Outer Hair Cell Function Research Articles

Unexplained multi-sensory neuropathy syndrome in young Tanzanian adults.

A unique syndrome affecting young adults of unexplained hearing loss often associated with uncorrectable poor visual acuity and lower extremity numbness is endemic in Dar es Salaam. This study characterized the hearing loss, associated it with other symptoms, and gathered information on potential causes. Forty-seven patients (23 men, 24 women) <40 years old with a symptom consistent with the syndrome, negative syphilis test, and no head injury history were recruited from Muhimbili National Hospital. 18 controls (10 men, 8 women) were recruited from the same neighborhoods as patients. Hearing ability and cochlear outer hair cell function (distortion-product otoacoustic emissions (DPOAEs)) were assessed, as were visual acuity and color vision. Peripheral neuropathy was evaluated using the Michigan Neuropathy Screening Instrument (MNSI), and physical examination. Blood C-reactive protein levels and toenail trace metal concentrations were measured. Environmental exposures were elicited using a questionnaire. Patients with at least two of the following signs were defined as having the syndrome: poor hearing with normal DPOAEs, vision not correctable to better than 20/30, or a MNSI score greater than 4. 29 participants met the case definition. CRP levels did not differ between groups but manganese, cobalt and tin levels were each greater in the cases than controls. No other environmental exposure differences were noted. Toenail manganese, cobalt, and tin levels were higher in those with the syndrome. These metals are potential neurotoxins suggesting a possible environmental origin for this unique and debilitating syndrome.

Unexplained Multi-Sensory Neuropathy Syndrome in Young Tanzanian Adults

Background: A unique syndrome affecting young adults of unexplained hearing loss often associated with uncorrectable poor visual acuity and lower extremity numbness is endemic in Dar es Salaam. This study characterized the hearing loss, associated it with other symptoms, and gathered information on potential causes. Methods: Forty-seven patients (23 men, 24 women) <40 years old with a symptom consistent with the syndrome, negative syphilis test, and no head injury history were recruited from Muhimbili National Hospital. 18 controls (10 men, 8 women) were recruited from the same neighborhoods as patients. Hearing ability and cochlear outer hair cell function (distortion-product otoacoustic emissions (DPOAEs)) were assessed, as were visual acuity and color vision. Peripheral neuropathy was evaluated using the Michigan Neuropathy Screening Instrument (MNSI), and physical examination. Blood C-reactive protein levels and toenail trace metal concentrations were measured. Environmental exposures were elicited using a questionnaire. Patients with at least two of the following signs were defined as having the syndrome: poor hearing with normal DPOAEs, vision not correctable to better than 20/30, or a MNSI score greater than 4. Findings: 29 participants met the case definition. CRP levels did not differ between groups but manganese, cobalt and tin levels were each greater in the cases than controls. No other environmental exposure differences were noted. Interpretation: Toenail manganese, cobalt, and tin levels were higher in those with the syndrome. These metals are potential neurotoxins suggesting a possible environmental origin for this unique and debilitating syndrome. Funding Statement: Hitchcock Foundation at the Dartmouth-Hitchcock Medical Center. Declaration of Interests: The authors have no financial or personal relationships that could inappropriately bias their work. Ethics Approval Statement: The research protocol was approved by the Committee for the Protection of Human Subjects of Dartmouth College and the Research Ethics Committee of Muhimbili University for Health and Allied Sciences. All participants gave written informed consent or parental consent and minor assent.

Peripheral deficits and phase-locking declines in aging adults

Age-related difficulties in speech understanding may arise from a decrease in the neural representation of speech sounds. A loss of outer hair cells or decrease in auditory nerve fibers may lead to a loss of temporal precision that can affect speech clarity. This study's purpose was to evaluate the peripheral contributors to phase-locking strength, a measure of temporal precision, in recordings to a sustained vowel in 30 younger and 30 older listeners with normal to near normal audiometric thresholds. Thresholds were obtained for pure tones and distortion-product otoacoustic emissions (DPOAEs). Auditory brainstem responses (ABRs) were recorded in quiet and in three levels of continuous white noise (+30, +20, and +10 dB SNR). Absolute amplitudes and latencies of Wave I in quiet and of Wave V across presentation conditions, in addition to the slope of Wave V amplitude and latency changes in noise, were calculated from these recordings. Frequency-following responses (FFRs) were recorded to synthesized /ba/ syllables of two durations, 170 and 260 ms, to determine whether age-related phase-locking deficits are more pronounced for stimuli that are sustained for longer durations. Phase locking was calculated for the early and late regions of the steady-state vowel for both syllables. Group differences were found for nearly every measure except for the slopes of Wave V latency and amplitude changes in noise. We found that outer hair cell function (DPOAEs) contributed to the variance in phase locking. However, the ABR and FFR differences were present after covarying for DPOAEs, suggesting the existence of temporal processing deficits in older listeners that are somewhat independent of outer hair cell function.

Melatonin reduces radiation damage in inner ear.

The purpose of this study was to use a murine model to determine if melatonin can protect the inner ear from radiation-induced damage. A total of 81 4-week-old Balb/c mice were randomly divided into five groups: control group; 50 mg/kg melatonin group; 5 mg/kg melatonin+radiotherapy group; 50 mg/kg melatonin+radiotherapy group; radiotherapy group. The radiotherapy groups received 16 Gy irradiation and melatonin was administered by intraperitoneal injection 30 min before radiotherapy. On days 3 and 7 after irradiation the function of outer hair cells was determined by auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAEs) testing, pathological changes of inner ear cells were observed by light microscopy, and the expression of prestin mRNA was determined. ABR thresholds were increased and wave I latencies were extended after radiotherapy; however, the increases were lower in the groups that received melatonin (P < 0.05). DPOAEs showed radiotherapy-induced hearing loss at 8–12 kHz, and hearing loss was greater on day 7 than day 3. However, hearing loss was less in the melatonin groups (P < 0.05). Histopathological examination showed irradiation resulted in breaks and distortion of the cochlear basement membrane, disruption of the stria vascularis, and swelling of outer hair cells. Melatonin reduced these changes. Radiotherapy upregulated prestin mRNA expression. Radiotherapy-induced upregulation of prestin was decreased in the melatonin groups (P < 0.05), and the decrease was greater in the 50 mg/kg melatonin group (P < 0.05). Melatonin protects against radiation-induced cochlear damage by reducing damage to outer hair cells.

Ototoxicity in cystic fibrosis patients receiving intravenous tobramycin for acute pulmonary exacerbation: Ototoxicity following tobramycin treatment

Aminoglycosides are commonly used to treat infections in CF patients and are highly ototoxic. The incidence of tobramycin-induced hearing loss, tinnitus, vertigo or dizziness (ototoxicity) varies widely from 0 to 56% secondary to variation in patient enrollment, dosing, audiometry, and ototoxic criteria. The aim of this study is to determine the incidence of ototoxicity after one course of once-daily IV tobramycin in CF patients.Adult CF patients with acute pulmonary exacerbations were enrolled on IV tobramycin (10 mg/kg/d, ≥10 days). Pure-tone audiometry was performed for standard and extended high frequencies in the sensitive range for ototoxicity (SRO). American-Speech-Language-Hearing-Association cochleotoxicity criteria were applied. Distortion product otoacoustic emissions (DPOAE) and the words-in-noise-test (WINT) were assessed. Tinnitus Functional Index (TFI) and Vertigo Symptoms Scale (VSS) were used.Eighteen CF patients, mean age 31.1 (18–59), were enrolled. The incidence of cochleotoxic change from baseline at 2 and 4 weeks post-treatment was 89% and 93%. For DPOAE, a measure of outer hair-cell function, the incidence of ≥5 dB decrease was 82% and 80%. For WINT, a measure of word recognition, the incidence of ≥10% decrease was 17% and 40%. For TFI, the incidence of ≥10pt increase was 12% and 8%, and for VSS, the incidence of ≥6pt increase was 0% and 8%.One course of IV tobramycin was sufficient to cause hearing loss and other ototoxic symptoms four weeks after treatment ended. Audiometric measures were more sensitive to ototoxic change than TFI & VSS. Age and duration of tobramycin treatment were not obvious factors for predicting ototoxicity.

Single and Dual Vector Gene Therapy with AAV9-PHP.B Rescues Hearing in Tmc1 Mutant Mice

AAV-mediated gene therapy is a promising approach for treating genetic hearing loss. Replacement or editing of the Tmc1 gene, encoding hair cell mechanosensory ion channels, is effective for hearing restoration in mice with some limitations. Efficient rescue of outer hair cell function and lack of hearing recovery with later-stage treatment remain issues to be solved. Exogenous genes delivered with the adeno-associated virus (AAV)9-PHP.B capsid via the utricle transduce both inner and outer hair cells of the mouse cochlea with high efficacy. Here, we demonstrate that AAV9-PHP.B gene therapy can promote hair cell survival and successfully rescues hearing in three distinct mouse models of hearing loss. Tmc1 replacement with AAV9-PHP.B in a Tmc1 knockout mouse rescues hearing and promotes hair cell survival with equal efficacy in inner and outer hair cells. The same treatment in a recessive Tmc1 hearing-loss model, Baringo, partially recovers hearing even with later-stage treatment. Finally, dual delivery of Streptococcus pyogenes Cas9 (SpCas9) and guide RNA (gRNA) in separate AAV9-PHP.B vectors selectively disrupts a dominant Tmc1 allele and preserves hearing in Beethoven mice, a model of dominant, progressive hearing loss. Tmc1-targeted gene therapies using single or dual AAV9-PHP.B vectors offer potent and versatile approaches for treating dominant and recessive deafness.

HCN channels in the mammalian cochlea: Expression pattern, subcellular location, and age-dependent changes.

Neuronal diversity in the cochlea is largely determined by ion channels. Among voltage‐gated channels, hyperpolarization‐activated cyclic nucleotide‐gated (HCN) channels open with hyperpolarization and depolarize the cell until the resting membrane potential. The functions for hearing are not well elucidated and knowledge about localization is controversial. We created a detailed map of subcellular location and co‐expression of all four HCN subunits across different mammalian species including CBA/J, C57Bl/6N, Ly5.1 mice, guinea pigs, cats, and human subjects. We correlated age‐related hearing deterioration in CBA/J and C57Bl/6N with expression levels of HCN1, −2, and −4 in individual auditory neurons from the same cohort. Spatiotemporal expression during murine postnatal development exposed HCN2 and HCN4 involvement in a critical phase of hair cell innervation. The huge diversity of subunit composition, but lack of relevant heteromeric pairing along the perisomatic membrane and axon initial segments, highlighted an active role for auditory neurons. Neuron clusters were found to be the hot spots of HCN1, −2, and −4 immunostaining. HCN channels were also located in afferent and efferent fibers of the sensory epithelium. Age‐related changes on HCN subtype expression were not uniform among mice and could not be directly correlated with audiometric data. The oldest mice groups revealed HCN channel up‐ or downregulation, depending on the mouse strain. The unexpected involvement of HCN channels in outer hair cell function where HCN3 overlaps prestin location emphasized the importance for auditory function. A better understanding may open up new possibilities to tune neuronal responses evoked through electrical stimulation by cochlear implants.

Cochlear microphonic latency predicts outer hair cell function in animal models and clinical populations

As recently reported, electrocochleography recorded in cochlear implant recipients showed reduced amplitude and shorter latency in patients with more severe high-frequency hearing loss compared with those with some residual hearing. As the response is generated primarily by receptor currents in outer hair cells, these variations in amplitude and latency may indicate outer hair cell function after cochlear implantation. We propose that an absence of latency shift when the cochlear microphonic is measured on two adjacent electrodes indicates an absence or dysfunction of outer hair cells between these electrodes. We test this preclinically in noise deafened guinea pigs (2 h of a 124 dB HL, 16–24 kHz narrow-band noise), and clinically, in electrocochleographic recordings made in cochlear implant recipients immediately after implantation. We found that normal hearing guinea pigs showed a progressive increase in latency from basal to apical electrodes. In contrast, guinea pigs with significantly elevated high-frequency hearing thresholds showed no change in cochlear microphonic latency measured on basal electrodes (located approximately at the 16–24 kHz location in the cochlea).. In the clinical cohort, a significant negative correlation existed between cochlear microphonic latency shifts and hearing thresholds at 1-, 2-, & 4 kHz when tested on electrodes located at the relevant cochlear tonotopic place. This reduction in latency shift was such that patients with no measurable hearing also had no detectable latency shift (place assessed by CT scan, r’s of -.70 to -.83). These findings suggest that electrocochleography can be used as a diagnostic tool to detect cochlear regions with functioning hair cells, which may be important for defining cross-over point for electro-acoustic stimulation.

Identifying three otopathologies in humans

ObjectivesHearing-in-noise (HIN) is a primary complaint of both the hearing impaired and the hearing aid user. Both auditory nerve (AN) function and outer hair cell (OHC) function are thought to contribute to HIN, but their relative contributions are still being elucidated. OHCs play a critical role in HIN by fine tuning the motion of the basilar membrane. Further, animal studies suggest that cochlear (auditory) synaptopathy, which is the loss of synaptic contact between hair cells and the AN, may be another cause of HIN difficulty. While there is evidence that cochlear synaptopathy occurs in animal models, there is debate as to whether cochlear synaptopathy is clinically significant in humans, which may be due to disparate methods of measuring noise exposure in humans and our high variability in susceptibility to noise damage. Rather than use self-reported noise exposure to define synaptopathic groups, this paper assumes that the general population exhibits a range of noise exposures and resulting otopathologies and defines cochlear synaptopathy “operationally” as low CAP amplitude accompanied by normal DPOAE levels in persons with low pure tone averages. The first question is whether the standard audiogram detects AN dysfunction and OHC dysfunction? The second question is whether HIN performance is primarily dependent on AN function, OHC function, or both functions? DesignAdult subjects have been recruited to participate in an ongoing study and variables such as age, self-reported gender, pure tone audiometry (0.25–20 kHz), subjective perception of HIN difficulty, Quick Speech-in Noise (QuickSIN) test, 45% time compressed word recognition (WR) in 10% reverberation and WR in the presence of ipsilateral speech-weighted noise have been collected. These variables were correlated with OHC function measured by distortion-product otoacoustic emission (DPOAE) signal to-noise-ratio (SNR), and AN function measured by compound action potential (CAP) peak amplitude and ratio to summating potential measured using electrocochleography. ResultsSynaptopathy, by this operational definition, may be present in as many as 30% of individuals with normal hearing. Persons hearing within normal limits may exhibit HIN difficulties, and persons with hearing within normal limits may exhibit two distinct types of otopathologies undetected by the standard audiogram (a.k.a. hidden hearing loss) namely operational cochlear synaptopathy and OHC dysfunction. AN untuning secondary to OHC dysfunction is a third otopathology that occurs in subjects with a Mild–Moderate sensorineural hearing loss (SNHL). Clinical norms for each of these otopathologies are presented. Finally, the data show that operational cochlear synaptopathy does not correlate with HIN dysfunction. Rather, HIN performance is primarily governed by OHC function, while AN untuning also plays a lesser but statistically significant role. ConclusionsThe results of this study suggest the following: (1) persons hearing within normal limits may exhibit HIN difficulties; (2) persons hearing within normal limits may exhibit undetected otopathologies, namely AN dysfunction and OHC dysfunction; (3) AN untuning secondary to OHC dysfunction occurs in subjects with Mild–Moderate SNHL; (4) HIN performance is primarily governed by OHC function rather than AN function.

Outer hair cell functioning in infants with iron deficiency anemia

BackgroundDistortion product otoacoustic emissions (DPOAE) in infants with Iron Deficiency Anemia (IDA) helps in understanding the cochlear status especially the functioning of outer hair cells. ObjectivesTo analyze the presence of DPOAE across frequencies and DP amplitude in infants with and without IDA. MethodDPOAE were recorded on 40 infants with IDA and 40 infants without IDA in the age range of 6–24 months. Cubic DPOAEs (2f1-f2) were measured at six f2 frequencies (1500 Hz, 2000 Hz, 3000 Hz, 4500 Hz, 6000 Hz & 8000 Hz) with primary tone stimulus of intensity L1 equal to 65 dBSPL and L2 equal to 55 dBSPL. Immittance audiometry was performed using 226 Hz probe tone prior to DPOAE recording to ascertain normal middle ear functioning. ResultsDPOAEs were present in all infants with and without IDA across frequencies tested. DP amplitude across the frequencies did not show any statistically significant difference (p < 0.05) between anemic and non-anemic groups. There was no statistically significant difference observed when both the groups were compared in terms of gender and age. ConclusionThe study indicated that reduced iron levels does not have any influence on outer hair cell functioning as there were no changes observed in the DPOAE amplitude levels between infants with IDA and infants without IDA. Iron status does not seem to have a direct effect on outer hair cell functioning in infants. However, fine structure DPOAEs can reveal even more precise changes (if any) that are caused due to iron deficiency.

The cochlear outer hair cell speed paradox

Cochlear outer hair cells (OHCs) are among the fastest known biological motors and are essential for high-frequency hearing in mammals. It is commonly hypothesized that OHCs amplify vibrations in the cochlea through cycle-by-cycle changes in length, but recent data suggest OHCs are low-pass filtered and unable to follow high-frequency signals. The fact that OHCs are required for high-frequency hearing but appear to be throttled by slow electromotility is the "OHC speed paradox." The present report resolves this paradox and reveals origins of ultrafast OHC function and power output in the context of the cochlear load. Results demonstrate that the speed of electromotility reflects how fast the cell can extend against the load, and does not reflect the intrinsic speed of the motor element itself or the nearly instantaneous speed at which the coulomb force is transmitted. OHC power output at auditory frequencies is revealed by emergence of an imaginary nonlinear capacitance reflecting the phase of electrical charge displacement required for the motor to overcome the viscous cochlear load.

Investigating the effects of noise exposure on self-report, behavioral and electrophysiological indices of hearing damage in musicians with normal audiometric thresholds

Musicians are at risk of hearing loss due to prolonged noise exposure, but they may also be at risk of early sub-clinical hearing damage, such as cochlear synaptopathy. In the current study, we investigated the effects of noise exposure on electrophysiological, behavioral and self-report correlates of hearing damage in young adult (age range = 18–27 years) musicians and non-musicians with normal audiometric thresholds. Early-career musicians (n = 76) and non-musicians (n = 47) completed a test battery including the Noise Exposure Structured Interview, pure-tone audiometry (PTA; 0.25–8 kHz), extended high-frequency (EHF; 12 and 16 kHz) thresholds, otoacoustic emissions (OAEs), auditory brainstem responses (ABRs), speech perception in noise (SPiN), and self-reported tinnitus, hyperacusis and hearing in noise difficulties. Total lifetime noise exposure was similar between musicians and non-musicians, the majority of which could be accounted for by recreational activities. Musicians showed significantly greater ABR wave I/V ratios than non-musicians and were also more likely to report experience of - and/or more severe - tinnitus, hyperacusis and hearing in noise difficulties, irrespective of noise exposure. A secondary analysis revealed that individuals with the highest levels of noise exposure had reduced outer hair cell function compared to individuals with the lowest levels of noise exposure, as measured by OAEs. OAE level was also related to PTA and EHF thresholds. High levels of noise exposure were also associated with a significant increase in ABR wave V latency, but only for males, and a higher prevalence and severity of hyperacusis. These findings suggest that there may be sub-clinical effects of noise exposure on various hearing metrics even at a relatively young age, but do not support a link between lifetime noise exposure and proxy measures of cochlear synaptopathy such as ABR wave amplitudes and SPiN. Closely monitoring OAEs, PTA and EHF thresholds when conventional PTA is within the clinically ‘normal’ range could provide a useful early metric of noise-induced hearing damage. This may be particularly relevant to early-career musicians as they progress through a period of intensive musical training, and thus interventions to protect hearing longevity may be vital.

A Preliminary Prototype High-Speed Feedback Control of an Artificial Cochlear Sensory Epithelium Mimicking Function of Outer Hair Cells.

A novel feedback control technique for the local oscillation amplitude in an artificial cochlear sensory epithelium that mimics the functions of the outer hair cells in the cochlea is successfully developed and can be implemented with a control time on the order of hundreds of milliseconds. The prototype artificial cochlear sensory epithelium was improved from that developed in our previous study to enable the instantaneous determination of the local resonance position based on the electrical output from a bimorph piezoelectric membrane. The device contains local patterned electrodes deposited with micro electro mechanical system (MEMS) technology that is used to detect the electrical output and oscillate the device by applying local electrical stimuli. The main feature of the present feedback control system is the principle that the resonance position is recognized by simultaneously measuring the local electrical outputs of all of the electrodes and comparing their magnitudes, which drastically reduces the feedback control time. In this way, it takes 0.8 s to control the local oscillation of the device, representing the speed of control with the order of one hundred times relative to that in the previous study using the mechanical automatic stage to scan the oscillation amplitude at each electrode. Furthermore, the intrinsic difficulties in the experiment such as the electrical measurement against the electromagnetic noise, adhesion of materials, and fatigue failure mechanism of the oscillation system are also shown and discussed in detail based on the many scientific aspects. The basic knowledge of the MEMS fabrication and the experimental measurement would provide useful suggestions for future research. The proposed preliminary prototype high-speed feedback control can aid in the future development of fully implantable cochlear implants with a wider dynamic range.

Investigation of hearing and outer hair cell function of the cochlea in patients with vitiligo.

Vitiligo is a systemic disease that affects not only the skin but also other areas that contain melanocytes, such as the inner ear. Studies of the effect of vitiligo on hearing loss have shown inconsistent results, and it is not clear which parameters related to the disease affect hearing. The aim of this study was to determine which disease-related parameters trigger damage to cochlear melanocytes in vitiligo patients and the effect of vitiligo on the outer hair cells and hearing. Thirty-one vitiligo patients and 40 sex- and age-matched healthy volunteers were evaluated. The conventional pure tone audiometry (C-PTA) test and the distortion product otoacoustic emission (DPOAE) test were performed. There was no significant difference between vitiligo patients and controls in C-PTA test results, but the DPOAE test results were abnormal in vitiligo patients. The duration of the disease was significantly related to hypoacusis. As the duration of the disease increases, cochlear dysfunction may increase. Especially in patients with longer disease duration, otoacoustic emission tests should be performed in addition to conventional audiometry tests for early detection of damage to the outer hair cells.

Familial auditory neuropathy spectrum disorder – A case report

Auditory Neuropathy Spectrum Disorder (ANSD) is a hearing disorder where outer hair cell function inside the cochlea is typical, but inner hair cell and/or the auditory nerve function is disrupted. It is a heterogeneous disorder which can have any congenital or acquired causes.

Update on Auditory Neuropathy/Dyssynchrony in Children

Auditory neuropathy/dyssynchrony (AN/AD) is a form of sensorineural hearing loss characterized by the presence of cochlear outer hair cell (OHC) function and absent or abnormal auditory neural function. This article is meant to update clinicians on best practices for diagnosis and management of AN/AD. Exciting advances in genetics present opportunities for additional evidence to classify AN/AD based on site of lesion, and may lead to additional understanding of the pathophysiology as well as prognosis. Cochlear implantation continues to be a highly effective intervention for managing AN/AD in pediatric patients. AN/AD can be a challenging condition to manage given the heterogeneity of its presentation and variety of options for management. Ultimately, clinicians must tailor treatment to the individual child which requires frequent follow-up and communication with families, educators, and other providers. Further research is needed to fully understand this disorder and advance evidence-based care for these children.

Distinct roles of stereociliary links in the nonlinear sound processing and noise resistance of cochlear outer hair cells

Outer hair cells (OHCs) play an essential role in hearing by acting as a nonlinear amplifier which helps the cochlea detect sounds with high sensitivity and accuracy. This nonlinear sound processing generates distortion products, which can be measured as distortion-product otoacoustic emissions (DPOAEs). The OHC stereocilia that respond to sound vibrations are connected by three kinds of extracellular links: tip links that connect the taller stereocilia to shorter ones and convey force to the mechanoelectrical transduction channels, tectorial membrane-attachment crowns (TM-ACs) that connect the tallest stereocilia to one another and to the overlying TM, and horizontal top connectors (HTCs) that link adjacent stereocilia. While the tip links have been extensively studied, the roles that the other two types of links play in hearing are much less clear, largely because of a lack of suitable animal models. Here, while analyzing genetic combinations of tubby mice, we encountered models missing both HTCs and TM-ACs or HTCs alone. We found that the tubby mutation causes loss of both HTCs and TM-ACs due to a mislocalization of stereocilin, which results in OHC dysfunction leading to severe hearing loss. Intriguingly, the addition of the modifier allele modifier of tubby hearing 1 in tubby mice selectively rescues the TM-ACs but not the HTCs. Hearing is significantly rescued in these mice with robust DPOAE production, indicating an essential role of the TM-ACs but not the HTCs in normal OHC function. In contrast, the HTCs are required for the resistance of hearing to damage caused by noise stress.

Acquired auditory neuropathy spectrum disorder after malaria treated with quinine.

Auditory neuropathy spectrum disorder (ANSD) can cause significant hearing impairment; it occurs when there is intact outer hair cell function in the inner ear, with a dyssynchronous neural response, thought to be due to dysfunction of the inner hair cells (IHCs), the synapse of the IHCs and the auditory nerve, or of the auditory nerve itself. This case report describes the onset of ANSD in a Malawian child after severe malaria treated with quinine. Diagnosis of ANSD was made by confirming the presence of otoacoustic emissions, together with the absence of auditory brainstem response and absent acoustic reflexes.

Transient-evoked otoacoustic emission signals predicting outcomes of acute sensorineural hearing loss in patients with Ménière’s disease

Background: Fluctuating hearing loss is characteristic of Ménière’s disease (MD) during acute episodes. However, no reliable audiometric hallmarks are available for counselling the hearing recovery possibility.Aims/objectives: To find parameters for predicting MD hearing outcomes.Material and methods: We applied machine learning techniques to analyse transient-evoked otoacoustic emission (TEOAE) signals recorded from patients with MD. Thirty unilateral MD patients were recruited prospectively after onset of acute cochleo-vestibular symptoms. Serial TEOAE and pure-tone audiogram (PTA) data were recorded longitudinally. Denoised TEOAE signals were projected onto the three most prominent principal directions through a linear transformation. Binary classification was performed using a support vector machine (SVM). TEOAE signal parameters, including signal energy and group delay, were compared between improved (PTA improvement: ≥15 dB) and nonimproved groups using Welch’s t-test.Results: Signal energy did not differ (p = .64) but a significant difference in 1-kHz (p = .045) group delay was recorded between improved and nonimproved groups. The SVM achieved a cross-validated accuracy of >80% in predicting hearing outcomes.Conclusions and significance: This study revealed that baseline TEOAE parameters obtained during acute MD episodes, when processed through machine learning technology, may provide information on outer hair cell function to predict hearing recovery.

Neurodynamics analysis of cochlear hair cell activity

There have been many studies on the effect of cochlea basal membrane movement on the resolution of different frequencies and intensities. However, these studies did not take into account the influence of power and energy consumption of the hair cells in the process of the electromotility movement, as well as the neurodynamic mechanism that produced this effect. This makes previous studies unable to fully clarify the function of outer hair cells (OHCs) and the mechanism of sound amplification. To this end, we introduce the gate conductance characteristics of the hair cells in the mechanical process of increasing frequency selectivity. The research finds that the low attenuation of OHCs membrane potential and the high gain in OHC power and energy consumption caused that OHC amplification is driven by electromotility. The research results show that the amplification of the OHCs is driven by low attenuation of membrane potential and high gain of power and energy consumption. This conclusion profoundly reveals the physiological mechanism of the electromotility movement.