Stimulation Of Auditory Nerve Research Articles

Understanding the impact of modiolus porosity on stimulation of spiral ganglion neurons by cochlear implants

Moderate-to-profound sensorineural hearing loss in humans is treatable by electrically stimulating the auditory nerve (AN) with a cochlear implant (CI). In the cochlea, the modiolus presents a porous bony interface between the CI electrode and the AN. New bone growth caused by the presence of the CI electrode or neural degeneration inflicted by ageing or otological diseases might change the effective porosity of the modiolus and, thereby, alter its electrical material properties. Using a volume conductor description of the cochlea, with the aid of a ‘mapped conductivity’ method and an ad-hoc ‘regionally kinetic’ equation system, we show that even a slight variation in modiolus porosity or pore distribution can disproportionately affect AN stimulation. Hence, because of porosity changes, an inconsistent CI performance might occur if neural degeneration or new bone growth progress after implantation. Appropriate electrical material properties in accordance with modiolar morphology and pathology should be considered in patient-specific studies. The present first-of-its-kind in-silico study advocates for contextual experimental studies to further explore the utility of modiolus porous morphology in optimising the CI outcome.

Psychophysical and Electrophysiological Parameters of the Auditory Analyzer as Indicators of the Effectiveness of Cochlear Implantation in Children with Bilateral Deafness

Background. The results of rehabilitation of patients with deafness after cochlear implantation (CI) depends on many factors related on both the individual characteristics of the patient and the technical parameters of auditory nerve stimulation — fitting of the CI system processor. Article presents the results of a study of the relationship between psychophysical (subjective) auditory sensations and electrophysiological (objective) responses of the structures of the auditory analyzer to electrical stimulation. Aims — to evaluate the relationship between the electrophysiological responses of the auditory analyzer and the psychophysical parameters of the thresholds of sound perception and speech intelligibility in patients with deafness after CI. Methods. The study involved 88 patients with IV bilateral sensorineural hearing loss, borderline with deafness, aged 6 to 17 years 11 months, among whom 54.5% (n = 48) of girls and 45.5% (n = 40) of boys. Of the patients included in the follow-up, 40% (n = 35) underwent unilateral CI; 60% (n = 53) of children underwent bilateral sequential CI. In our study, slightly more than half of the patients (51.1%) had experience using the CI system for more than 5 years. At the first stage of the study, we studied the relationship between sound perception thresholds (tonal audiometry in a free field) and registered ASSR (auditory steady state response) thresholds in patients with deafness receiving rehabilitation after CI surgery. The relationship between behavioral and electrophysiological (objective) thresholds was assessed by comparing the results of tonal threshold audiometry and the responses received during ASSR registration to acoustic stimulation in a free field. At the second stage, patients with indications for processor fitting (decreased speech intelligibility, uncomfortable sensations) underwent speech audiometry, evoked compound action potentials (ECAP) registration, according to the results of which the parameters of the CI processor were changed. Results. Research data showed the effectiveness of the approach of including the ECAP registration test, followed by the creation of a fitting card and correction of the level of auditory nerve stimulation under the control of speech audiometry in the protocol of fitting the CI processor. Conclusions. The results of the conducted studies allow us to recommend the use of the developed method of objective recording of sound perception thresholds in patients with CI as one of the indicators of the effectiveness of CI in the early period of auditory-speech rehabilitation in patients with undeveloped speech skills and when it is impossible to conduct objective tests accompanied by supra-threshold stimulation of the auditory nerve.

Neuromodulation for Atrial Fibrillation Control.

Trigger and functional substrate are related to the tone of autonomic nervous system, and the role of the autonomic nerve is more significant in paroxysmal atrial fibrillation (AF) compared to non-paroxysmal AF. We have several options for neuromodulation to help to manage patients with AF. Neuromodulation targets can be divided into efferent and afferent pathways. On the efferent side, block would be an intuitive approach. However, permanent block is hard to achieve due to completeness of the procedure and reinnervation issues. Temporary block such as botulinum toxin injection into ganglionated plexi would be a possible option for post-cardiac surgery AF. Low-level subthreshold stimulation could also prevent AF, but the invasiveness of the procedure is the barrier for the general use. On the afferent side, block is also an option. Various renal denervation approaches are currently under investigation. Auditory vagus nerve stimulation is one of the representative low-level afferent stimulation methods. This technique is noninvasive and easy to apply, so it has the potential to be widely utilized if its efficacy is confirmed.

Potential uses of auditory nerve stimulation to modulate immune responses in the inner ear and auditory brainstem.

Bioelectronic medicine uses electrical stimulation of the nervous system to improve health outcomes throughout the body primarily by regulating immune responses. This concept, however, has yet to be applied systematically to the auditory system. There is growing interest in how cochlear damage and associated neuroinflammation may contribute to hearing loss. In conjunction with recent findings, we propose here a new perspective, which could be applied alongside advancing technologies, to use auditory nerve (AN) stimulation to modulate immune responses in hearing health disorders and following surgeries for auditory implants. In this article we will: (1) review the mechanisms of inflammation in the auditory system in relation to various forms of hearing loss, (2) explore nerve stimulation to reduce inflammation throughout the body and how similar neural-immune circuits likely exist in the auditory system (3) summarize current methods for stimulating the auditory system, particularly the AN, and (4) propose future directions to use bioelectronic medicine to ameliorate harmful immune responses in the inner ear and auditory brainstem to treat refractory conditions. We will illustrate how current knowledge from bioelectronic medicine can be applied to AN stimulation to resolve inflammation associated with implantation and disease. Further, we suggest the necessary steps to get discoveries in this emerging field from bench to bedside. Our vision is a future for AN stimulation that includes additional protocols as well as advances in devices to target and engage neural-immune circuitry for therapeutic benefits.

Cochlear Implant Insertion Routes and Intra-operative Electrophysiological Measurements: A Retrospective Analysis at a Tertiary Care Centre.

Cochlear implant surgeries are performed by different surgical routes, Round window (RW), Extended round window (Ext RW) and Cochleostomy (C). Optimum intracochlear electrode placement is important to achieve a successful outcome. Intra-operative electrophysiological testing (Impedance and ECAP) is crucial to assess the device function and appropriate electrode placement. The variability of portal of electrode insertion might affect the neural response and its characteristics. To compare the intraoperative electrophysiological measurements (Impedance and ECAP) for possible differences on auditory nerve stimulation across the three electrode insertion routes. This is a retrospective data analysis of 47 cochlear implant recipients at a tertiary care centre in Central India over a period of 4 years. They were broadly divided into two groups depending on the electrode design as full banded and half banded. Intraoperative impedances were recorded for different modes of stimulation and ECAP measured at E5, E10, E15, E20 electrodes in both the groups across the three insertion routes. In the half-banded group, the impedance values in different modes CG, MP1, MP2, MP1 + 2 did not differ significantly among the surgical routes at all four electrodes. While in the full banded group, for CG mode impedance values at E5 differ significantly. Rest for other modes in full banded group, there was no statistically significant difference across the three routes. The present study supports that there is no statistically significant difference in the intraoperative impedance and ECAP measurements in both, the full-banded and half- banded electrodes across the three surgical routes implying that, all the three surgical approaches provide equal stimulation of the auditory nerve. The CI surgeon can select the electrode insertion portal based on the surgical anatomy, the implant type and individual preferences.

Clinical Feasibility and Familiarization Effects of Device Delay Mismatch Compensation in Bimodal CI/HA Users.

Subjects utilizing a cochlear implant (CI) in one ear and a hearing aid (HA) on the contralateral ear suffer from mismatches in stimulation timing due to different processing latencies of both devices. This device delay mismatch leads to a temporal mismatch in auditory nerve stimulation. Compensating for this auditory nerve stimulation mismatch by compensating for the device delay mismatch can significantly improve sound source localization accuracy. One CI manufacturer has already implemented the possibility of mismatch compensation in its current fitting software. This study investigated if this fitting parameter can be readily used in clinical settings and determined the effects of familiarization to a compensated device delay mismatch over a period of 3-4 weeks. Sound localization accuracy and speech understanding in noise were measured in eleven bimodal CI/HA users, with and without a compensation of the device delay mismatch. The results showed that sound localization bias improved to 0°, implying that the localization bias towards the CI was eliminated when the device delay mismatch was compensated. The RMS error was improved by 18% with this improvement not reaching statistical significance. The effects were acute and did not further improve after 3 weeks of familiarization. For the speech tests, spatial release from masking did not improve with a compensated mismatch. The results show that this fitting parameter can be readily used by clinicians to improve sound localization ability in bimodal users. Further, our findings suggest that subjects with poor sound localization ability benefit the most from the device delay mismatch compensation.

Using time difference analysis algorithms to measure the response time of rat auditory cortex neurons to auditory nerve stimulation

Measurement of the information flow along the ascending auditory pathway from the periphery to the auditory cortex (AC) has been given much attention in neurocomputation. While the neurophysiological mechanisms of the auditory pathway have been well studied, the temporal resolution and relationships among the auditory centers are still under investigation, especially when suffering from acoustic trauma that results in peripheral deficits and neural signal changes in the auditory system. In this study, we measured rat AC neurons and auditory nerve (AN) signals in digital format by using two new algorithms to calculate the neural response time of the AC neurons to electrical stimulation of the AN and quantify the neural information flow in the temporal domain. One algorithm compared time difference of neural spikes directly, which was based on the conventional idea of spike train in neurocomputation. The other employed a modified cross-correlation algorithm. Both algorithms shared the same pre-signal processing of spike selection. The statistical results by the two methods were compared and various parameters in the algorithms and their impact on the accuracy of the results were discussed. To test the effectiveness of the proposed method, the time difference between the AC to AN activities was calculated by both algorithms with raw neural signals collected. The neural signals from the animals were measured before and after noise trauma, and one of the animals received intra-modiolus electrical stimulation (IMES) to stimulate the AN. The results from using the two algorithms were generally consistent, and the biological mechanisms behind the time delay results between AC and AN activities were discussed.

Integrative Treatment for Tinnitus Combining Repeated Facial and Auriculotemporal Nerve Blocks With Stimulation of Auditory and Non-auditory Nerves.

BackgroundTinnitus is a prevalent condition (>10% of the population) affecting the quality of life of 0.5–3% of the population. Although several treatments have been proposed, most of these lack evidence of efficacy in the treatment of chronic tinnitus. Thus, we aimed to evaluate an integrative treatment strategy for subacute and chronic tinnitus.MethodsThis retrospective chart review study included 55 patients with tinnitus (subacute, n = 15; chronic, n = 40) who underwent repeated nerve blocks after stimulation of the trigeminal (V) and facial (VII) nerves to modulate the auditory and non-auditory nervous systems via the vestibulocochlear (VIII) cranial nerve pathways. We used a simplified smiley tinnitus-visual analog scale (T-VAS) with scores ranging from 0 to 10 combining the effect of tinnitus loudness, distress, and quality of life as the outcome measure to evaluate the efficacy of our treatment method. Statistical analyses were performed using SPSS (version 18.0, SPSS Inc., Chicago, IL, United States), one-way and two-way analysis of variance.ResultsIn more than 87.5% of patients (14/15 subacute, 35/40 chronic), tinnitus disappeared or had significantly reduced by the end of the treatment. The mean T-VAS score reduced significantly from 7.13 to 0.60 in the subacute group and from 7.73 to 1.53 in the chronic group by the end of treatment (p < 0.05). The benefits were maintained after treatment cessation and at the 1-year follow-up. The average number of treatment procedures was 9.8 ± 3.589 (range, 5–15) in the subacute group and 9.775 ± 3.717 (range, 5–18) in the chronic group.ConclusionOur results show that the proposed integrative approach is highly effective in treating subacute and chronic tinnitus and represents a promising therapeutic approach.

Toward a method of achieving balanced stimulation of bilateral auditory nerves: Evidence from children receiving matched and unmatched bilateral cochlear implants simultaneously

ObjectivesTo identify whether mismatched bilateral cochlear implants compromise balanced stimulation of the two auditory nerves and establish asymmetric hearing in children. MethodsBehavioural and electrophysiological measures were completed in 47 children receiving bilateral CIs in the same surgery (simultaneously): 27 children received a peri‑modiolar N24RE array in one ear and a 422 anti-modiolar array in the other (experimental group) and 20 children received 2 peri‑modiolar arrays (control group). Differences in current levels between the two devices were measured by electrically evoked compound action potentials (ECAPs) at the time of surgery. These data were compared with minimum and maximum comfortably loud levels programmed in each speech processor (T-levels, C-levels, respectively) after 12 months of bilateral CI use. Asymmetries in functional hearing between arrays were measured in open set speech perception testing between 3 to 5 years of CI use. ResultsHigher current levels were required from the anti-modiolar than peri‑modiolar array to evoke balanced interaural ECAP amplitudes (mismatched group: mean ± SD difference: -9.9 ± 22.6; matched group: -0.8 ± 26.5). This difference was larger in the experimental group than control group (t = -2.51; p = 0.016) and remained constant with increases in current level from ECAP threshold to maximum amplitudes (dynamic range) in many but not all children in both groups. T and C-levels were poorly predictive of levels needed to evoke balanced ECAP amplitudes in children with mismatched devices (F(1, 312) = 1.3, p = 0.263). Speech perception scores were more asymmetric between ears in children using bilateral mismatched arrays (mean ± SD: 73.8 ± 16.4 at the peri‑modiolar array; 57.7 ± 26.4 at the anti-modiolar array), compared to children with bilateral matched arrays (right ear: 78.0 ± 10.4; left ear: 74.9 ± 13.5). ConclusionHigher current level requirements at the anti-modiolar array compared to the peri‑modiolar array in children with bilateral mismatched CIs are not fully accounted for in device programming. Mismatched electrodes in children receiving bilateral cochlear implants increases the risk of asymmetric hearing.

Input of sensory, limbic, basal ganglia and pallial/cortical information into the ventral/lateral habenula: Functional principles in anuran amphibians

The habenula – a phylogenetically old brain structure present in all vertebrates – is involved in pain processing, reproductive behaviors, sleep-wake cycles, stress responses, reward, and learning. We performed intra- and extracellular recordings of ventral habenula (VHb) neurons in the isolated brain of anurans and revealed similar cell and response properties to those reported for the lateral habenula of mammals. We identified tonic regular, tonic irregular, rhythmic firing, and silent VHb neurons. Transitions between these firing patterns were observed during spontaneous activity. Electrical stimulation of various brain areas demonstrated VHb input of auditory, optic, limbic, basal ganglia, and pallial information. This resulted in three different response behaviors in VHb neurons: excitation, inhibition, or alternating facilitation and suppression of neuronal activity. Spontaneously changing activity patterns were observed to modulate, reset, or suppress the response behavior of VHb neurons, indicating a gating mechanism. This could be a network status or context dependent selection mechanism for which information are transmitted to task relevant brain areas (i.e., sensory system, limbic system, basal ganglia). Furthermore, alternating facilitation and suppression sequences upon auditory nerve stimulation correlated positively fictive motor activities recorded via the compound potential of the vagal nerve. Stimulation of the auditory nerve or the habenula led to facilitation, suppression, or alternating facilitation and suppression of neuronal activity in putative dopaminergic neurons. Due to complex habenula feedback loops with basal ganglia, limbic, and sensory systems, the habenula involvement in a variety of functions might therefore be explained by a modulatory effect on a task-relevant input stream.

Investigating the Electrical Properties of Different Cochlear Implants.

Aim:This study characterises and compares electrical properties and current spread across four different makes of cochlear implants with differing electrode designs using a 3D-printed artificial cochlear model.Background:Cochlear implants are currently limited by current spread within the cochlea, which causes low spectral resolution of auditory nerve stimulation. Different cochlear implant makes vary in electrode size, shape, number, and configuration. How these differences affect cochlear implant current spread and function is not well known.Method:Each cochlear implant was inserted into a linear cochlear model containing recording electrodes along its length. Biphasic monopolar stimulation of each implant electrode was carried out, and the resultant waveform and transimpedance matrix (TIM) data obtained from the recording electrodes. This was repeated with each implant rotated 180 degrees in the cochlea model to examine the effects of electrode orientation. Impedance spectroscopy was also carried out at the apex, middle, and base of the model.Results:The four cochlear implants displayed similar TIM profiles and waveforms. One hundred eighty degrees rotation of each cochlear implant made little difference to the TIM profiles. Impedance spectroscopy demonstrated broad similarities in amplitude and phase across the implants, but exhibited differences in certain electrical parameters.Conclusion:Implants with different designs demonstrate similar electrical performance, regardless of electrode size and spacing or electrode array dimension. In addition, rotatory maneuvers during cochlear implantation surgery are unlikely to change implant impedance properties.

Effects of different electrodes used in bone-guided extracochlear implants on electrical stimulation of auditory nerves in guinea pigs.

Objective:Conventional cochlear implants provide patients who are deaf with hearing via electrical intracochlear stimulations. Stimulation electrodes are inserted into the cochlea through a cochleostomy or round window membrane (RWM) approach. However, these methods might induce cochlear ossificans and loss of residual hearing by damaging inner ear structures. To avoid an invasive electrode insertion, we developed a novel bone-guided extracochlear implant that stimulated the auditory nerves between the cochlear bones and the RWM to prevent cochlea damage. Power consumption plays an important role in wireless implantable electronic devices. Therefore, we aimed to investigate the effects of different electrodes on the stimulating threshold currents of the auditory nerve and the power consumption of bone-guided extracochlear implants using a commercial stimulator.Materials and Methods:Inert aurum (Au) electrodes were compared with biocompatible platinum (Pt) and iridium oxide (IrOx) electrodes in practical implantable applications. IrOx electrodes were used for their high-charge storage capacity, low impedance, and biocompatibility. The electrodes were fabricated via sputtering and were experimentally characterized with cyclic voltammetry and then examined using in vivo tests.Results:Based on electrical auditory brainstem responses, IrOx electrodes yielded lower acoustic nerve-stimulating threshold currents (132 μA) compared with Au electrodes (204 μA). IrOx electrodes also had a lower acoustic nerve stimulating threshold current (132 μA) compared with Pt electrodes (168 μA).Conclusion:As expected, IrOx electrodes were beneficial in the development of multielectrode bone-guided extracochlear implants, with the lowest acoustic nerve-stimulating threshold and current consumptions compared with Au and Pt electrodes.

Towards the optical cochlear implant: optogenetic approaches for hearing restoration.

Cochlear implants (CIs) are considered the most successful neuroprosthesis as they enable speech comprehension in the majority of half a million CI users suffering from sensorineural hearing loss. By electrically stimulating the auditory nerve, CIs constitute an interface re‐connecting the brain and the auditory scene, providing the patient with information regarding the latter. However, since electric current is hard to focus in conductive environments such as the cochlea, the precision of electrical sound encoding—and thus quality of artificial hearing—is limited. Recently, optogenetic stimulation of the cochlea has been suggested as an alternative approach for hearing restoration. Cochlear optogenetics promises increased spectral selectivity of artificial sound encoding, hence improved hearing, as light can conveniently be confined in space to activate the auditory nerve within smaller tonotopic ranges. In this review, we discuss the latest experimental and technological developments of cochlear optogenetics and outline the remaining challenges on the way to clinical translation.

Excitatory-Inhibitory Synaptic Coupling in Avian Nucleus Magnocellularis.

The activity of neurons is determined by the balance between their excitatory and inhibitory synaptic inputs. Neurons in the avian nucleus magnocellularis (NM) integrate monosynaptic excitatory and polysynaptic inhibitory inputs from the auditory nerve, and transmit phase-locked output to higher auditory centers. The excitatory input is graded tonotopically, such that neurons tuned to higher frequency receive fewer, but larger, axon terminals. However, it remains unknown how the balance between excitatory and inhibitory inputs is determined in NM. We here examined synaptic and spike responses of NM neurons during stimulation of the auditory nerve in thick brain slices of chicken of both sexes, and found that the excitatory-inhibitory balance varied according to tonotopic region, ensuring reliable spike output across frequencies. Auditory nerve stimulation elicited IPSCs in NM neurons regardless of tonotopic region, but the dependence of IPSCs on intensity varied in a systematic way. In neurons tuned to low frequency, IPSCs appeared and increased in parallel with EPSCs with elevation of intensity, which expanded dynamic range by preventing saturation of spike generation. On the other hand, in neurons tuned to higher frequency, IPSCs were smaller than EPSCs and had higher thresholds for activation, thus facilitating high-fidelity transmission. Computer simulation confirmed that these differences in inhibitory input were optimally matched to the patterns of excitatory input, and enabled appropriate level of neuronal output for wide intensity and frequency ranges of sound in the auditory system.SIGNIFICANCE STATEMENT Neurons in nucleus magnocellularis encode timing information of sound across wide intensity ranges by integrating excitatory and inhibitory synaptic inputs from the auditory nerve, but underlying synaptic mechanisms of this integration are not fully understood. We here show that the excitatory-inhibitory relationship was expressed differentially at each tonotopic region; the relationship was linear in neurons tuned to low-frequency, expanding dynamic range by preventing saturation of spike generation; by contrast inhibitory input remained much smaller than excitatory input in neurons tuned to higher frequency, thus ensuring high-fidelity transmission. The tonotopic regulation of excitatory and inhibitory input optimized the output across frequencies and intensities, playing a fundamental role in the timing coding pathway in the auditory system.

D-Stellate Neurons of the Ventral Cochlear Nucleus Decrease in Auditory Nerve-Evoked Activity during Age-Related Hearing Loss.

Age-related hearing loss (ARHL) is associated with weakened inhibition in the central auditory nervous system including the cochlear nucleus. One of the main inhibitory neurons of the cochlear nucleus is the D-stellate neuron, which provides extensive glycinergic inhibition within the local neural network. It remains unclear how physiological activities of D-stellate neurons change during ARHL and what are the underlying mechanisms. Using in vitro whole-cell patch clamp technique, we studied the intrinsic membrane properties of D-stellate neurons, the changes of their firing properties, and the underlying mechanisms in CBA/CaJ mice at the ages of 3–4 months (young), 17–19 months (middle age), and 27–33 months (aged). We found that the intrinsic membrane properties of D-stellate neurons were unchanged among these three age groups. However, these neurons showed decreased firing rate with age in response to sustained auditory nerve stimulation. Further investigation showed that auditory nerve-evoked excitatory postsynaptic currents (EPSCs) were significantly reduced in strength with age. These findings suggest that D-stellate neurons receive weakened synaptic inputs from the auditory nerve and decreased sound driven activity with age, which are expected to reduce the overall inhibition and enhance the central gain in the cochlear nucleus during ARHL.

Laser stimulation of auditory nerves through a tympanic membrane

Laser stimulation of auditory nerves through a tympanic membrane

Chronic Deafness Degrades Temporal Acuity in the Electrically Stimulated Auditory Pathway

Electrical stimulation of the auditory nerve with a penetrating intraneural (IN) electrode in acutely deafened cats produces much more restricted spread of excitation than is obtained in that preparation with a conventional cochlear implant (CI) as reported by Middlebrooks and Snyder (J Assoc Res Otolaryngol 8:258–279, 2007). That suggests that a future auditory prosthesis employing IN stimulation might offer human patients greater frequency selectivity than is available with a present-day CI. Nevertheless, it is a concern that the electrical field produced by an IN electrode might be too restricted to produce adequate stimulation of the partially depopulated auditory nerve of a deaf patient. We evaluated this by testing responses to IN and CI stimulation in adult-deafened cats. Activation of the auditory pathway was monitored by recording from the central nucleus of the inferior colliculus (ICC). Cats deaf for 153–277 days exhibited a ~ 30 % loss of auditory nerve fibers compared to cats deaf for < 18 h. Contrary to our concern, measures of thresholds and dynamic ranges showed no significant deafness-related impairment of excitation by IN or CN stimulation. Surprisingly, however, temporal acuity decreased dramatically in these adult-deafened cats, as demonstrated by a marked decrease in the maximum rate of electrical cochlear stimulation to which ICC neurons synchronized to IN or CI stimulation. For instance, half of ICC neurons synchronized to IN stimulation up to 203 pulses per second (pps) in acute deafness, whereas that number dropped to 79 pps for chronic deafness. Such a loss of temporal acuity might contribute to the poor sensitivity to temporal fine structure that has been reported in human CI users. Seemingly, the degraded temporal acuity that we observed in cats was even worse than the fine-structure sensitivity of human CI users, suggesting that most patients experience some improvement of temporal acuity resulting from restoration of patterned auditory nerve stimulation by a CI.

TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad.

BackgroundSensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized.MethodsA family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD. Whole‐exome sequencing was performed to identify coding sequence variants shared by both family members, and screened against genes relevant to hearing loss and variants known to be associated with SNHL and ANSD.ResultsBoth family members are successful cochlear implant users, demonstrating effective auditory nerve stimulation with their devices. Genetic analyses revealed a mutation (rs35725509) in the TMTC2 gene, which has been reported previously as a likely genetic cause of SNHL in another family of Northern European descent.ConclusionThis study represents the first confirmation of the rs35725509 variant in an independent family as a likely cause for the complex hearing loss phenotype (SNHL and ANSD) observed in this family dyad.

Radiate and Planar Multipolar Neurons of the Mouse Anteroventral Cochlear Nucleus: Intrinsic Excitability and Characterization of their Auditory Nerve Input.

Radiate and planar neurons are the two major types of multipolar neurons in the ventral cochlear nucleus (VCN). Both cell types receive monosynaptic excitatory synaptic inputs from the auditory nerve, but have different responses to sound and project to different target regions and cells. Although the intrinsic physiology and synaptic inputs to planar neurons have been previously characterized, the radiate neurons are less common and have not been as well studied. We studied both types of multipolar neurons and characterized their properties including intrinsic excitability, synaptic dynamics of their auditory nerve inputs, as well as their neural firing properties to auditory nerve stimulation. Radiate neurons had a faster member time constant and higher threshold current to fire spikes than planar neurons, but the maximal firing rate is the same for both cell types upon large current injections. Compared to planar neurons, radiate neurons showed spontaneous postsynaptic currents with smaller size, and slower but variable kinetics. Auditory nerve stimulation progressively recruited synaptic inputs that were smaller and slower in radiate neurons, over a broader range of stimulus strength. Synaptic inputs to radiate neurons showed less depression than planar neurons during low rates of repetitive activity, but the synaptic depression at higher rates was similar between two cell types. However, due to the slow kinetics of the synaptic inputs, synaptic transmission in radiate neurons showed prominent temporal summation that contributed to greater synaptic depolarization and a higher firing rate for repetitive auditory nerve stimulation at high rates. Taken together, these results show that radiate multipolar neurons integrate a large number of weak synaptic inputs over a broad dynamic range, and have intrinsic and synaptic properties that are distinct from planar multipolar neurons. These properties enable radiate neurons to generate powerful inhibitory inputs to target neurons during high levels of afferent activity. Such robust inhibition is expected to dynamically modulate the excitability of many cell types in the cochlear nuclear complex.

Comparison of Single-Channel and Multi-Channel Cochlear Implants in Terms of Encoding Strategy and Speech Understanding: A Systematic Review

Introduction: The term channel in cochlear implant systems refers to the number of stimulation sites within the inner ear or cochlea that determines the range of frequencies or pitches. Unlike single-channel cochlear implant, a multi-channel cochlear implant system divides the incoming signal into various frequency bands, and then, transmits it to various stimulation areas within the inner ear. Having more stimulation sites entails two main goals: 1- As the auditory nerve fibers in the cochlea are tonotopically organized, higher number of electrodes leads to better frequency separation; 2- The areas in the cochlea with inappropriate or no response to electrical stimulation will be avoided in the programming of the device, and the components of the processed input frequency will accompany the stimulation. The goal of the present study was to compare the frequency encoding strategy and its effect on speech understanding of the recipients of single-channel and multi-channel cochlear implant systems. Materials and Methods: Published researches were identified by reviewing scientific databases (PubMed, Science Direct, and Google Scholar) from 1965 to 2016 using relevant keywords. The researches were selected based on the inclusion and exclusion criteria. Results: Single-channel cochlear implants encode the frequency based on the rate of firing of electrical impulses. Multi-channel cochlear implants ‎use the spatial strategy theory for frequency encoding, wherein the different frequencies of the auditory signal are separated and presented in a tonotopic manner along the length of the cochlea via the electrode array. The spatial and temporal encoding of the sound frequencies can be partly replicated by multi-channel stimulation of the auditory nerve. Encoding strategy in multi-channel cochlear implant consists of feature extraction and wave form. The resulting speech understanding of the single-channel and multi-channel systems was also assessed. Conclusion: Based on the findings, it can be concluded that single-channel cochlear implant systems with temporal encoding of frequency do not adequately convey speech information, whereas multi-channel cochlear implants ‎have more similarities to the tonotopic map of the cochlea and provide a better speech understanding in comparison to single channel devices.