

ABSTRACT

Hearing loss is a common side effect of many tumor treatments. However, hearing loss can also occur as a direct result of certain tumors of the nervous system, the most common of which are the vestibular schwannomas (VS). These tumors arise from Schwann cells of the vestibulocochlear nerve and their main cause is the loss of function of NF2, with 95 % of cases being sporadic and 5 % being part of the rare neurofibromatosis type 2 (NF2)-related Schwannomatosis. Genetic variations in NF2 do not fully explain the clinical heterogeneity of VS, and interactions between Schwann cells and their microenvironment appear to be critical for tumor development. Preclinical in vitro and in vivo models of VS are needed to develop prognostic biomarkers and targeted therapies. In addition to VS, other tumors can affect hearing. Meningiomas and other masses in the cerebellopontine angle can compress the vestibulocochlear nerve due to their anatomic proximity. Gliomas can disrupt several neurological functions, including hearing; in fact, glioblastoma multiforme, the most aggressive subtype, may exhibit early symptoms of auditory alterations. Besides, treatments for high-grade tumors, including chemotherapy or radiotherapy, as well as incomplete resections, can induce long-term auditory dysfunction. Because hearing loss can have an irreversible and dramatic impact on quality of life, it should be considered in the clinical management plan of patients with tumors, and monitored throughout the course of the disease.


Subject(s)
Hearing Loss , Hearing , Neuroma, Acoustic , Humans , Neuroma, Acoustic/pathology , Neuroma, Acoustic/physiopathology , Neuroma, Acoustic/complications , Hearing Loss/physiopathology , Hearing Loss/etiology , Hearing Loss/pathology , Animals , Neurilemmoma/pathology , Neurilemmoma/complications , Neurilemmoma/therapy , Vestibulocochlear Nerve/pathology , Vestibulocochlear Nerve/physiopathology , Risk Factors , Neurofibromatosis 2/genetics , Neurofibromatosis 2/complications , Neurofibromatosis 2/pathology , Neurofibromatosis 2/physiopathology , Neurofibromatosis 2/therapy , Meningioma/pathology , Meningioma/physiopathology , Meningioma/complications
9.
Med J Malaysia ; 79(3): 360-369, 2024 May.
Article in English | MEDLINE | ID: mdl-38817071

ABSTRACT

INTRODUCTION: Various evaluation tools have been developed to track the growth of auditory-related behaviours of children with hearing loss during intervention. However, the reliability and validity of currently available outcome measures remain uncertain due to the lack of information on their psychometric properties. A lack of reliable outcome measures may jeopardise intervention quality and affect these children's listening skills progression. This scoping review aims to explore the mechanics of producing or developing an outcome measure either completely new or adapted from the original version that is considered as having robust statistical properties. MATERIALS AND METHODS: A scoping review was conducted across four databases (PubMed, ScienceDirect, Scopus and Google Scholar). The included articles were written in English, published between January 2010 and June 2023, and specific to predefined keywords. Two independent reviewers screened and selected the final papers using the PRISMScR checklist. A code framework was created to extract information about the publications and conducted by one reviewer. The results were reported using descriptive statistics and narrative synthesis. RESULTS: The final analysis were conducted on 22 articles out of 452 articles screened. The review identified seven outcome measures presented in various languages. The outcome measures found were the Auditory Behaviour in Everyday Life (ABEL), Functional Listening Index for Paediatric (FLI-P), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), Integrated Scales of Development (ISD), LittlEARS Auditory Questionnaire (LEAQ), Parent's Evaluation of Aural/Oral Performance in Children (PEACH), Parent's Evaluation of Aural/Oral Performance in Children Diary (PEACH Diary), Teachers' Evaluation of Aural/Oral Performance in Children (TEACH) and Parent's Evaluation of Aural/Oral Performance in Children Plus (PEACH+). A total of 13 studies focused on translating, adapting and validating an outcome measure while the remaining investigations validated either the translated or original version of the outcome measures. All original instruments were developed in English and among Western culture, except for the LEAQ which was designed in the German language and for the German population. The outcome measures identified were translated and adapted into Spanish, Turkish, Persian, Hebrew, Arabic, Malay, Yoruba, Polish, Swedish, Hindi, Portuguese, Kannada and Mandarin. CONCLUSION: All studies performed an extensive evaluation of psychometric properties and feasibility studies to produce an excellent quality of auditory-related behaviour outcome measure for clinical use with the intended population. A new outcome measure, FLI-P, was found to be clinically useful for the primary provider of learning to listen and spoken language training for children with hearing impairment in Malaysia, i.e., the speech-language therapists.


Subject(s)
Hearing Loss , Outcome Assessment, Health Care , Humans , Hearing Loss/physiopathology , Hearing Loss/psychology , Child , Child, Preschool , Psychometrics , Infant , Reproducibility of Results
10.
Otolaryngol Head Neck Surg ; 170(6): 1712-1722, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38494745

ABSTRACT

OBJECTIVE: To examine the association between hearing function, assessed with pure-tone average (PTA) of air conduction thresholds, and 24-hour ambulatory blood pressure (BP) in older adults. STUDY DESIGN: Cross-sectional study. SETTING: A total of 1404 community-dwelling individuals aged ≥65 years from the Seniors-ENRICA cohort were examined. METHODS: Hearing loss was defined as PTA > 40-AudCal hearing loss decibels (dB-aHL) in the better ear for standard frequency (0.5, 1, and 2 kHz), speech frequency (0.5, 1, 2, and 4 kHz), and high frequency (3, 4, and 8 kHz). Circadian BP patterns were calculated as the percentage decline in systolic BP during the night, and participants were classified as dipper, nondipper, and riser. Ambulatory hypertension was defined as BP ≥ 130/80 mm Hg (24 hour), ≥135/85 (daytime), and ≥120/70 (nighttime) or on antihypertensive treatment. Analyses were performed with linear- and logistic-regression models adjusted for the main confounders. RESULTS: In multivariable analyses, the PTA was associated with higher nighttime systolic BP [ß coefficient per 20 dB-aHL increment standard frequency (95% confidence interval, CI): 2.41 mm Hg (0.87, 3.95); ß (95% CI) per 20 dB-aHL increment speech frequency 2.17 mm Hg (0.70, 3.64)]. Among hypertensive patients, hearing loss at standard and high-frequency PTA was associated with the riser BP pattern [odds ratio: 2.01 (95% CI, 1.03-3.93) and 1.45 (1.00-2.09), respectively]; also, hearing loss at standard PTA was linked to uncontrolled nighttime BP [1.81 (1.01-3.24)]. CONCLUSION: PTA was associated with higher nighttime BP, and hearing loss with a riser BP pattern and uncontrolled BP in older hypertensives.


Subject(s)
Blood Pressure Monitoring, Ambulatory , Hearing Loss , Hypertension , Humans , Aged , Male , Female , Cross-Sectional Studies , Hypertension/physiopathology , Hearing Loss/physiopathology , Audiometry, Pure-Tone , Circadian Rhythm/physiology , Blood Pressure/physiology , Aged, 80 and over
11.
Eur J Neurosci ; 59(9): 2373-2390, 2024 May.
Article in English | MEDLINE | ID: mdl-38303554

ABSTRACT

Humans have the remarkable ability to integrate information from different senses, which greatly facilitates the detection, localization and identification of events in the environment. About 466 million people worldwide suffer from hearing loss. Yet, the impact of hearing loss on how the senses work together is rarely investigated. Here, we investigate how a common sensory impairment, asymmetric conductive hearing loss (AHL), alters the way our senses interact by examining human orienting behaviour with normal hearing (NH) and acute AHL. This type of hearing loss disrupts auditory localization. We hypothesized that this creates a conflict between auditory and visual spatial estimates and alters how auditory and visual inputs are integrated to facilitate multisensory spatial perception. We analysed the spatial and temporal properties of saccades to auditory, visual and audiovisual stimuli before and after plugging the right ear of participants. Both spatial and temporal aspects of multisensory integration were affected by AHL. Compared with NH, AHL caused participants to make slow, inaccurate and unprecise saccades towards auditory targets. Surprisingly, increased weight on visual input resulted in accurate audiovisual localization with AHL. This came at a cost: saccade latencies for audiovisual targets increased significantly. The larger the auditory localization errors, the less participants were able to benefit from audiovisual integration in terms of saccade latency. Our results indicate that observers immediately change sensory weights to effectively deal with acute AHL and preserve audiovisual accuracy in a way that cannot be fully explained by statistical models of optimal cue integration.


Subject(s)
Sound Localization , Visual Perception , Humans , Female , Adult , Male , Visual Perception/physiology , Sound Localization/physiology , Young Adult , Saccades/physiology , Auditory Perception/physiology , Hearing Loss/physiopathology , Photic Stimulation/methods , Acoustic Stimulation/methods , Space Perception/physiology
12.
Nat Rev Nephrol ; 20(5): 295-312, 2024 May.
Article in English | MEDLINE | ID: mdl-38287134

ABSTRACT

Hearing loss affects nearly 1.6 billion people and is the third-leading cause of disability worldwide. Chronic kidney disease (CKD) is also a common condition that is associated with adverse clinical outcomes and high health-care costs. From a developmental perspective, the structures responsible for hearing have a common morphogenetic origin with the kidney, and genetic abnormalities that cause familial forms of hearing loss can also lead to kidney disease. On a cellular level, normal kidney and cochlea function both depend on cilial activities at the apical surface, and kidney tubular cells and sensory epithelial cells of the inner ear use similar transport mechanisms to modify luminal fluid. The two organs also share the same collagen IV basement membrane network. Thus, strong developmental and physiological links exist between hearing and kidney function. These theoretical considerations are supported by epidemiological data demonstrating that CKD is associated with a graded and independent excess risk of sensorineural hearing loss. In addition to developmental and physiological links between kidney and cochlear function, hearing loss in patients with CKD may be driven by specific medications or treatments, including haemodialysis. The associations between these two common conditions are not commonly appreciated, yet have important implications for research and clinical practice.


Subject(s)
Renal Insufficiency, Chronic , Humans , Renal Insufficiency, Chronic/physiopathology , Renal Insufficiency, Chronic/complications , Hearing Loss/etiology , Hearing Loss/physiopathology , Hearing Loss, Sensorineural/etiology , Hearing Loss, Sensorineural/physiopathology
13.
JAMA Otolaryngol Head Neck Surg ; 149(7): 571-578, 2023 07 01.
Article in English | MEDLINE | ID: mdl-37166823

ABSTRACT

Importance: Hearing loss is the most important modifiable risk factor for cognitive impairment; however, the association of hearing loss with anatomical and functional connectivity is not fully understood. This association may be elucidated by evaluating the findings of newer imaging technologies. Objectives: To evaluate the association of hearing loss with anatomical and functional connectivity in patients with mild cognitive impairment (MCI) by using multimodal imaging technology. Design, Setting, and Participants: This was a prospective cross-sectional study of patients with MCI under the care of a neurology clinic at the Soonchunhyang University Bucheon Hospital (Republic of Korea) from April to September 2021. Data were analyzed from April 1 to June 30, 2022. Main Outcomes and Measures: Pure tone averages (PTA) and word recognition scores were used to measure hearing acuity. Magnetic resonance imaging (MRI) and positron emission tomography scans of the brain were used to assess functional and anatomical connectivity. Results of diffusion MRI, voxel- and surface-based morphometric imaging, and global brain amyloid standardized uptake ratio were analyzed. Neuroimaging parameters of patients with MCI plus hearing loss were compared with those of patients with MCI and no hearing loss. Correlation analyses among neuroimaging parameters, PTA, and word recognition scores were performed. Results: Of 48 patients with MCI, 30 (62.5%) had hearing loss (PTA >25 dB) and 18 (37.5%) did not (PTA ≤25 dB). Median (IQR) age was 73.5 (69.0-78.0) years in the group with hearing loss and 75.0 (65.0-78.0) years in the group with normal hearing; there were 20 (66.7%) and 14 (77.8%) women in each group, respectively. The group with MCI plus hearing loss demonstrated decreased functional connectivity between the bilateral insular and anterior divisions of the cingulate cortex, and decreased fractional anisotropy in the bilateral fornix, corpus callosum forceps major and tapetum, left parahippocampal cingulum, and left superior thalamic radiation. Fractional anisotropy in the corpus callosum forceps major and bilateral parahippocampal cingulum negatively correlated with the severity of hearing loss shown by PTA testing. The 2 groups were not significantly different in global ß-amyloid uptake, gray matter volume, and cortical thickness. Conclusion and Relevance: The findings of this prospective cross-sectional study suggest that alterations in the salience network may contribute to the neural basis of cognitive impairment associated with hearing loss in patients who are on the Alzheimer disease continuum.


Subject(s)
Alzheimer Disease , Cognitive Dysfunction , Deafness , Hearing Loss , Humans , Female , Aged , Male , Cross-Sectional Studies , Prospective Studies , Neuropsychological Tests , Brain/diagnostic imaging , Magnetic Resonance Imaging/methods , Alzheimer Disease/diagnosis , Hearing Loss/physiopathology , Deafness/physiopathology
14.
J Biol Chem ; 299(5): 104631, 2023 05.
Article in English | MEDLINE | ID: mdl-36963494

ABSTRACT

For decades, sarcomeric myosin heavy chain proteins were assumed to be restricted to striated muscle where they function as molecular motors that contract muscle. However, MYH7b, an evolutionarily ancient member of this myosin family, has been detected in mammalian nonmuscle tissues, and mutations in MYH7b are linked to hereditary hearing loss in compound heterozygous patients. These mutations are the first associated with hearing loss rather than a muscle pathology, and because there are no homologous mutations in other myosin isoforms, their functional effects were unknown. We generated recombinant human MYH7b harboring the D515N or R1651Q hearing loss-associated mutation and studied their effects on motor activity and structural and assembly properties, respectively. The D515N mutation had no effect on steady-state actin-activated ATPase rate or load-dependent detachment kinetics but increased actin sliding velocity because of an increased displacement during the myosin working stroke. Furthermore, we found that the D515N mutation caused an increase in the proportion of myosin heads that occupy the disordered-relaxed state, meaning more myosin heads are available to interact with actin. Although we found no impact of the R1651Q mutation on myosin rod secondary structure or solubility, we observed a striking aggregation phenotype when this mutation was introduced into nonmuscle cells. Our results suggest that each mutation independently affects MYH7b function and structure. Together, these results provide the foundation for further study of a role for MYH7b outside the sarcomere.


Subject(s)
Hearing Loss , Myosin Heavy Chains , Animals , Humans , Mice , Actins/metabolism , Cell Line , Chlorocebus aethiops , COS Cells , Hearing Loss/genetics , Hearing Loss/physiopathology , Kinetics , Mutation , Myosin Heavy Chains/genetics , Myosin Heavy Chains/metabolism , Protein Aggregates/genetics , Recombinant Proteins/genetics , Recombinant Proteins/metabolism
15.
Trends Hear ; 27: 23312165231151468, 2023.
Article in English | MEDLINE | ID: mdl-36946195

ABSTRACT

Electroencephalography could serve as an objective tool to evaluate hearing aid benefit in infants who are developmentally unable to participate in hearing tests. We investigated whether speech-evoked envelope following responses (EFRs), a type of electroencephalography-based measure, could predict improved audibility with the use of a hearing aid in children with mild-to-severe permanent, mainly sensorineural, hearing loss. In 18 children, EFRs were elicited by six male-spoken band-limited phonemic stimuli--the first formants of /u/ and /i/, the second and higher formants of /u/ and /i/, and the fricatives /s/ and /∫/--presented together as /su∫i/. EFRs were recorded between the vertex and nape, when /su∫i/ was presented at 55, 65, and 75 dB SPL using insert earphones in unaided conditions and individually fit hearing aids in aided conditions. EFR amplitude and detectability improved with the use of a hearing aid, and the degree of improvement in EFR amplitude was dependent on the extent of change in behavioral thresholds between unaided and aided conditions. EFR detectability was primarily influenced by audibility; higher sensation level stimuli had an increased probability of detection. Overall EFR sensitivity in predicting audibility was significantly higher in aided (82.1%) than unaided conditions (66.5%) and did not vary as a function of stimulus or frequency. EFR specificity in ascertaining inaudibility was 90.8%. Aided improvement in EFR detectability was a significant predictor of hearing aid-facilitated change in speech discrimination accuracy. Results suggest that speech-evoked EFRs could be a useful objective tool in predicting hearing aid benefit in children with hearing loss.


Subject(s)
Hearing Aids , Hearing Loss , Speech Perception , Adolescent , Child , Female , Humans , Male , Evoked Potentials, Auditory , Hearing Loss/physiopathology , Hearing Loss/therapy , Speech Perception/physiology , Speech/physiology
16.
J Speech Lang Hear Res ; 65(7): 2709-2719, 2022 07 18.
Article in English | MEDLINE | ID: mdl-35728021

ABSTRACT

PURPOSE: The effect of onset asynchrony on dichotic vowel segregation and identification in normal-hearing (NH) and hearing-impaired (HI) listeners was examined. We hypothesized that fusion would decrease and identification performance would improve with increasing onset asynchrony. Additionally, we hypothesized that HI listeners would gain more benefit from onset asynchrony. METHOD: A total of 18 adult subjects (nine NH, nine HI) participated. Testing included dichotic presentation of synthetic vowels, /i/, /u/, /a/, and /ae/. Vowel pairs were presented with the same or different fundamental frequency (f o; f o = 106.9, 151.2, or 201.8 Hz) across the two ears and one onset asynchrony of 0, 1, 2, 4, 10, or 20 ms throughout a block (one block = 80 runs). Subjects identified the one or two vowels that they perceived on a touchscreen. Subjects were not informed that two vowels were always presented or that there was onset asynchrony. RESULTS: The effect of onset asynchrony on fusion and vowel identification was greatest in both groups when Δf o = 0 Hz. Mean fusion scores across increasing onset asynchronies differed significantly between the two groups with HI listeners exhibiting less fusion across pooled Δf o. There was no significant difference with identification performance. CONCLUSIONS: As onset asynchrony increased, dichotic vowel fusion decreased and identification performance improved. Onset asynchrony exerted a greater effect on fusion and identification of vowels when Δf o = 0, especially in HI listeners. Therefore, the temporal cue promotes segregation in both groups of listeners, especially in HI listeners when the f o cue was unavailable.


Subject(s)
Cues , Hearing Loss , Hearing , Speech Perception , Adult , Hearing/physiology , Hearing Loss/physiopathology , Humans , Speech Perception/physiology
17.
J Speech Lang Hear Res ; 65(6): 2343-2363, 2022 06 08.
Article in English | MEDLINE | ID: mdl-35623338

ABSTRACT

PURPOSE: Growing evidence suggests that fatigue associated with listening difficulties is particularly problematic for children with hearing loss (CHL). However, sensitive, reliable, and valid measures of listening-related fatigue do not exist. To address this gap, this article describes the development, psychometric evaluation, and preliminary validation of a suite of scales designed to assess listening-related fatigue in CHL: the pediatric versions of the Vanderbilt Fatigue Scale (VFS-Peds). METHOD: Test development employed best practices, including operationalizing the construct of listening-related fatigue from the perspective of target respondents (i.e., children, their parents, and teachers). Test items were developed based on input from these groups. Dimensionality was evaluated using exploratory factor analyses (EFAs). Item response theory (IRT) and differential item functioning (DIF) analyses were used to identify high-quality items, which were further evaluated and refined to create the final versions of the VFS-Peds. RESULTS: The VFS-Peds is appropriate for use with children aged 6-17 years and consists of child self-report (VFS-C), parent proxy-report (VFS-P), and teacher proxy-report (VFS-T) scales. EFA of child self-report and teacher proxy data suggested that listening-related fatigue was unidimensional in nature. In contrast, parent data suggested a multidimensional construct, composed of mental (cognitive, social, and emotional) and physical domains. IRT analyses suggested that items were of good quality, with high information and good discriminability. DIF analyses revealed the scales provided a comparable measure of fatigue regardless of the child's gender, age, or hearing status. Test information was acceptable over a wide range of fatigue severities and all scales yielded acceptable reliability and validity. CONCLUSIONS: This article describes the development, psychometric evaluation, and validation of the VFS-Peds. Results suggest that the VFS-Peds provide a sensitive, reliable, and valid measure of listening-related fatigue in children that may be appropriate for clinical use. Such scales could be used to identify those children most affected by listening-related fatigue, and given their apparent sensitivity, the scales may also be useful for examining the effectiveness of potential interventions targeting listening-related fatigue in children. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.19836154.


Subject(s)
Auditory Perception , Hearing Loss , Mental Fatigue , Surveys and Questionnaires , Adolescent , Auditory Perception/physiology , Child , Hearing Loss/physiopathology , Humans , Mental Fatigue/diagnosis , Parents , Proxy , Psychometrics , Reproducibility of Results , School Teachers
18.
Sci Rep ; 12(1): 3083, 2022 02 23.
Article in English | MEDLINE | ID: mdl-35197556

ABSTRACT

Although significant progress has been made in understanding outcomes following cochlear implantation, predicting performance remains a challenge. Duration of hearing loss, age at implantation, and electrode positioning within the cochlea together explain ~ 25% of the variability in speech-perception scores in quiet using the cochlear implant (CI). Electrocochleography (ECochG) responses, prior to implantation, account for 47% of the variance in the same speech-perception measures. No study to date has explored CI performance in noise, a more realistic measure of natural listening. This study aimed to (1) validate ECochG total response (ECochG-TR) as a predictor of performance in quiet and (2) evaluate whether ECochG-TR explained variability in noise performance. Thirty-five adult CI recipients were enrolled with outcomes assessed at 3-months post-implantation. The results confirm previous studies showing a strong correlation of ECochG-TR with speech-perception in quiet (r = 0.77). ECochG-TR independently explained 34% of the variability in noise performance. Multivariate modeling using ECochG-TR and Montreal Cognitive Assessment (MoCA) scores explained 60% of the variability in speech-perception in noise. Thus, ECochG-TR, a measure of the cochlear substrate prior to implantation, is necessary but not sufficient for explaining performance in noise. Rather, a cognitive measure is also needed to improve prediction of noise performance.


Subject(s)
Audiometry, Evoked Response , Cochlear Implantation , Cochlear Implants , Cognition/physiology , Hearing Loss/psychology , Hearing Loss/surgery , Noise , Speech Perception/physiology , Adult , Age Factors , Audiometry , Female , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Humans , Male , Treatment Outcome
19.
Neurosci Lett ; 772: 136493, 2022 02 16.
Article in English | MEDLINE | ID: mdl-35114332

ABSTRACT

Autophagy plays a pathogenic role in neurodegenerative disease. However, the involvement of autophagy in the pathogenesis of age-related hearing loss (ARHL) remains obscure. Naturally aged C57BL/6J mice were used to identify the role of autophagy in ARHL, and rapamycin, a mammalian target of rapamycin (mTOR) inhibitor, was administered for 34 weeks to explore the potential therapeutic effect of rapamycin in ARHL. We found that the number of autophagosomes and the expression of microtubule-associated protein 1 light chain 3B (LC3B) decreased as the mice aged. The expression of autophagy-related (Atg) proteins, including Beclin1 and Atg5, and the ratio of LC3-II/I was reduced in aged mice, while mTOR activity in aged mice gradually increased. Rapamycin improved the auditory brainstem response (ABR) threshold (at 8, 12, and 24 kHz). Further exploration demonstrated that spiral ganglion neuron (SGN) density was enhanced in response to administration of rapamycin. The rate of apoptosis in the basal turn SGNs was decreased, whereas autophagy activity was increased in the experimental group. Meanwhile, mTOR activity in the experimental group was decreased. Our findings indicate that age-related deficiency in autophagy may lead to increased apoptosis of aged SGNs. Rapamycin enhances autophagy of SGNs by inhibiting mTOR activation, resulting in amelioration of ARHL. Therapeutic strategy targeting autophagy may provide a potential approach for treating ARHL.


Subject(s)
Aging/pathology , Autophagy , Hearing Loss/drug therapy , Sirolimus/pharmacology , Spiral Ganglion/drug effects , Aging/metabolism , Animals , Autophagy-Related Protein 5/metabolism , Beclin-1/metabolism , Evoked Potentials, Auditory, Brain Stem , Hearing Loss/metabolism , Hearing Loss/physiopathology , Male , Mice , Mice, Inbred C57BL , Microtubule-Associated Proteins/metabolism , Sirolimus/therapeutic use , Spiral Ganglion/metabolism , Spiral Ganglion/physiopathology , TOR Serine-Threonine Kinases/metabolism
20.
PLoS One ; 17(2): e0263516, 2022.
Article in English | MEDLINE | ID: mdl-35134072

ABSTRACT

The ability to determine a sound's location is critical in everyday life. However, sound source localization is severely compromised for patients with hearing loss who receive bilateral cochlear implants (BiCIs). Several patient factors relate to poorer performance in listeners with BiCIs, associated with auditory deprivation, experience, and age. Critically, characteristic errors are made by patients with BiCIs (e.g., medial responses at lateral target locations), and the relationship between patient factors and the type of errors made by patients has seldom been investigated across individuals. In the present study, several different types of analysis were used to understand localization errors and their relationship with patient-dependent factors (selected based on their robustness of prediction). Binaural hearing experience is required for developing accurate localization skills, auditory deprivation is associated with degradation of the auditory periphery, and aging leads to poorer temporal resolution. Therefore, it was hypothesized that earlier onsets of deafness would be associated with poorer localization acuity and longer periods without BiCI stimulation or older age would lead to greater amounts of variability in localization responses. A novel machine learning approach was introduced to characterize the types of errors made by listeners with BiCIs, making them simple to interpret and generalizable to everyday experience. Sound localization performance was measured in 48 listeners with BiCIs using pink noise trains presented in free-field. Our results suggest that older age at testing and earlier onset of deafness are associated with greater average error, particularly for sound sources near the center of the head, consistent with previous research. The machine learning analysis revealed that variability of localization responses tended to be greater for individuals with earlier compared to later onsets of deafness. These results suggest that early bilateral hearing is essential for best sound source localization outcomes in listeners with BiCIs.


Subject(s)
Hearing Loss, Bilateral/physiopathology , Sound Localization/physiology , Acoustic Stimulation/methods , Adult , Age Factors , Age of Onset , Aged , Aged, 80 and over , Auditory Perception/physiology , Cochlear Implantation/methods , Cochlear Implants/adverse effects , Cues , Deafness/physiopathology , Female , Hearing/physiology , Hearing Loss/physiopathology , Hearing Tests , Humans , Male , Middle Aged , Sound
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