scholarly journals Model-based hearing-restoration strategies for cochlear synaptopathy pathologies

2022 ◽  
Author(s):  
Fotios Drakopoulos ◽  
Viacheslav Vasilkov ◽  
Alejandro Osses Vecchi ◽  
Tijmen Wartenberg ◽  
Sarah Verhulst
Keyword(s):  
Hearing Aids ◽  
Speech Intelligibility ◽  
Noise Exposure ◽  
Young Group ◽  
Outer Hair Cells ◽  
Auditory Signal ◽  
Application Range ◽  
Processing Strategies ◽  
Age Related ◽  
Restoration Strategies

It is well known that ageing and noise exposure are important causes of sensorineural hearing loss, and can result in damage of the outer hair cells or other structures of the inner ear, including synaptic damage to the auditory nerve (AN), i.e., cochlear synaptopathy (CS). Despite the suspected high prevalence of CS among people with self-reported hearing difficulties but seemingly normal hearing, conventional hearing-aid algorithms do not compensate for the functional deficits associated with CS. Here, we present and evaluate a number of auditory signal-processing strategies designed to maximally restore AN coding for listeners with CS pathologies. We evaluated our algorithms in subjects with and without suspected age-related CS to assess whether physiological and behavioural markers associated with CS can be improved. Our data show that after applying our algorithms, envelope-following responses and perceptual amplitude-modulation sensitivity were consistently enhanced in both young and older listeners. Speech intelligibility showed small improvements across participants, with the young group benefitting the most from processed speech. Our proposed hearing-restoration algorithms can be rapidly executed and can thus extend the application range of current hearing aids and hearables, while leaving sound amplification unaffected.

scholarly journals mTOR Signaling in the Inner Ear as Potential Target to Treat Hearing Loss

2021 ◽  
Vol 22 (12) ◽  
pp. 6368
Author(s):  
Maurizio Cortada ◽  
Soledad Levano ◽  
Daniel Bodmer
Keyword(s):  
Hearing Loss ◽  
Cell Survival ◽  
Inner Ear ◽  
Hearing Aids ◽  
Noise Exposure ◽  
Mammalian Target Of Rapamycin ◽  
Mtor Signaling ◽  
Curative Therapy ◽  
Age Related ◽  
Survival Pathways

Hearing loss affects many people worldwide and occurs often as a result of age, ototoxic drugs and/or excessive noise exposure. With a growing number of elderly people, the number of people suffering from hearing loss will also increase in the future. Despite the high number of affected people, for most patients there is no curative therapy for hearing loss and hearing aids or cochlea implants remain the only option. Important treatment approaches for hearing loss include the development of regenerative therapies or the inhibition of cell death/promotion of cell survival pathways. The mammalian target of rapamycin (mTOR) pathway is a central regulator of cell growth, is involved in cell survival, and has been shown to be implicated in many age-related diseases. In the inner ear, mTOR signaling has also started to gain attention recently. In this review, we will emphasize recent discoveries of mTOR signaling in the inner ear and discuss implications for possible treatments for hearing restoration.

scholarly journals Age-related hearing loss pertaining to potassium ion channels in the cochlea and auditory pathway

2020 ◽  
Author(s):  
Barbara Peixoto Pinheiro ◽  
Barbara Vona ◽  
Hubert Löwenheim ◽  
Lukas Rüttiger ◽  
Marlies Knipper ◽  
...  
Keyword(s):  
Hair Cells ◽  
Noise Exposure ◽  
Auditory Pathway ◽  
Surface Expression ◽  
Potassium Ion ◽  
Outer Hair Cells ◽  
Potassium Ion Channels ◽  
Potassium Ion Channel ◽  
Age Related ◽  
Age Related Hearing Loss

AbstractAge-related hearing loss (ARHL) is the most prevalent sensory deficit in the elderly and constitutes the third highest risk factor for dementia. Lifetime noise exposure, genetic predispositions for degeneration, and metabolic stress are assumed to be the major causes of ARHL. Both noise-induced and hereditary progressive hearing have been linked to decreased cell surface expression and impaired conductance of the potassium ion channel KV7.4 (KCNQ4) in outer hair cells, inspiring future therapies to maintain or prevent the decline of potassium ion channel surface expression to reduce ARHL. In concert with KV7.4 in outer hair cells, KV7.1 (KCNQ1) in the stria vascularis, calcium-activated potassium channels BK (KCNMA1) and SK2 (KCNN2) in hair cells and efferent fiber synapses, and KV3.1 (KCNC1) in the spiral ganglia and ascending auditory circuits share an upregulated expression or subcellular targeting during final differentiation at hearing onset. They also share a distinctive fragility for noise exposure and age-dependent shortfalls in energy supply required for sustained surface expression. Here, we review and discuss the possible contribution of select potassium ion channels in the cochlea and auditory pathway to ARHL. We postulate genes, proteins, or modulators that contribute to sustained ion currents or proper surface expressions of potassium channels under challenging conditions as key for future therapies of ARHL.

scholarly journals Supplementary Effect of Choline Alfoscerate on Speech Recognition in Patients With Age-Related Hearing Loss: A Prospective Study in 34 Patients (57 Ears)

2021 ◽  
Vol 13 ◽  
Author(s):  
Gina Na ◽  
Sang Hyun Kwak ◽  
Seung Hyun Jang ◽  
Hye Eun Noh ◽  
Jungghi Kim ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Speech Intelligibility ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Control Group ◽  
Age Related ◽  
Monosyllabic Words ◽  
The Difference ◽  
Age Related Hearing Loss

To investigate the effect of choline alfoscerate (CA) on hearing amplification in patients with age related hearing loss, we performed a prospective case-control observational study from March 2016 to September 2020. We assessed patients with bilateral word recognition score (WRS) <50% using monosyllabic words. The patients were 65–85 years old, without any history of dementia, Alzheimer’s disease, parkinsonism, or depression. After enrollment, all patients started using hearing aids (HA). The CA group received a daily dose of 800 mg CA for 11 months. We performed between-group comparisons of audiological data, including pure tone audiometry, WRS, HA fitting data obtained using real-ear measurement (REM), and the Abbreviated Profile of Hearing Aid benefit scores after treatment. After CA administration, the WRS improved significantly in the CA group (4.2 ± 8.3%), but deteriorated in the control group (−0.6 ± 8.1%, p = 0.035). However, there was no significant between-group difference in the change in pure tone thresholds and aided speech intelligibility index calculated from REM. These findings suggest that the difference in WRS was relevant to central speech understanding rather than peripheral audibility. Therefore, administering oral CA could effectively enrich listening comprehension in older HA users.

Shifts in Maximum Audiovisual Integration with Age

2018 ◽  
Vol 31 (3-4) ◽  
pp. 191-212 ◽  
Author(s):  
Samantha D. Jansen ◽  
Joseph R. Keebler ◽  
Alex Chaparro
Keyword(s):  
Working Memory ◽  
Cognitive Abilities ◽  
Speech Intelligibility ◽  
Visual Cues ◽  
Recognition Performance ◽  
Working Memory Capacity ◽  
Audiovisual Integration ◽  
Auditory Signal ◽  
Age Related ◽  
Audiovisual Processing

Listeners attempting to understand speech in noisy environments rely on visual and auditory processes, typically referred to as audiovisual processing. Noise corrupts the auditory speech signal and listeners naturally leverage visual cues from the talker’s face in an attempt to interpret the degraded auditory signal. Studies of speech intelligibility in noise show that the maximum improvement in speech recognition performance (i.e., maximum visual enhancement or VEmax), derived from seeing an interlocutor’s face, is invariant with age. Several studies have reported that VEmaxis typically associated with a signal-to-noise (SNR) of −12 dB; however, few studies have systematically investigated whether the SNR associated with VEmaxchanges with age. We investigated if VEmaxchanges as a function of age, whether the SNR at VEmaxchanges as a function of age, and what perceptual/cognitive abilities account for or mediate such relationships. We measured VEmaxon a nongeriatric adult sample () ranging in age from 20 to 59 years old. We found that VEmaxwas age-invariant, replicating earlier studies. No perceptual/cognitive measures predicted VEmax, most likely due to limited variance in VEmaxscores. Importantly, we found that the SNR at VEmaxshifts toward higher (quieter) SNR levels with increasing age; however, this relationship is partially mediated by working memory capacity, where those with larger working memory capacities (WMCs) can identify speech under lower (louder) SNR levels than their age equivalents with smaller WMCs. The current study is the first to report that individual differences in WMC partially mediate the age-related shift in SNR at VEmax.

A Family With Autosomal-Dominant Progressive Sensorineural Hearing Loss

1996 ◽  
Vol 5 (1) ◽  
pp. 23-32 ◽  
Author(s):  
Chris Halpin ◽  
Barbara Herrmann ◽  
Margaret Whearty
Keyword(s):  
Speech Production ◽  
Hearing Aids ◽  
Role Models ◽  
Speech Intelligibility ◽  
Large Scale ◽  
Speech Language Pathology ◽  
The Family ◽  
Patient Will ◽  
Language Pathology

The family described in this article provides an unusual opportunity to relate findings from genetic, histological, electrophysiological, psychophysical, and rehabilitative investigation. Although the total number evaluated is large (49), the known, living affected population is smaller (14), and these are spread from age 20 to age 59. As a result, the findings described above are those of a large-scale case study. Clearly, more data will be available through longitudinal study of the individuals documented in the course of this investigation but, given the slow nature of the progression in this disease, such studies will be undertaken after an interval of several years. The general picture presented to the audiologist who must rehabilitate these cases is that of a progressive cochlear degeneration that affects only thresholds at first, and then rapidly diminishes speech intelligibility. The expected result is that, after normal language development, the patient may accept hearing aids well, encouraged by the support of the family. Performance and satisfaction with the hearing aids is good, until the onset of the speech intelligibility loss, at which time the patient will encounter serious difficulties and may reject hearing aids as unhelpful. As the histological and electrophysiological results indicate, however, the eighth nerve remains viable, especially in the younger affected members, and success with cochlear implantation may be expected. Audiologic counseling efforts are aided by the presence of role models and support from the other affected members of the family. Speech-language pathology services were not considered important by the members of this family since their speech production developed normally and has remained very good. Self-correction of speech was supported by hearing aids and cochlear implants (Case 5’s speech production was documented in Perkell, Lane, Svirsky, & Webster, 1992). These patients received genetic counseling and, due to the high penetrance of the disease, exhibited serious concerns regarding future generations and the hope of a cure.

Comparison of In-the-Ear and Over-the-Ear Hearing Aid Fittings

1986 ◽  
Vol 51 (4) ◽  
pp. 362-369 ◽  
Author(s):  
Donna M. Risberg ◽  
Robyn M. Cox
Keyword(s):  
Hearing Aids ◽  
High Frequency ◽  
Comparative Evaluation ◽  
Speech Intelligibility ◽  
Hearing Aid ◽  
Hearing Aid Fitting ◽  
The Difference ◽  
Functional Gain ◽  
The Relationship

A custom in-the-ear (ITE) hearing aid fitting was compared to two over-the-ear (OTE) hearing aid fittings for each of 9 subjects with mild to moderately severe hearing losses. Speech intelligibility via the three instruments was compared using the Speech Intelligibility Rating (SIR) test. The relationship between functional gain and coupler gain was compared for the ITE and the higher rated OTE instruments. The difference in input received at the microphone locations of the two types of hearing aids was measured for 10 different subjects and compared to the functional gain data. It was concluded that (a) for persons with mild to moderately severe hearing losses, appropriately adjusted custom ITE fittings typically yield speech intelligibility that is equal to the better OTE fitting identified in a comparative evaluation; and (b) gain prescriptions for ITE hearing aids should be adjusted to account for the high-frequency emphasis associated with in-the-concha microphone placement.

Performance Improvement of Digital Hearing Aid Systems

2015 ◽  
Vol 1 ◽  
pp. 27
Author(s):  
Isiaka Ajewale Alimi
Keyword(s):  
Noise Reduction ◽  
Hearing Aids ◽  
Digital Signal Processor ◽  
Speech Intelligibility ◽  
Communication Systems ◽  
Hearing Aid ◽  
Spectral Subtraction ◽  
Residual Spectrum ◽  
Digital Hearing Aids ◽  
Digital Hearing Aid

Digital hearing aids addresses the issues of noise and speech intelligibility that is associated with the analogue types. One of the main functions of the digital signal processor (DSP) of digital hearing aid systems is noise reduction which can be achieved by speech enhancement algorithms which in turn improve system performance and flexibility. However, studies have shown that the quality of experience (QoE) with some of the current hearing aids is not up to expectation in a noisy environment due to interfering sound, background noise and reverberation. It is also suggested that noise reduction features of the DSP can be further improved accordingly. Recently, we proposed an adaptive spectral subtraction algorithm to enhance the performance of communication systems and address the issue of associated musical noise generated by the conventional spectral subtraction algorithm. The effectiveness of the algorithm has been confirmed by different objective and subjective evaluations. In this study, an adaptive spectral subtraction algorithm is implemented using the noise-estimation algorithm for highly non-stationary noisy environments instead of the voice activity detection (VAD) employed in our previous work due to its effectiveness. Also, signal to residual spectrum ratio (SR) is implemented in order to control the amplification distortion for speech intelligibility improvement. The results show that the proposed scheme gives comparatively better performance and can be easily employed in digital hearing aid system for improving speech quality and intelligibility.

scholarly journals Zone-specific damage of the olfactory epithelium under protein restriction

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Ayinuer Tuerdi ◽  
Shu Kikuta ◽  
Makoto Kinoshita ◽  
Teru Kamogashira ◽  
Kenji Kondo ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Olfactory Epithelium ◽  
Control Diet ◽  
Intervention Strategy ◽  
Protein Restriction ◽  
Outer Hair Cells ◽  
Basal Cells ◽  
Cell Dynamics ◽  
Age Related ◽  
Positive Effect

AbstractOxidative stress causes tissue damage, affecting age-related pathologies. Protein restriction (PR) provides a powerful intervention strategy for reducing oxidative stress, which may have a positive effect on individual organs. However, it is unknown whether PR intervention influences the olfactory system. Here, we investigated how 10 months of PR could affect the cell dynamics of the olfactory epithelium (OE) in mice. We found that PR reduced age-related loss of outer hair cells in the cochlea, providing preventive effects against age-related hearing loss. In contrast, PR resulted in reduced mature olfactory sensory neurons (OSNs), increased proliferative basal cells, and increased apoptotic OSNs in zone 1 (the only area containing neurons expressing NQO1 [quinone dehydrogenase 1]) of the OE in comparison with animals given a control diet. Substantial oxidative stress occurred in NQO1-positive cells and induced apoptotic OSNs in zone 1. These results indicate that in contrast to the positive effect on the auditory system, PR induces oxidative stress and structurally and functionally negative effects on OSNs in zone 1, which is probably involved in the bioactivation of NQO1.

scholarly journals Integrated Bimodal Fitting for Unilateral CI Users with Residual Contralateral Hearing

2021 ◽  
Vol 11 (2) ◽  
pp. 200-206
Author(s):  
Gennaro Auletta ◽  
Annamaria Franzè ◽  
Carla Laria ◽  
Carmine Piccolo ◽  
Carmine Papa ◽  
...  
Keyword(s):  
Cochlear Implants ◽  
Hearing Aids ◽  
Speech Intelligibility ◽  
Hearing Aid ◽  
Performance Difference ◽  
Test Conditions ◽  
Significant Difference ◽  
Noisy Conditions ◽  
New Formula ◽  
Better Than

Background: The aim of this study was to compare, in users of bimodal cochlear implants, the performance obtained using their own hearing aids (adjusted with the standard NAL-NL1 fitting formula) with the performance using the Phonak Naìda Link Ultra Power hearing aid adjusted with both NAL-NL1 and a new bimodal system (Adaptive Phonak Digital Bimodal (APDB)) developed by Advanced Bionics and Phonak Corporations. Methods: Eleven bimodal users (Naìda CI Q70 + contralateral hearing aid) were enrolled in our study. The users’ own hearing aids were replaced with the Phonak Naìda Link Ultra Power and fitted following the new formula. Speech intelligibility was assessed in quiet and noisy conditions, and comparisons were made with the results obtained with the users’ previous hearing aids and with the Naída Link hearing aids fitted with the NAL-NL1 generic prescription formula. Results: Using Phonak Naìda Link Ultra Power hearing aids with the Adaptive Phonak Digital Bimodal fitting formula, performance was significantly better than that with the users’ own rehabilitation systems, especially in challenging hearing situations for all analyzed subjects. Conclusions: Speech intelligibility tests in quiet settings did not reveal a significant difference in performance between the new fitting formula and NAL-NL1 fittings (using the Naída Link hearing aids), whereas the performance difference between the two fittings was very significant in noisy test conditions.

scholarly journals IGF-1 Haploinsufficiency Causes Age-Related Chronic Cochlear Inflammation and Increases Noise-Induced Hearing Loss

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1686
Author(s):  
Adelaida M. Celaya ◽  
Lourdes Rodríguez-de la Rosa ◽  
Jose M. Bermúdez-Muñoz ◽  
José M. Zubeldia ◽  
Carlos Romá-Mateo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inflammatory Cytokines ◽  
Noise Exposure ◽  
Sensorineural Deafness ◽  
Serum Levels ◽  
Genetic Syndromes ◽  
Expression Ratio ◽  
Postnatal Maturation ◽  
Age Related ◽  
Underlying Mechanisms

Insulin-like growth factor 1 (IGF-1) deficiency is an ultrarare syndromic human sensorineural deafness. Accordingly, IGF-1 is essential for the postnatal maturation of the cochlea and the correct wiring of hearing in mice. Less severe decreases in human IGF-1 levels have been associated with other hearing loss rare genetic syndromes, as well as with age-related hearing loss (ARHL). However, the underlying mechanisms linking IGF-1 haploinsufficiency with auditory pathology and ARHL have not been studied. Igf1-heterozygous mice express less Igf1 transcription and have 40% lower IGF-1 serum levels than wild-type mice. Along with ageing, IGF-1 levels decreased concomitantly with the increased expression of inflammatory cytokines, Tgfb1 and Il1b, but there was no associated hearing loss. However, noise exposure of these mice caused increased injury to sensory hair cells and irreversible hearing loss. Concomitantly, there was a significant alteration in the expression ratio of pro- and anti-inflammatory cytokines in Igf1+/− mice. Unbalanced inflammation led to the activation of the stress kinase JNK and the failure to activate AKT. Our data show that IGF-1 haploinsufficiency causes a chronic subclinical proinflammatory age-associated state and, consequently, greater susceptibility to stressors. This work provides the molecular bases to further understand hearing disorders linked to IGF-1 deficiency.

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