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This study investigated the effects of two temporal response properties of the auditory nerve (i.e., neural adaptation and recovery from neural adaptation) on speech perception performance in postlingually deafened adult cochlear implant (CI) users.Study participants included 18 postlingually deafened adults who were Cochlear Nucleus device users with a full electrode array insertion in the test ear(s). Neural adaptation and adaptation recovery of the auditory nerve (AN) were evaluated using electrophysiological measures of the electrically evoked compound action potential (eCAP). The amount of neural adaptation was quantified by the adaptation index within three time windows: 0 to 8.89 (window 1), 44.44 to 50.00 (window 2), and 94.44 to 100.00 ms (window 3). The speed of neural adaptation was estimated using a two-parameter power law function. To evaluate adaptation recovery of the AN, eCAPs to the last pulse of the 100-ms pulse train were recorded at masker-probe-intervals ranging from 1.054 to 256 ms in logarithmic steps. The amount of adaptation recovery was quantified by the adaptation recovery ratio. The time-constant of adaptation recovery was estimated using an exponential function with up to three components. Speech perception performance was evaluated by measuring consonant-nucleus-consonant (CNC) word scores presented in quiet and in speech-shaped noise at a signal-to-noise ratio (SNR) of +10 dB. One-tailed Pearson Product Moment correlation tests were used (1) to assess the associations among parameters of neural adaptation and adaptation recovery and (2) to evaluate the strength of association between these parameters and CNC word scores measured in quiet and in noise. The contributions of different parameters quantifying neural adaptation and adaptation recovery on speech perception scores were evaluated using multivariable linear regression analyses.The Pearson Product Moment correlation coefficient demonstrated a moderate, negative correlation between the speed of adaptation recovery and CNC word scores measured in quiet and in noise. The speed of adaptation recovery accounted for 14.1% of variability in CNC word scores measured in quiet and 16.7% of variability in CNC word scores measured in noise. The correlation strengths between CNC word scores and the adaptation index, the adaptation recovery ratio and the speed of neural adaptation ranged from negligible to weak.The speed of adaptation recovery plays a more important role than other features of neural adaptation and adaptation recovery of the AN in speech perception in postlingually deafened adult CI users. Patients with prolonged adaptation recovery tend to show poorer speech perception performance.

The electrically evoked compound action potential (eCAP) is a direct measure of the responsiveness of the auditory nerve to electrical stimulation from a cochlear implant (CI). CIs offer a unique opportunity to study the auditory nerve’s electrophysiological behavior in individual human subjects over time. In order to understand exactly how the eCAP relates to the condition of the auditory nerve, it is crucial to compare changes in the eCAP over time in a controlled model of deafness-induced auditory nerve degeneration. In the present study, 10 normal-hearing young adult guinea pigs were implanted and deafened 4 weeks later, so that the effect of deafening could be monitored within-subject over time. Following implantation, but before deafening, most examined eCAP characteristics significantly changed, suggesting increasing excitation efficacy (e.g., higher maximum amplitude, lower threshold, shorter latency). Conversely, inter-phase gap (IPG) effects on these measures – within-subject difference measures that have been shown to correlate well with auditory nerve survival – did not vary for most eCAP characteristics. After deafening, we observed an initial increase in excitability (steeper slope of the eCAP amplitude growth function (AGF), lower threshold, shorter latency and peak width) which typically returned to normal-hearing levels within a week, after which a slower process, probably reflecting spiral ganglion cell loss, took place over the remaining 6 weeks (e.g., decrease in maximum amplitude, AGF slope, peak area, and IPG effect for AGF slope; increase in IPG effect for latency). Our results suggest that gradual changes in peak width and latency reflect the rate of neural degeneration, while peak area, maximum amplitude, and AGF slope reflect neural population size, which may be valuable for clinical diagnostics.

Auditory neuropathy is a disorder of auditory dysfunction characterized by the normal function of the outer hair cells and malfunction of the inner hair cells, synapses, postsynapses and/or auditory afferent nervous system. This review summarizes the process of discovery and naming of auditory neuropathy and describes the acquired, associated genetic disorders and management available.In the last 40 years, auditory neuropathy has undergone a process of discovery, naming and progressive elucidation of its complex pathological mechanisms. Recent studies have revealed numerous acquired and inherited causative factors associated with auditory neuropathy. Studies have analyzed the pathogenic mechanisms of various genes and the outcomes of cochlear implantation. New therapeutic approaches, such as stem cell therapy and gene therapy are the future trends in the treatment of auditory neuropathy.A comprehensive understanding of the pathogenic mechanisms is crucial in illustrating auditory neuropathy and assist in developing future management strategies.

Exposure to nontraumatic noise in vivo drives long-lasting changes in auditory nerve synapses, which may influence hearing, but the induction mechanisms are not known. We mimicked activity in acute slices of the cochlear nucleus from mice of both sexes by treating them with high potassium, after which voltage-clamp recordings from bushy cells indicated that auditory nerve synapses had reduced EPSC amplitude, quantal size, and vesicle release probability (P(r)). The effects of high potassium were prevented by blockers of nitric oxide (NO) synthase and protein kinase A. Treatment with the NO donor, PAPA-NONOate, also decreased P(r), suggesting NO plays a central role in inducing synaptic changes. To identify the source of NO, we activated auditory nerve fibers specifically using optogenetics. Strobing for 2 h led to decreased EPSC amplitude and P(r), which was prevented by antagonists against ionotropic glutamate receptors and NO synthase. This suggests that the activation of AMPA and NMDA receptors in postsynaptic targets of auditory nerve fibers drives release of NO, which acts retrogradely to cause long-term changes in synaptic function in auditory nerve synapses. This may provide insight into preventing or treating disorders caused by noise exposure. SIGNIFICANCE STATEMENT Auditory nerve fibers undergo long-lasting changes in synaptic properties in response to noise exposure in vivo, which may contribute to changes in hearing. Here, we investigated the cellular mechanisms underlying induction of synaptic changes using high potassium and optogenetic stimulation in vitro and identified important signaling pathways using pharmacology. Our results suggest that auditory nerve activity drives postsynaptic depolarization through AMPA and NMDA receptors, leading to the release of nitric oxide, which acts retrogradely to regulate presynaptic neurotransmitter release. These experiments revealed that auditory nerve synapses are unexpectedly sensitive to activity and can show dramatic, long-lasting changes in a few hours that could affect hearing.

Aging is associated with auditory nerve (AN) functional deficits and decreased inhibition in the central auditory system, amplifying central responses in a process referred to here as central gain. Although central gain increases response amplitudes, central gain may not restore disrupted response timing. In this translational study, we measured responses putatively generated by the AN and auditory midbrain in younger and older mice and humans. We hypothesized that older mice and humans exhibit increased central gain without an improvement in inter-trial synchrony in the midbrain. Our data demonstrated greater age-related deficits in AN response amplitudes than auditory midbrain response amplitudes, as shown by significant interactions between inferred neural generator and age group, indicating increased central gain in auditory midbrain. However, synchrony decreases with age in both the AN and midbrain responses. These results reveal age-related increases in central gain without concomitant improvements in synchrony, consistent with those predictions based on decreases in inhibition. Persistent decreases in synchrony may contribute to auditory processing deficits in older mice and humans.

Characterize the contribution of the auditory nerve neurophonic (ANN) to electrocochleography (ECochG) of pediatric cochlear implant (CI) recipients with and without auditory nerve spectrum disorder (ANSD).ECochG is an emerging technique for predicting outcomes in CI recipients. Its utility may be increased by separating the cochlear microphonic (CM), produced by hair cells, from the ANN, the evoked potential correlate of neural phase-locking, which are mixed in the ongoing portion of the response to low frequency tone bursts.Responses to tone bursts of different frequency and intensities were recorded from the round window of pediatric CI recipients. Separation of the CM and ANN was performed using a model of the underlying processes that lead to the shapes of the observed waveforms.Preoperative mean pure tone amplitudes of the included ANSD (n = 36) and non-ANSD subjects (n = 123), were similar (89.5 and 93.5, p = 0.1). Total of 1,024 ECochG responses to frequency and intensity series were recorded. The mean correlation ( r ) between the input and the modeled signals was 0.973 ± 0.056 (standard deviation). The ANN magnitudes were higher in the ANSD group (ANOVAs, F = 26.5 for frequency and 21.9 for intensity, df's = 1, p 's0.001). However, its relative contribution to the overall signal was lower (ANOVAs, F = 25.8 and 12.1, df = 1, p 's0.001).ANN was detected in low frequency ECochG responses but not high frequency responses in both ANSD and non-ANSD subjects. ANSD subjects, evidence of neural contribution in responses to low frequency stimuli was highly variable and often comparable to signals recorded in non-ANSD subjects. The computational model revealed that on average the ANN comprised a lower proportion of the overall signal than in non-ANSD subjects.

Auditory nerve injury is one of the most common nerve injury complications of skull base fractures. However, there is currently a lack of auxiliary examination methods for its direct diagnosis. The purpose of this study was to find a more efficient and accurate means of diagnosis for auditory nerve injury.Through retrospectively analyzing the results of brainstem auditory evoked potential (BAEP) and high-resolution CT (HRCT) in 37 patients with hearing impairment following trauma from January 1, 2018 to July 31, 2020, the role of the two inspection methods in the diagnosis of auditory nerve injury was studied. Inclusion criteria were patient had a clear history of trauma and unilateral hearing impairment after trauma; while exclusion criteria were: (1) severe patient with a Glasgow coma scale score ≤5 because these patients were classified as severe head injury and admitted to the intensive care unit, (2) patient in the subacute stage admitted 72 h after trauma, and (3) patient with prior hearing impairment before trauma. According to Goodman's classification of hearing impairment, the patients were divided into low/medium/severe injury groups. In addition, patients were divided into HRCT-positive and negative groups for further investigation with their BAEP results. The positive rates of BEAP for each group were observed, and the results were analyzed by Chi-square test (p 0.05, regarded as statistical difference).A total of 37 patients were included, including 21 males and 16 females. All of them were hospitalized patients with GCS score of 6-15 at the time of admission. The BAEP positive rate in the medium and severe injury group was 100%, which was significantly higher than that in the low injury group (27.27%) (p 0.01). The rate of BEAP positivity was significantly higher in the HRCT-positive group (20/30, 66.7%) than in the HRCT-negative group (1/7, 14.3%) (p 0.05). Twenty patients (54.05%) were both positive for BEAP and HRCT test, and considered to have auditory nerve damage. Six patients (16.22%) were both negative for BEAP and HRCT test, and 10 patients (27.03%) were BAEP-negative but HRCT-positive: all the 16 patients were considered as non-neurological injury. The rest 1 case (2.70%) was BAEP-positive but HRCT-negative, which we speculate may have auditory nerve concussion.By way of BAEP combining with skull base HRCT, we may improve the accuracy of the diagnosis of auditory nerve injury. Such a diagnostic strategy may be beneficial to guiding treatment plans and evaluating prognosis.

Introduction Cochlear implantation is an effective treatment method for severe to profound hearing loss. Many factors that may influence cochlear implantation success have been explained in previous studies. Apart from those, minor differences in size of normal cochlear nerves may affect postoperative performance. Objective To investigate whether the minor differences in cochlear nerve size in normal cochlear nerves affect postoperative cochlear implant performance. Methods 30 pediatric prelingually deaf patients who were treated with cochlear implantation were included in this study. From the reconstructed parasagittal magnetic resonance images, the diameter and cross-sectional area of the cochlear nerve on the ipsilateral and contralateral side were measured. Auditory evaluations were performed 1, 3, 6 and 12 months following the first fitting. All the analysis was performed by using EARS®, evaluation of auditory responses to speech tool. Correlation between cochlear nerve diameter, cross-sectional area and postoperative auditory perception was analyzed to determine whether variation in cochlear nerve size contributes to postoperative auditory performance. Results The mean diameter of the cochlear nerve on the ipsilateral side was 718.4 μm (504.5 − 904.3 μm) and mean cross sectional area was 0.015 cm2 (0.012 − 0.018 cm2). On the contralateral side the mean cochlear nerve diameter was 714.4 μm (502.6 − 951.4 μm) and mean cross sectional area was 0.014 cm2 (0.011 − 0.019 cm2). The correlation between the diameter and cross-sectional area of the ipsilateral and contralateral cochlear nerve revealed no significance. Mean score at first month monosyllable-trochee-polysyllable test, MTP1, was 0.17 (0.08 − 0.33), at 6th month with 6 words test, 6th month MTP6 was 0.72 (0.39 − 1.0), at 6th month with 12 words, 6th month MTP 12 was 0.46 (0.17 − 0.75) and at 12th month with 12 words, 12th month MTP12 was 0.73 (0.25 − 1.0). There was no correlation between the monosyllable-trochee-polysyllable test, values at any time with the diameter of the ipsilateral cochlear nerve. However, the first month MTP, 6th month MTP6 and 12th month MTP12 correlated with the cross-sectional area of the ipsilateral cochlear nerve. Conclusion Measuring the cross sectional area of the normal- appearing cochlear nerve may give important prognostic knowledge on cochlear implant outcomes. In patients with a larger cross sectional area the auditory performance was better and faster. Although normal appearing, slight differences on cross sectional area of the cochlear nerve may affect performance. Measuring the size of the cochlear nerve on parasagittal magnetic resonance images may provide beneficial information on the postoperative rehabilitation process. Resumo Introdução O implante coclear é um método de tratamento eficaz para a perda auditiva grave a profunda. Muitos fatores que podem influenciar o sucesso do implante coclear foram explicados em estudos anteriores. Além desses fatores, pequenas diferenças no tamanho dos nervos cocleares normais podem afetar o desempenho pós-operatório. Objetivo Investigar se pequenas diferenças no tamanho dos nervos cocleares normais afetam o desempenho pós-operatório do implante coclear. Método Foram incluídos neste estudo 30 pacientes pediátricos surdos pré-linguais, tratados com implante coclear. A partir de imagens de ressonância magnética parassagitais reconstruídas, foram medidos o diâmetro e a área de seção transversal do nervo coclear no lado ipsilateral e contralateral. As avaliações auditivas foram feitas 1, 3, 6 e 12 meses após a primeira adaptação. Todas as análises foram feitas com a ferramenta EARS® (do inglês evaluation of auditory responses to speech). A correlação entre o diâmetro do nervo coclear, a área transversal e a percepção auditiva pós-operatória foi analisada para determinar se a variação no tamanho do nervo coclear contribui para o desempenho auditivo pós-operatório. Resultados O diâmetro médio do nervo coclear no lado ipsilateral foi de 718,4 μm (504,5 a 904,3 μm) e a área da seção transversal média foi de 0,015 cm2 (0,012-0,018 cm2). No lado contralateral, o diâmetro médio do nervo coclear foi de 714,4 μm (502,6 a 951,4 μm) e a área da seção transversal média foi de 0,014 cm2 (0,011 a 0,019 cm2). A correlação entre o diâmetro e a área transversal do nervo coclear ipsilateral e contralateral não revelou qualquer significância. O escore médio do teste monosyllable-trochee-polysyllable no primeiro mês, denominado MTP1, foi de 0,17 (0,08-0,33), no sexto mês com teste de 6 palavras, MTP6, foi de 0,72 (0,39-1,0), no sexto mês com 12 palavras, MTP12, foi de 0,46 (0,17-0,75) e no 12° mês com 12 palavras, MTP12, foi de 0,73 (0,25-1,0). Não houve correlação entre os valores do teste monosyllable-trochee-polysyllable em qualquer momento com o diâmetro do nervo coclear ipsilateral. Entretanto, o teste monosyllable-trochee-polysyllable do primeiro mês, do 6° mês, e o do 12° mês correlacionaram-se com a área transversal do nervo coclear ipsilateral. Conclusão Medir a área da secção transversal do nervo coclear de aparência normal pode fornecer conhecimento prognóstico importante sobre os resultados do implante coclear. Em pacientes com área da secção transversal maior, o desempenho auditivo foi melhor e mais rápido. Embora o nervo coclear pareça normal, pequenas diferenças na área da secção transversal do nervo coclear podem afetar o desempenho, de forma que a medida do tamanho do nervo coclear nas imagens de ressonância magnética na projeção parassagital pode fornecer informações benéficas sobre o processo de reabilitação pós-operatória.

To report the novel use of intraoperative electrically evoked stapedial reflex (eSR) for cochlear nerve monitoring during simultaneous translabyrinthine resection of vestibular schwannoma (VS) and cochlear implantation.Clinical capsule report with video demonstration.Tertiary academic referral center.A 58-year-old female presented with a small right intracanalicular VS with associated asymmetrical right moderate to severe sensorineural hearing loss, poor word recognition, tinnitus, and disequilibrium. Based on patient symptomatology and goals, simultaneous CI with translabyrinthine resection of the VS was performed.Cochlear implantation before the tumor was resected facilitated intraoperative eSRs by delivering repeated single-electrode stimulations through the cochlear implant (CI) electrode during tumor resection. A pulse duration of 50-us and a current amplitude of 200-CL or 648-us was used to elicit eSRs visible through the facial recess. Intraoperative eSR was monitored in conjunction with electrically evoked compound action potentials via neural response telemetry and electrical auditory brainstem response.Despite the transient evoked compound action potentials amplitude and electrical auditory brainstem response latency changes, the visually observed eSR was preserved and remained robust throughout tumor dissection, indicating an intact cochlear nerve. Four weeks postoperatively, the patient exhibited open-set speech capacity (14% CNC and 36% AzBio in quiet).The current study demonstrates the feasibility of using intraoperative eSR via a CI electrode to monitor cochlear nerve integrity during VS resection, which may indicate successful CI outcomes. These preliminary findings require further substantiation in a larger study.

OBJECTIVE: This study aimed to 1) characterize the amount and the speed of recovery from neural adaptation at the auditory nerve (AN), and 2) assess their associations with advanced age in postlingually deafened adult cochlear implant (CI) users. DESIGN: Study participants included 25 postlingually deafened adult, Cochlear™ Nucleus® device users, ranging in age between 24.83 and 83.21 years at the time of testing. The stimulus was a 100-ms pulse train presented at four pulse rates: 500, 900, 1800 and 2400 pulses per second (pps). The pulse trains were presented at the maximum comfortable level measured for the 2400-pps pulse train. The electrically evoked compound action potential (eCAP) evoked by the last pulse of the pulse train (i.e., the probe pulse) was recorded. The remaining pulses of the pulse train served as the pulse-train masker. The time interval between the probe pulse and the last pulse of the pulse-train masker (i.e., masker-probe-interval, MPI) systematically increased from 0.359 ms up to 256 ms. The adaptation recovery function (ARF) was obtained by plotting normalized eCAP amplitudes (re: the eCAP amplitude measured at the MPI of 256 ms) as a function of MPIs. The adaptation recovery ratio (ARR) was defined as the ratio between the eCAP amplitude measured at the MPI of 256 ms and that measured for the single-pulse stimulus presented at the same stimulation level. The time constants of the ARF were estimated using a mathematical model with an exponential function with up to three components. Generalized Linear Mixed effects Models were used to compare ARRs and time constants measured at different electrode locations and pulse rates, as well as to assess the effect of advanced age on these dependent variables. RESULTS: There were three ARF types observed in this study. The ARF type observed in the same study participant could be different at different electrode locations and/or pulse rates. Substantial variations in both the amount and the speed of neural adaptation recovery among study participants were observed. The ARR was significantly affected by pulse rate but was not affected by electrode location. The effect of electrode location on the time constants of the ARF were not statistically significant. Pulse rate had a statistically significant effect on τ(1,) but not on τ(2) or τ(3). There was no statistically significant effect of age on the ARR or the time constants of the ARF. CONCLUSIONS: Neural adaptation recovery processes at the AN demonstrate substantial variations among human CI users. The recovery pattern can be nonmonotonic with up to three phases. While the amount of neural adaptation recovery decreases as pulse rate increases, only the speed of the first phase of neural adaptation recovery is affected by pulse rate. Electrode location or advanced age has no robust effect on neural adaptation recovery processes at the level of the AN for a 100-ms pulse-train masker with pulse rates of 500–2400 pps. The lack of sufficient participants in this study who were 40 years of age or younger at the time of testing might have precluded a thorough assessment of the effect of advanced age.

Sound-level coding in the auditory nerve is achieved through the progressive recruitment of auditory nerve fibers (ANFs) that differ in threshold of activation and in the stimulus level at which the spike rate saturates. To investigate the functional state of the ANFs, the electrophysiological tests routinely used in clinics only capture the first action potentials firing in synchrony at the onset of the acoustic stimulation. Assessment of other properties (e.g., spontaneous rate and adaptation time constants) requires single-fiber recordings directly from the nerve, which for ethical reasons is not allowed in humans. By combining neuronal activity measurements at the round window and signal-processing algorithms, we constructed a peristimulus time response (PSTR), with a waveform similar to the peristimulus time histograms (PSTHs) derived from single-fiber recordings in young adult female gerbils. Simultaneous recordings of round-window PSTR and single-fiber PSTH provided models to predict the adaptation kinetics and spontaneous rate of the ANFs tuned at the PSTR probe frequency. The predictive model derived from gerbils was then validated in female mice and finally applied to humans by recording PSTRs from the auditory nerve in normal-hearing patients who underwent cerebellopontine angle surgeries. A rapid adaptation time constant of ∼3 ms and a mean spontaneous rate of ∼22 spikes/s in the 4 kHz frequency range were found. This study offers a promising diagnostic tool to map the human auditory nerve, thus opening new avenues to better understanding auditory neuropathies, tinnitus, and hyperacusis.SIGNIFICANCE STATEMENTNeural adaptation in auditory nerve fibers corresponds to the reduction in the neuronal activity to prolonged or repeated sound stimulation. For obvious ethical reasons, single-fiber recordings from the auditory nerve are not feasible in humans, creating a critical gap in extending data obtained using animal models to humans. Using electrocochleography in rodents, we inferred adaptation kinetics and spontaneous discharge rates of the auditory nerve fibers in humans. Routinely used in basic and clinical laboratories, this tool will provide a better understanding of auditory disorders such as neuropathies, tinnitus, and hyperacusis, and will help to improve hearing-aid fittings.

Auditory function declines with age, as evidenced by communication difficulties in challenging listening environments for older adults. Declining auditory function may arise, in part, from an age-related loss and/or inactivity of low-spontaneous-rate (SR) auditory nerve (AN) fibers, a subgroup of neurons important for suprathreshold processing. Compared to high-SR fibers, low-SR fibers take longer to recover from prior stimulation. Taking advantage of this difference, the forward-masked recovery function paradigm estimates the relative proportions of low- and high-SR fibers in the AN by quantifying the time needed for AN responses to recover from prior stimulation (ΔT(recovery)). Due to the slower recovery of low-SR fibers, ANs that need more time to fully recover (longer ΔT(recovery)) are estimated to have a larger proportion of low-SR fibers than ANs that need less time (shorter ΔT(recovery)). To test the hypothesis that low-SR fiber activity is reduced in older humans, the current study assessed recovery functions in 32 older and 16 younger adults using the compound action potential. Results show that ΔT(recovery) is shorter for older adults than for younger adults, consistent with a theorized age-related loss and/or inactivity of low-SR fibers. ΔT(recovery) did not differ between individuals with and without a prior history of noise exposure as assessed by self-report. This study is the first to successfully assess forward-masked recovery functions in both younger and older adults and provides important insights into the structural and functional changes occurring in the AN with increasing age.

The architecture of the efferent auditory system enables prioritization of strongly overlapping spatiotemporal cochlear activation patterns elicited by relevant and irrelevant inputs. So far, attempts at finding such attentional modulations of cochlear activity delivered indirect insights in humans or required direct recordings in animals. The extent to which spiral ganglion cells forming the human auditory nerve are sensitive to selective attention remains largely unknown. We investigated this question by testing the effects of attending to either the auditory or visual modality in human cochlear implant (CI) users (3 female, 13 male). Auditory nerve activity was directly recorded with standard CIs during a silent (anticipatory) cue-target interval. When attending the upcoming auditory input, ongoing auditory nerve activity within the theta range (5-8 Hz) was enhanced. Crucially, using the broadband signal (4-25 Hz), a classifier was even able to decode the attended modality from single-trial data. Follow-up analysis showed that the effect was not driven by a narrow frequency in particular. Using direct cochlear recordings from deaf individuals, our findings suggest that cochlear spiral ganglion cells are sensitive to top-down attentional modulations. Given the putatively broad hair-cell degeneration of these individuals, the effects are likely mediated by alternative efferent pathways compared with previous studies using otoacoustic emissions. Successful classification of single-trial data could additionally have a significant impact on future closed-loop CI developments that incorporate real-time optimization of CI parameters based on the current mental state of the user.SIGNIFICANCE STATEMENTThe efferent auditory system in principle allows top-down modulation of auditory nerve activity; however, evidence for this is lacking in humans. Using cochlear recordings in participants performing an audiovisual attention task, we show that ongoing auditory nerve activity in the silent cue-target period is directly modulated by selective attention. Specifically, ongoing auditory nerve activity is enhanced within the theta range when attending upcoming auditory input. Furthermore, over a broader frequency range, the attended modality can be decoded from single-trial data. Demonstrating this direct top-down influence on auditory nerve activity substantially extends previous works that focus on outer hair cell activity. Generally, our work could promote the use of standard cochlear implant electrodes to study cognitive neuroscientific questions.

OBJECTIVE: This study aimed to investigate the associations between advanced age and the amount and the speed of neural adaptation of the electrically-stimulated auditory nerve (AN) in postlingually deafened adult cochlear implant (CI) users. DESIGN: Study participants included 26 postlingually deafened adult CI users, ranging in age between 28.7 and 84.0 years (mean: 63.8 years, std: 14.4 years) at the time of testing. All study participants used a Cochlear(™) Nucleus® device with a full electrode array insertion in the test ear. The stimulus was a 100-ms pulse train with a pulse rate of 500, 900, 1800 or 2400 pulses per second (pps) per channel. The stimulus was presented at the maximum comfortable level measured at 2400 pps with a presentation rate of 2 Hz. Neural adaptation of the AN was evaluated using electrophysiological measures of the electrically evoked compound action potential (eCAP). The amount of neural adaptation was quantified by the adaptation index (AI) within three time windows: around 0–8 ms (window 1), 44–50 ms (window 2) and 94–100 ms (window 3). The speed of neural adaptation was quantified using a two-parameter power law estimation. In 23 participants, four electrodes across the electrode array were tested. In three participants, three electrodes were tested. Results measured at different electrode locations were averaged for each participant at each pulse rate in order to get an overall representation of neural adaptation properties of the AN across the cochlea. Linear Mixed Models (LMMs) were used 1) to evaluate the effects of age at testing and pulse rate on the speed of neural adaptation, and 2) to assess the effects of age at testing, pulse rate, and duration of stimulation (i.e., time window) on the amount of neural adaptation in these participants. RESULTS: There was substantial variability in both the amount and the speed of neural adaptation of the AN among study participants. The amount and the speed of neural adaptation increased at higher pulse rates. In addition, larger amounts of adaptation were observed for longer durations of stimulation. There was no significant effect of age on the speed or the amount of neural adaptation. CONCLUSIONS: The amount and the speed of neural adaptation of the AN are affected by both the pulse rate and the duration of stimulation, with higher pulse rates and longer durations of stimulation leading to faster and greater neural adaptation. Advanced age does not affect neural adaptation of the AN in postlingually deafened, middle-aged and elderly adult CI users.

Introduction: The rates of cochlear nerve abnormalities and cochlear malformations in pediatric unilateral hearing loss (UHL) are conflicting in the literature, with important implications on management. The aim of this study was to investigate the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL). Methods: A retrospective chart review of pediatric subjects n = 291) at a tertiary referral center was conducted. MRI brain and computed tomography temporal bone were reviewed for the presence of inner ear malformations and/or CND. Status of the ipsilateral cochlear nerve and inner ear was evaluated. Pure tone average (PTA) at 500, 1,000 and 2,000 Hz was assessed. Results: 204 subjects with UHL and 87 subjects with AHL were included. CND (aplasia or hypoplasia) was demonstrated in 61 pediatric subjects with UHL (29.9%) and 10 with AHL (11.5%). Ipsilateral cochlear malformations were noted in 25 subjects with UHL (12.3%) and 11 with AHL (12.6%), and ipsilateral vestibular malformations in 23 (11.3%) and 12 (13.8%) ears, respectively. Median PTA was statistically significantly higher in ears with CND (98.33) than ears with normal nerves (90.84). Discussion/Conclusion: Imaging demonstrated a high incidence of inner ear malformations, particularly CND, in pediatric subjects with UHL. Auditory findings indicated CND cannot be ruled out by thresholds alone as some CND ears did demonstrate measurable hearing. Radiologic evaluation by MRI should be performed in all patients within this population to guide counseling and management of hearing loss based on etiology, with implications on candidacy for cochlear implantation.

It is critical for hearing that the descending cochlear efferent system provides a negative feedback to hair cells to regulate hearing sensitivity and protect hearing from noise. The medial olivocochlear (MOC) efferent nerves project to outer hair cells (OHCs) to regulate OHC electromotility, which is an active cochlear amplifier and can increase hearing sensitivity. Here, we report that the MOC efferent nerves also could innervate supporting cells (SCs) in the vicinity of OHCs to regulate hearing sensitivity. MOC nerve fibers are cholinergic, and acetylcholine (ACh) is a primary neurotransmitter. Immunofluorescent staining showed that MOC nerve endings, presynaptic vesicular acetylcholine transporters (VAChTs), and postsynaptic ACh receptors were visible at SCs and in the SC area. Application of ACh in SCs could evoke a typical inward current and reduce gap junctions (GJs) between them, which consequently enhanced the direct effect of ACh on OHCs to shift but not eliminate OHC electromotility. This indirect, GJ-mediated inhibition had a long-lasting influence. In vivo experiments further demonstrated that deficiency of this GJ-mediated efferent pathway decreased the regulation of active cochlear amplification and compromised the protection against noise. In particular, distortion product otoacoustic emission (DPOAE) showed a delayed reduction after noise exposure. Our findings reveal a new pathway for the MOC efferent system via innervating SCs to control active cochlear amplification and hearing sensitivity. These data also suggest that this SC GJ-mediated efferent pathway may play a critical role in long-term efferent inhibition and is required for protection of hearing from noise trauma. NEW & NOTEWORTHY The cochlear efferent system provides a negative feedback to control hair cell activity and hearing sensitivity and plays a critical role in noise protection. We reveal a new efferent control pathway in which medial olivocochlear efferent fibers have innervations with cochlear supporting cells to control their gap junctions, therefore regulating outer hair cell electromotility and hearing sensitivity. This supporting cell gap junction-mediated efferent control pathway is required for the protection of hearing from noise.

The auditory nerve (AN) of the inner ear is the primary conveyor of acoustic information from sensory hair cells to the brainstem. Approximately 95% of peripheral AN fibers are myelinated by glial cells. The integrity of myelin and the glial-associated paranodal structures at the node of Ranvier is critical for normal AN activity and axonal survival and function in the central auditory nervous system. However, little is known about the node of Ranvier’s spatiotemporal development in the AN, how the aging process (or injury) affects the activity of myelinating glial cells, and how downstream alterations in myelin and paranodal structure contribute to AN degeneration and sensorineural hearing loss. Here, we characterized two types of Ranvier nodes— the axonal node and the ganglion node—in the mouse peripheral AN, and found that they are distinct in several features of postnatal myelination and age-related degeneration. Cellular, molecular, and structure–function correlations revealed that the two node types are each critical for different aspects of peripheral AN function. Neural processing speed and synchrony is associated with the length of the axonal node, while stimulus level-dependent amplitude growth and action potentials are associated with the ganglion node. Moreover, our data indicate that dysregulation of glial cells (e.g., satellite cells) and degeneration of the ganglion node structure are an important new mechanism of age-related hearing loss.

Postimplantation facial nerve stimulation is a common side-effect of intracochlear electrical stimulation. Facial nerve stimulation occurs when electric current intended to stimulate the auditory nerve, spread beyond the cochlea to excite the nearby facial nerve, causing involuntarily facial muscle contractions. Facial nerve stimulation can often be resolved through adjustments in speech processor fitting but, in some instances, these measures exhibit limited benefit or may have a detrimental effect on speech perception. In this study, apical reference stimulation mode was investigated as a potential intervention to facial nerve stimulation. Apical reference stimulation is a bipolar stimulation strategy in which the most apical electrode is used as the reference electrode for stimulation on all the other intracochlear electrodes.A person-specific model of the human cochlea, facial nerve and electrode array, coupled with a neural model, was used to predict excitation of auditory and facial nerve fibers. These predictions were used to evaluate the effectiveness in reducing facial nerve stimulation using apical reference stimulation. Predictions were confirmed in psychoacoustic tests by determining auditory comfort and threshold levels for the apical reference stimulation mode while capturing electromyography data in two participants.Models predicted a favorable outcome for apical reference stimulation, as facial nerve fiber thresholds were higher and auditory thresholds were lower, in direct comparison to conventional monopolar stimulation. Psychophysical tests also illustrated decreased auditory thresholds and increased dynamic range during apical reference stimulation. Furthermore, apical reference stimulation resulted in lower electromyography energy levels, compared to conventional monopolar stimulation, which suggests a reduction in facial nerve stimulation. Subjective feedback corroborated that apical reference stimulation alleviated facial nerve stimulation.Apical reference stimulation may be a viable strategy to alleviate facial nerve stimulation considering the improvements in dynamic range and auditory thresholds, complemented with a reduction in facial nerve stimulation symptoms.

Key points Cochlear nucleus small cells receive input from low/medium spontaneous rate auditory nerve fibers and medial olivocochlear neurons. Electrical stimulation of medial olivocochlear neurons in the VNTB and blocking cholinergic input to small cells using atropine demonstrates an excitatory cholinergic input to small cells, which increases responses to suprathreshold sound. We show that unique inputs to small cells produce superior sound intensity coding. This coding of intensity is preserved in the presence of background noise, an effect exclusive to this cell type in the cochlear nucleus. These results suggest that small cells serve an essential function in the ascending auditory system, which may be relevant to disorders such as hidden hearing loss. Abstract Understanding communication signals, especially in noisy environments, is crucial to social interactions. Yet, as we age, acoustic signals can be disrupted by cochlear damage and the subsequent auditory nerve fiber degeneration. The most vulnerable-medium and high-threshold-auditory nerve fibers innervate various cell types in the cochlear nucleus, among which, the small cells are unique in receiving this input exclusively. Furthermore, small cells project to medial olivocochlear (MOC) neurons, which in turn send branched collaterals back into the small cell cap (SCC). Here, we use single-unit recordings to characterise small cell firing characteristics and demonstrate superior intensity coding in this cell class. We show converse effects when activating/blocking the MOC system, demonstrating that small-cell unique coding properties are facilitated by direct cholinergic input from the MOC system. Small cells also maintain tone-level coding in the presence of background noise. Finally, small cells precisely code low-frequency modulation more accurately than other ventral CN (VCN) cell types, demonstrating accurate envelope coding that may be important for vocalisation processing. These results highlight the small cell-olivocochlear circuit as a key player in signal processing in noisy environments, which may be selectively degraded in aging or after noise insult. This article is protected by copyright. All rights reserved.

Although clinical use of the auditory brainstem response (ABR) to detect retrocochlear disorders has been largely replaced by imaging in recent years, the discovery of cochlear synaptopathy has thrown this foundational measure of auditory function back into the spotlight. Whereas modern imaging now allows for the noninvasive detection of vestibular schwannomas, imaging technology is not currently capable of detecting cochlear synaptopathy, the loss of the synaptic connections between the inner hair cells and afferent auditory nerve fibers. However, animal models indicate that the amplitude of the first wave of the ABR, a far-field evoked potential generated by the synchronous firing of auditory nerve fibers, is highly correlated with synaptic integrity. This has led to many studies investigating the use of the ABR as a metric of synaptopathy in humans. However, these studies have yielded mixed results, leading to a lack of consensus about the utility of the ABR as an indicator of synaptopathy. This review summarizes the animal and human studies that have investigated the ABR as a measure of cochlear synaptic function, discusses factors that may have contributed to the mixed findings and the lessons learned, and provides recommendations for future use of this metric in the research and clinical settings.

Objective Axenfeld-Rieger syndrome (ARS) type 3 is a rare autosomal dominant disease, characterized by anterior segment dysgenesis of the eye, hearing loss, and cardiac defects. ARS type 3 is highly associated with FOXC1 mutations, which induces developmental disorders of neural crest cells. Most studies about ARS patients focused on ophthalmologic findings, but details in their hearing loss have not yet been revealed. In this report, we investigated audiological and otological manifestations in the ARS type 3 patient who had the novel heterozygous FOXC1 mutation leading deletion at the forkhead DNA-binding domain. Methods and Results Pure tone audiometry showed bilateral sensorineural hearing loss (SNHL) and audiological examinations confirmed that major dysfunctions existed in the cochlea, rather than the spiral ganglion neurons and the cochlear nerve. CT and MRI revealed the hypoplastic cochlea at both sides. Given that the 6p25 deletion syndrome, lacking one allele of the FOXC1 gene, shows similar, but more severe cochlear malformations than the present case, the FOXC1 mutations might contribute to the hypoplasia and dysfunctions in the cochlea. Conclusion To our knowledge, this is the first report demonstrating that the ARS type 3 patient with the FOXC1 mutation has the hypoplasia and dysfunctions in the cochlea, which results in bilateral SNHL.

ZusammenfassungAusgewertet wurden 86 Patienten mit einem retrosigmoidal mikrochirurgisch resezierten Vestibularisschwannom im Tumorstadium Koos II–IV. Es zeigte sich, dass im Bereich des inneren Gehörgangs in über 2/3 der Fälle der Nervus cochlearis dem elektroneurographisch leicht zu identifizierenden Nervus facialis in immer wiederkehrenden ähnlichen Verlaufsmustern folgte. Ausgehend vom Fundus erleichterte dies die frühzeitige Identifizierung und damit den Kontinuitätserhalt des Nervus cochlearis im Verlauf des inneren Gehörgangs. Dies war vor allem dann von Bedeutung, wenn ein sicherer Funktionserhalt aufgrund der Tumorgröße oder -formation trotz intraoperativer Ableitung somatosensorischer Potenziale nicht sicher gewährleistet werden konnte, aber die Möglichkeit einer späteren Hörrehabilitation mit einem Cochleaimplantat bestehen bleiben sollte. Präoperative Magnetresonanztomographie(MRT)-Sequenzen ergaben zwar in einigen Fällen einen Hinweis auf die möglichen Nervenverläufe, die intraoperative Darstellung im inneren Gehörgang war der MRT aber überlegen.

A common complaint of older adults is difficulty understanding speech, particularly in challenging listening conditions. Accumulating evidence suggests that these difficulties may reflect a loss and/or dysfunction of auditory nerve (AN) fibers. We used a novel approach to study age-related changes in AN structure and several measures of AN function, including neural synchrony, in 58 older adults and 42 younger adults. AN activity was measured in response to an auditory click (compound action potential; CAP), presented at stimulus levels ranging from 70 to 110 dB pSPL. Poorer AN function was observed for older than younger adults across CAP measures at higher but not lower stimulus levels. Associations across metrics and stimulus levels were consistent with age-related AN disengagement and AN dyssynchrony. High-resolution T2-weighted structural imaging revealed age-related differences in the density of cranial nerve VIII, with lower density in older adults with poorer neural synchrony. Individual differences in neural synchrony were the strongest predictor of speech recognition, such that poorer synchrony predicted poorer recognition of time-compressed speech and poorer speech recognition in noise for both younger and older adults. These results have broad clinical implications and are consistent with an interpretation that age-related atrophy at the level of the AN contributes to poorer neural synchrony and may explain some of the perceptual difficulties of older adults.SIGNIFICANCE STATEMENTDifferences in auditory nerve (AN) pathophysiology may contribute to the large variations in hearing and communication abilities of older adults. However, current diagnostics focus largely on the increase in detection thresholds, which is likely because of the absence of indirect measures of AN function in standard clinical test batteries. Using novel metrics of AN function, combined with estimates of AN structure and auditory function, we identified age-related differences across measures that we interpret to represent age-related reductions in AN engagement and poorer neural synchrony. Structure-function associations are consistent with an explanation of AN deficits that arise from age-related atrophy of the AN. Associations between neural synchrony and speech recognition suggest that individual and age-related deficits in neural synchrony contribute to speech recognition deficits.

A difference in fundamental frequency (F0) between two vowels is an important segregation cue prior to identifying concurrent vowels. To understand the effects of this cue on identification due to age and hearing loss, Chintanpalli, Ahlstrom, and Dubno [(2016). J. Acoust. Soc. Am. 140, 4142–4153] collected concurrent vowel scores across F0 differences for younger adults with normal hearing (YNH), older adults with normal hearing (ONH), and older adults with hearing loss (OHI). The current modeling study predicts these concurrent vowel scores to understand age and hearing loss effects. The YNH model cascaded the temporal responses of an auditory-nerve model from Bruce, Efrani, and Zilany [(2018). Hear. Res. 360, 40–45] with a modified F0-guided segregation algorithm from Meddis and Hewitt [(1992). J. Acoust. Soc. Am. 91, 233–245] to predict concurrent vowel scores. The ONH model included endocochlear-potential loss, while the OHI model also included hair cell damage; however, both models incorporated cochlear synaptopathy, with a larger effect for OHI. Compared with the YNH model, concurrent vowel scores were reduced across F0 differences for ONH and OHI models, with the lowest scores for OHI. These patterns successfully captured the age and hearing loss effects in the concurrent-vowel data. The predictions suggest that the inability to utilize an F0-guided segregation cue, resulting from peripheral changes, may reduce scores for ONH and OHI listeners.

Introduction Cryptococcus spp. is a fungus responsible for 600,000 deaths per year worldwide, mainly in immunosuppressed subjects. However, 20% of cases occur in immunocompetent subjects. Neuropathic disorders involving the auditory nerve have been reported, but vestibular disorders have never been described in detail. We report the case of an immunocompetent man, who presented audiovestibular disorders leading to a diagnosis of cryptococcal meningitis. Case report A 39-year-old man was referred for balance disorders and right sensorineural hearing loss. He presented right vestibulo-saccular impairment and bilateral absence of auditory brainstem responses. Brain MRI was suggestive of cryptococcal meningitis. A cystic lesion in the right flocculus compressed the vestibulocochlear nerve. During monthly follow-up, pure tone audiometry gradually improved and speech audiometry in silence returned to normal. Partial resynchronization of the auditory afferent pathways was observed only on the contralateral side to vestibulocochlear nerve compression, while complete recovery of saccular function was observed. Discussion Cryptococcal meningitis in immunocompetent subjects may be accompanied by lesions of the auditory and vestibular afferent pathways. Recovery of hearing and balance was observed in response to medical treatment and early vestibular rehabilitation.

Objectives Histologic reports of temporal bones of ears with vestibular schwannomas (VSs) have indicated findings of endolymphatic hydrops (ELH) in some cases. The main goal of this investigation was to test ears with VSs to determine if they exhibit electrophysiological characteristics similar to those of ears expected to experience ELH. Design Fifty-three subjects with surgically confirmed VS aged ≥18 and with normal middle ear status were included in this study. In addition, a second group of adult subjects (n = 24) undergoing labyrinthectomy (n = 6) or endolymphatic sac decompression and shunt (ELS) placement (n = 18) for poorly controlled vestibular symptoms associated with Meniere's disease (MD) participated in this research. Intraoperative electrocochleography (ECochG) from the round window was performed using tone burst stimuli. Audiometric testing and word recognition scores (WRS) were performed preoperatively. ECochG amplitudes, cochlear microphonic/auditory nerve neurophonic (ANN) in the form of the "ongoing" response and summation potential, were analyzed and compared between the two groups of subjects. In addition, to evaluate any effect of auditory nerve function, the auditory nerve score was calculated for each subject. Pure-tone averages were obtained using the average air conduction thresholds at 0.5, 1, and 2 kHz while WRS was assessed using Northwestern University Auditory Test No. 6 word lists. Results In the VS group the average pure-tone averages and WRS were 59.6 dB HL and 44.8%, respectively, while in the MD group they were 52.3 dB HL and 73.8%. ECochG findings in both groups revealed a reduced trend in amplitude of the ongoing response with increased stimulus frequency. The summation potential amplitudes of subjects with VS were found to be less negative than the MD subjects for nearly all test frequencies. Finally, the VS group exhibited poorer amounts of auditory nerve function compared to the MD group. Conclusions The current findings suggest cochlear pathology (e.g., hair cell loss) in both groups but do not support the hypothesis that VSs cause ELH.

GABAergic inhibition in neurons plays a critical role in determining the output of neural circuits. Neurons in avian nucleus magnocellularis (NM) use several tonotopic-region-dependent specializations to relay the timing information of sound in the auditory nerve to higher auditory nuclei. Previously, we showed that feedforward GABAergic inhibition in NM has a different dependence on the level of auditory nerve activity, with the low-frequency region having a low-threshold and linear relationship, while the high-frequency region has a high-threshold and step-like relationship. However, it remains unclear how the GABAergic synapses are tonotopically regulated and interact with other specializations of NM neurons. In this study, we examined GABAergic transmission in the NM of chickens of both sexes and explored its contributions to the temporal coding of sound at each tonotopic region. We found that the number and size of unitary GABAergic currents and their reversal potential were finely tuned at each tonotopic region in the NM. At the lower-frequency region, unitary GABAergic currents were larger in number but smaller in size. In addition, their reversal potential was close to the resting potential of neurons, which enabled reliable inhibition despite the smaller potassium conductance. At the higher-frequency region, on the other hand, unitary GABAergic currents were fewer, larger, and highly depolarizing, which enabled powerful inhibition via activating the large potassium conductance. Thus, we propose that GABAergic synapses are coordinated with the characteristics of excitatory synapses and postsynaptic neurons, ensuring the temporal coding for wide frequency and intensity ranges.SIGNIFICANCE STATEMENTWe found in avian cochlear nucleus that the number and size of unitary GABAergic inputs differed among tonotopic regions and correlated to respective excitatory inputs; it was larger in number but smaller in size for neurons tuned to lower-frequency sound. Furthermore, GABAergic reversal potential also differed among the regions in accordance with the size of Kv1 current; it was less depolarized in the lower-frequency neurons with smaller Kv1 current. These differentiations of GABAergic transmission maximized the effects of inhibition at each tonotopic region, ensuring precise and reliable temporal coding across frequencies and intensities. Our results emphasize the importance of optimizing characteristics of GABAergic transmission within individual neurons for proper neural circuit function.

Background and objective Vestibular schwannoma is a benign tumour that originates in the eighth cranial nerve. It is termed intralabyrinthine schwannoma (ILS) when it develops in the inner ear, this being a rare origin. We present our experience in the management of three patients with ILS. Materials and methods The results of tumour excision and cochlear implantation were evaluated in three patients with ILS: two intracochlear schwannomas (ICS) and one intravestibular schwannoma (IVS). Results Prior to surgery, all patients presented progressive sensorineural hearing loss and tinnitus. Complete tumour resection and cochlear implantation was possible in all patients, with favourable hearing rehabilitation. Conclusions The therapeutic approach will depend on tumour size, growth rate, degree of hearing loss and presence of vestibular symptoms. Cochlear implantation (CI) in patients with ILS is possible when the cochlear nerve is present and functional. CI in patients, whether or not preceded by tumour excision, is an option with good hearing results in selected patients.

The human inner ear contains minute three-dimensional neurosensory structures that are deeply embedded within the skull base, rendering them relatively inaccessible to regenerative therapies for hearing loss. Here we provide a detailed characterisation of the functional architecture of the space that hosts the cell bodies of the auditory nerve to make them safely accessible for the first time for therapeutic intervention. We used synchrotron phase-contrast imaging which offers the required microscopic soft-tissue contrast definition while simultaneously displaying precise bony anatomic detail. Using volume-rendering software we constructed highly accurate 3-dimensional representations of the inner ear. The cell bodies are arranged in a bony helical canal that spirals from the base of the cochlea to its apex; the canal volume is 1.6 μL but with a diffusion potential of 15 μL. Modelling data from 10 temporal bones enabled definition of a safe trajectory for therapeutic access while preserving the cochlea’s internal architecture. We validated the approach through surgical simulation, anatomical dissection and micro-radiographic analysis. These findings will facilitate future clinical trials of novel therapeutic interventions to restore hearing.

Octopus cells are remarkable projection neurons of the mammalian cochlear nucleus, with extremely fast membranes and wide-frequency tuning. They are considered prime examples of coincidence detectors but are poorly characterized in vivo. We discover that octopus cells are selective to frequency sweep direction, a feature that is absent in their auditory nerve inputs. In vivo intracellular recordings reveal that direction selectivity does not derive from across-frequency coincidence detection but hinges on the amplitudes and activation sequence of auditory nerve inputs tuned to clusters of hot spot frequencies. A simple biophysical octopus cell model excited with real nerve spike trains recreates direction selectivity through interaction of intrinsic membrane conductances with the activation sequence of clustered excitatory inputs. We conclude that octopus cells are sequence detectors, sensitive to temporal patterns across cochlear frequency channels. The detection of sequences rather than coincidences is a much simpler but powerful operation to extract temporal information. ispartof: PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA vol:119 issue:44 ispartof: location:United States status: published

Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score ≤1 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score >1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

Background High-resolution MR imaging enables the visualization of individual nerves in the internal auditory canal (IAC). Cochlear nerve deficiency (CND) is recognized as one of the major causes of sensory neural hearing loss (SNHL), especially in cases of unilateral hearing loss in childhood. Some patients with CND are thought to have accompanying vestibular nerve deficiency (VND). However, there have been few reports focusing on VND and vestibular function in these children. Aims The aim of this study was to evaluate the frequency of VND and vestibular dysfunction in children with unilateral SNHL caused by CND. Material and methods Thirty-eight children with unilateral SNHL, who were diagnosed with CND by 3 T-MRI, were evaluated for VND and underwent caloric testing and cervical vestibular evoked potential (cVEMP). Results Fourteen of 38 patients (37%) had VND, and eleven (29%) of the patients [ten of the patients (71%) with VND] had at least one vestibular dysfunction. The patients with VND had significantly worse hearing and an IAC of smaller diameter than did patients without VND. Conclusions and significance We should pay attention to VND as well as vestibular dysfunction in hearing loss patients with CND.

OBJECTIVE To study the auditory outcome of simultaneous translabyrinthine vestibular schwannoma (VS) resection and cochlear implantation (CI) after successful cochlear nerve preservation. STUDY DESIGN A retrospective case series and patient questionnaire. SETTING Quaternary referral center for skull base pathologies. PATIENTS Patients with small (

Objective To compare conventional method and compressed‐sensing (CS) accelerated 3D balanced fast field echo imaging (bFFE) of inner ear. Methods Twenty patients with suspected inner ear disease underwent CS accelerated 3D‐bFFE (CS‐bFFE) and conventional 3D‐bFFE (Con‐bFFE) by a 3T MRI. The overall image quality, motion artifacts, and image quality of specific structures of inner ear were assessed on ordinal scales by three radiologists who were blinded to the scan protocols. Kendall W test was used to evaluate interobserver agreement and Wilcoxon test was performed to compare the image quality and motion artifacts between CS‐bFFE and Con‐bFFE. Results The acquisition duration of CS‐bFFE (1 min 53 s) was 49% faster than Con‐bFFE. Three radiologists had good inter‐observer agreement of image quality (Kendall W value of 0.829 for CS‐bFFE and 0.815 for Con‐bFFE) and motion artifacts evaluation (Kendall W value of 0861 for CS‐bFFE and 0.707 for Con‐bFFE). The better overall image quality of CS‐bFFE was assessed (4.93 ± 0.23 for CS‐bFFE, 4.53 ± 0.70 for Con‐bFFE, Z = −2.254, p = 0.024). The image quality score of facial and cochlear nerve gained higher in CS‐bFFE (4.93 ± 0.23 for CS‐bFFE, 4.58 ± 0.64 for Con‐bFFE, Z = −2.094, p = 0.036). No significant difference of motion artifacts (p = 0.050) between CS‐bFFE and Con‐bFFE. Conclusions The CS‐bFFE improves image quality and reduces acquisition time significantly, and it is a feasible MRI protocol for inner ear imaging.

Cochlear implants use electrical stimulation of the auditory nerve to restore the sensation of hearing to deaf people. Unfortunately, the stimulation current spreads extensively within the cochlea, resulting in “blurring” of the signal, and hearing that is far from normal. Current spread can be indirectly measured using the implant electrodes for both stimulating and sensing, but this provides incomplete information near the stimulating electrode due to electrode-electrolyte interface effects. Here, we present a 3D-printed “unwrapped” physical cochlea model with integrated sensing wires. We integrate resistors into the walls of the model to simulate current spread through the cochlear bony wall, and “tune” these resistances by calibration with an in-vivo electrical measurement from a cochlear implant patient. We then use this model to compare electrical current spread under different stimulation modes including monopolar, bipolar and tripolar configurations. Importantly, a trade-off is observed between stimulation amplitude and current focusing among different stimulation modes. By combining different stimulation modes and changing intracochlear current sinking configurations in the model, we explore this trade-off between stimulation amplitude and focusing further. These results will inform clinical strategies for use in delivering speech signals to cochlear implant patients.

Neural stimulation with infrared radiation has been explored for brain tissue, peripheral nerves, and cranial nerves including the auditory nerve. Initial experiments were conducted at wavelengths between λ = 1850 and λ = 2140 nm and the radiant energy was delivered with square pulses. Water absorption of the infrared radiation at λ = 1860 nm is similar to absorption at wavelengths between λ = 1310 and λ = 1600 nm, which are in the radiation wavelength range used for the communication industry. Technology for those wavelengths has already been developed and miniaturized and is readily available. The possibility of the infrared light to evoke compound action potentials (CAP) in the cochlea at λ = 1,375, λ = 1,460, and λ = 1550 nm was explored and compared to that of λ = 1860 nm in guinea pigs. Furthermore, rise and fall times of the 100 μs long pulses were changed and four basic pulse shapes (square, triangular, ramp-up, and ramp-down) were explored in their ability to evoke a CAP. In animals with pure tone threshold averages (PTAs) above 70 dB SPL, the results show that the favorable wavelength is λ = 1460 nm to reach threshold for stimulation and λ = 1375 nm or λ = 1460 nm for obtaining maximum amplitude. The most favorable pulse shape is either ramp-up or triangular.

The traditional method of recording electrically evoked compound action potentials (ECAPs), as implemented in the MAESTRO clinical software (standard ART), requires manual adjustments during threshold determination through a specialist. The "FineGrain" research tool (FineGrain RT) uses a continuous stimulation paradigm combined with automatic ECAP threshold determination. The aim of this study was to compare the FineGrain RT with standard ART.ECAPs were recorded with standard ART and the FineGrain RT in paediatric cochlear implant recipients. Different stimulation rates were used for FineGrain ECAP recordings (40, 60, and 76 Hz).Thirteen children (6 - 19 years) participated in this study - nine were bilaterally and four unilaterally implanted, resulting in a total of twenty-two implanted ears.ECAP threshold determination success rates were similar between the two approaches (92% and 89%) and ECAP thresholds correlated well (r: 0.94,TheFineGrain research approach is a reliable replacement for standard ART in clinical practice.

Human hearing loss is often accompanied by comorbidities like tinnitus. This perception of phantom sounds without external stimulation is affecting up to 15% of the adult population and can lead to psychological disorders like depression. Animal studies in rodents could show that tinnitus may not only be a result of hearing loss due to cochlear hair cell damage but can also be a consequence of synaptopathy at the inner hair cells already induced by moderate sound traumata (hidden hearing loss). In this study we investigate the synaptopathy previously shown in mice in our animal model, the Mongolian gerbil, and relate it to behavioral signs of tinnitus in this species. Tinnitus was induced by a mild monaural acoustic trauma leading to a monaural noise induced hearing loss in the animals quantified by auditory brainstem response audiometry. Behavioral signs of tinnitus percepts were detected by measurement of pre-pulse inhibition of the acoustic startle response in a gap-noise paradigm in about two-thirds of the animals. 14 days after trauma the cochleae of traumatized and non-traumatized ears were isolated and inner hair cell synapses were counted within several spectral regions of the cochlea. In the here presented preliminary data inner hair cell synaptopathy was only found in animals with behavioral signs of tinnitus, independent of type of hearing loss. On the other hand, animals with apparent hearing loss but without behavioral signs of tinnitus showed a reduction in amplitudes of auditory brainstem response waves I/II (cochlear nerve and dorsal cochlear nucleus) but no significant changes in the number of synapses at the inner hair cells. We conclude – in line with the literature – that hearing loss is caused by damage to the inner and / or outer hair cells but that the development of tinnitus, at least in our animal model is closely linked to synaptopathy at the inner hair cells.

Sporadic vestibular schwannomas (VS) can be treated using radiation therapy, microsurgical resection or a wait‐and‐scan approach. In general, small VS are usually followed (wait‐and‐scan) until a significant amount of growth per year is seen. In a recent study, 212 cases of VS were observed over time; 66% showed growth, of which 30% were fast growing (doubling in size in a year). 1 Frischer et al examined cases of VS after radiosurgery (Gamma Knife), and found a tumour control rate of 92% after 5 years of follow‐up. 2 However, among patients with initial Gardner‐Robertson class 1 or 2 hearing, only 55% maintained serviceable hearing (class 1 or 2) after 2 years, and 34% after 10 years. Several other studies have reported similar results. 3 , 4 A recent meta‐analysis concluded that among patients who undergo microsurgical treatment with a hearing‐preserving technique, 35–49% still have serviceable hearing five years later. 5 The above‐mentioned findings support the conclusion that most VS will require treatment after some period of observation. However, the currently available treatment options carry a high risk of loss of serviceable hearing. Some authors believe that the possibility of cochlear implantation in the setting of VS should completely change the treatment strategy for this disease. 6 However, patient outcomes with cochlear implants (CI) show a wide variation—with some patients having no auditory perception, while others exhibit open‐set speech understanding. 7 The intraoperative testing of cochlear nerve function using electrically evoked brainstem response audiometry (eABR) provides a means of objectively assessing cochlear nerve conduction, and has been increasingly applied in this setting. 7 , 8 In the present study, we aimed to assess outcomes of patients undergoing VS resection and CI, and to develop a new scoring system to preoperatively identify suitable patients for this treatment course. Appropriate preoperative classification of patients can have important impacts on patient counseling and expectations.

Objectives Amplitudes of electrically evoked compound action potentials (eCAPs) as a function of the stimulation level constitute the eCAP amplitude growth function (AGF). The slope of the eCAP AGF (i.e., rate of growth of eCAP amplitude as a function of stimulation level), recorded from subjects with cochlear implants (CIs), has been widely used as an indicator of survival of cochlear nerve fibers. However, substantial variation in the approach used to calculate the slope of the eCAP AGF makes it difficult to compare results across studies. In this study, we developed an improved slope-fitting method by addressing the limitations of previously used approaches and ensuring its application for the estimation of the maximum slopes of the eCAP AGFs recorded in both animal models and human listeners with various etiologies. Design The new eCAP AGF fitting method was designed based on sliding window linear regression. Slopes of the eCAP AGF estimated using this new fitting method were calculated and compared with those estimated using four other fitting methods reported in the literature. These four methods were nonlinear regression with a sigmoid function, linear regression, gradient calculation, and boxcar smoothing. The comparison was based on the fitting results of 72 eCAP AGFs recorded from 18 acutely implanted guinea pigs, 46 eCAP AGFs recorded from 23 chronically implanted guinea pigs, and 2094 eCAP AGFs recorded from 200 human CI users from 4 patient populations. The effect of the choice of input units of the eCAP AGF (linear versus logarithmic) on fitting results was also evaluated. Results The slope of the eCAP AGF was significantly influenced by the slope-fitting method and by the choice of input units. Overall, slopes estimated using all five fitting methods reflected known patterns of neural survival in human patient populations and were significantly correlated with speech perception scores. However, slopes estimated using the newly developed method showed the highest correlation with spiral ganglion neuron density among all five fitting methods for animal models. In addition, this new method could reliably and accurately estimate the slope for 4 human patient populations, while the performance of the other methods was highly influenced by the morphology of the eCAP AGF. Conclusions The novel slope-fitting method presented in this study addressed the limitations of the other methods reported in the literature and successfully characterized the slope of the eCAP AGF for various animal models and CI patient populations. This method may be useful for researchers in conducting scientific studies and for clinicians in providing clinical care for CI users.

Cochlear implants are considered the gold standard therapy for subjects with severe hearing loss and deafness. Cochlear implants bypass the damaged hair cells and directly stimulate spiral ganglion neurons (SGNs) of the auditory nerve. Hence, the presence of functional SGNs is crucial for speech perception in electric hearing with a cochlear implant. In deaf individuals, SGNs progressively degenerate due to the lack of neurotrophic support, normally provided by sensory cells of the inner ear. Adipose-derived stromal cells (ASCs) are known to produce neurotrophic factors. In a guinea pig model of sensory hearing loss and cochlear implantation, ASCs were autologously transplanted into the scala tympani prior to insertion of a cochlear implant on one side. Electrically evoked auditory brain stem responses (eABR) were recorded 8 weeks after cochlear implantation. At conclusion of the experiment, the cochleae were histologically evaluated. Compared to untreated control animals, transplantation of ASCs resulted in an increased number of SGNs and their peripheral neurites. In ASC-transplanted animals, mean eABR thresholds were lower and suprathreshold amplitudes larger, suggesting a larger population of intact auditory nerve fibers. Moreover, when compared to controls, amplitude-level functions of eABRs in ASC transplanted animals demonstrated steeper slopes in response to increasing interphase gaps (IPGs), indicative of better functionality of the auditory nerve. In summary, results suggest that transplantation of autologous ASCs into the deaf inner ear may have protective effects on the survival of SGNs and their peripheral processes and may thus contribute to long-term benefits in speech discrimination performance in cochlear implant subjects.

The synapse between inner hair cells and auditory nerve fiber dendrites shows large excitatory postsynaptic currents (EPSCs), which are either monophasic or multiphasic. Multiquantal or uniquantal (flickering) release of neurotransmitter has been proposed to underlie the unusual multiphasic waveforms. Here the nature of multiphasic waveforms is analyzed using EPSCs recorded in vitro in rat afferent dendrites. Spontaneous EPSCs were deconvolved into a sum of presumed release events having monophasic EPSC waveforms. Results include, first, the charge of EPSCs is about the same for multiphasic versus monophasic EPSCs. Second, EPSC amplitudes decline with the number of release events per EPSC. Third, there is no evidence of a mini-EPSC. Most results can be accounted for by versions of either uniquantal or multiquantal release. However, serial neurotransmitter release in multiphasic EPSCs shows properties that are not fully explained by either model, especially that the amplitudes of individual release events are established at the beginning of a multiphasic EPSC, constraining possible models of vesicle release. NEW & NOTEWORTHY How do monophasic and multiphasic waveshapes arise in auditory-nerve dendrites; mainly are they uniquantal, arising from release of a single vesicle, or multiquantal, requiring several vesicles? The charge injected by excitatory postsynaptic currents (EPSCs) is the same for monophasic or multiphasic EPSCs, supporting uniquantal release. Serial adaptation of responses to sequential EPSCs favors a multiquantal model. Finally, neurotransmitter partitioning into similar sized release boluses occurs at the first bolus in the EPSC, not easily explained with either model.

This is an update to a previously published scoping review on the subject matter from other Indian authors. It is widely known that tinnitus is difficult to cure. Hence, it is worthwhile to study its management trends across the globe. As there are equivocal views on the effectiveness of any particular therapies, it is not advisable to conduct a conventional interventional review oriented towards treatment and its outcomes. Therefore, we plan to conduct a scoping review to cover the best possible evidence from the health system perspective. Although there is a scoping review done in past on this subject by Makar SK and the team in 2017, we expect our study to include additional qualitative insights on disease management with a holistic viewpoint. The previous study did not use any research question mnemonic. But, we will use standard mnemonic recommended for scoping reviews by Joanna Briggs Institute of Evidence-based Medicine. Also, Makar SK et al. provided no information about the registration of their scoping review protocol. Hence, this updated, systematic method of scoping review is envisaged to include comprehensive evidence on the subject matter with a more scientific approach .

Preservation of hearing is one of the tenets of vestibular schwannoma management. In recent years, cochlear implants have been employed with increasing use in patients who have suffered profound sensorineural hearing loss due to the natural history of vestibular schwannoma or due to injury to neurovascular anatomy at time of surgical resection.Cochlear implantation has been found to be an effective modality for hearing restoration following vestibular schwannoma. Simultaneous cochlear implantation has been employed by an increased number of centers around the world and has been shown to provide restoration of open set speech perception and return of binaural hearing. Ongoing use of electrically evoked auditory brainstem response (ABR) has improved our detection of viable cochlear nerves and provided insight into those who would benefit from this procedure. Finally, minimally invasive approaches to the internal auditory canal and intralabyrinthine tumors have been described. These methods frequently employ simultaneous cochlear implantation and have emphasized that hearing preservation remains possible with surgical excision despite the location of the tumor.Cochlear implantation is an effective modality for hearing restoration following vestibular schwannoma excision.

Detection of cochlear nerve deficiency (CND) is usually straightforward using magnetic resonance imaging (MRI). In patients in whom MRI cannot be performed or imaging provides equivocal findings, computed tomography (CT) of the temporal bone might offer indirect evidence of CND. Our study aimed to derive a cut-off value for the diameter of the cochlear nerve canal (CNC) and internal auditory canal (IAC) in temporal bone CT to predict CND.This retrospective study included 70 children with sensorineural hearing loss (32 with CND and 38 control patients). The height, width, and cross-sectional area of the IAC and diameter of the CNCs were determined using temporal bone CT. Receiver operating characteristic (ROC) and Student's t-tests were performed for each parameter.The mean diameter of the CNCs was significantly smaller in children with CND than in the control group (1.2 mm versus 2.4 mm, p .001). The optimal threshold for CNC for separation of the two groups was 1.9 mm, resulting in a sensitivity of 98.7 % and specificity of 89.2 %. The IAC dimensions could not distinguish between children with CND and controls.A CNC diameter of less than 1.9 mm is a reliable predictor of CND in children with sensorineural hearing loss.· A small cochlear nerve canal predicts cochlear nerve deficiency (CND). · The size of the internal auditory canal cannot predict CND. · Whenever MRI is impossible or ambigous, CT can rule out CND.· Sorge M, Sorge I, Pirlich M et al. Diameter of the Cochlear Nerve Canal predicts Cochlear Nerve Deficiency in Children with Sensorineural Hearing Loss. Fortschr Röntgenstr 2022; 194: 1132 - 1139.ZIEL: Eine Nervus-Cochlearis-Defizienz (CND) kann mittels Magnetresonanztomografie (MRT) in der Regel einfach diagnostiziert werden. Bei Patienten, bei denen eine MRT kontraindiziert ist oder keine eindeutigen Ergebnisse erbringt, kann die Computertomografie (CT) des Schläfenbeins einen indirekten Nachweis der CND liefern. Ziel unserer Studie war es, einen Schwellenwert für den Durchmesser des Canalis nervi cochlearis (CNC) und des inneren Gehörgangs (IAC) im Schläfenbein-CT für die Vorhersage einer CND zu ermitteln. An der retrospektiven Studie nahmen 70 Kinder mit Innenohrschwerhörigkeit teil (32 mit CND und 38 Kontrollpatienten). Die Höhe, Breite und Querschnittsfläche der IAC und der Durchmesser der CNC wurden mittels Schläfenbein-CT bestimmt. Für jeden Parameter wurde eine Receiver Operating Characteristic (ROC) und ein Studentʼs t-Tests durchgeführt. Der mittlere Durchmesser der CNCs war bei Kindern mit CND deutlich kleiner als in der Kontrollgruppe (1,2 mm gegenüber 2,4 mm, p .001). Der optimale Schwellenwert zur Unterscheidung der beiden Gruppen lag für den CNC bei 1,9 mm mit einer Sensitivität von 98,7 % und einer Spezifität von 89,2 %. Die Dimensionen des IAC waren für die Unterscheidung zwischen Kindern mit und ohne CND nicht geeignet. Ein CNC-Durchmesser unter 1,9 mm ist ein zuverlässiger Prädiktor für CND bei Kindern mit sensorineuralem Hörverlust. · Ein kleiner Canalis nervi cochlearis ist ein exzellenter Prädiktor einer Nervus-Cochlearis-Defizienz (CND). · Die Größe des inneren Gehörgangs hingegen ist kein guter Prädiktor. · Wenn eine MRT kontraindiziert ist, kann die CT eine CND ausschließen.

To stimulate the auditory nerve, cochlear implants directly inject electrical current into surrounding tissue via an implanted electrode array. While many cochlear implant users achieve strong speech perception scores, there remains significant variability. Since cochlear implant electrode arrays are surrounded by a conductive fluid, perilymph, a spread of excitation occurs. The functionality of the cochlea is spatially dependent, and a wider area of excitation negatively affects the hearing of the user. Importantly, magnetic fields are unaffected by the material properties of biological components. To utilize the electromagnetic properties of the human ear, a microcoil array was developed. The microcoils are 4-turn solenoids with a 250- [Formula: see text] turn radius and a 31.75- [Formula: see text] wire radius, coated with Parylene-C. The efficient design was implemented to accelerate testing. The obtained results describe stimulation capabilities. Functionality was validated using a frequency response analyzer to measure how the generated electromagnetic power radiates in space. 99.8% power loss was observed over a 100- [Formula: see text] separation between a pair of identical microcoils. Obtained through finite-element modeling, the microcoils can be driven by a 60 mA, 5 kHz, sinusoidal input for 10 minutes before predicted inflammation. Rattay's activating function was calculated to evaluate the magnetic stimulation effect of external fields on target neurons. Combined with the frequency response analysis, magnitude and spatial effects of the generated potential is established. As a result, each microcoil requires a 400- [Formula: see text]-wide area for each independent stimulation channel, which is 84% narrower than a commercial cochlear array channel, thereby suggesting greater spatial selectivity.

Determining the etiology of severe-to-profound sensorineural hearing loss (SP-SNHL) in pediatric subjects is particularly important in aiding the decision for auditory rehabilitation. We aimed to update the etiologic spectrum of pediatric SP-SNHL by combining internal auditory canal (IAC)-MRI with comprehensive and state-of-the-art genetic testings. From May 2013 to September 2020, 119 cochlear implantees under the age of 15 years with SP-SNHL were all prospectively recruited. They were subjected to genetic tests, including exome sequencing, and IAC-MRI for etiologic diagnosis. Strict interpretation of results were made based on ACMG/AMP guidelines and by an experienced neuroradiologist. The etiology was determined in of 65.5% (78/119) of our cohort. If only one of the two tests was done, the etiologic diagnostic rate would be reduced by at least 21.8%. Notably, cochlear nerve deficiency (n = 20) detected by IAC-MRI topped the etiology list of our cohort, followed by DFNB4 (n = 18), DFNB1 (n = 10), DFNB9 (n = 10) and periventricular leukomalacia associated with congenital CMV infection (n = 8). Simultaneous application of state-of-the-art genetic tests and IAC-MRI is essential for etiologic diagnosis, and if lesions of the auditory nerve or central nerve system are carefully examined on an MRI, we can identify the cause of deafness in more than 65% of pediatric SP-SNHL cases.

Review the effectiveness of an alternative mapping procedure of a precurved electrode array in the internal auditory canal (IAC).A 7-year-old bilateral cochlear implant (CI) recipient of precurved arrays transferred to the study site and demonstrated no speech recognition with the left CI. Imaging revealed bilateral incomplete partition type III malformations. For the left CI, four contacts were observed in the basal cochlear turn and 18 contacts were coiled in the IAC. The family decided against revision surgery. Pitch ranking was completed to map the contacts in the IAC that were perceptually discrete and tonotopically organized.For the left CI, PB-K word recognition improved from no recognition to 32% after 1 month and to 52% after 3 months. In the bilateral CI condition, performance improved from 56 to 72% after 1 month and 80% after 3 months.A precurved array in the IAC creates difficult management decisions. Direct stimulation of the auditory nerve resulted in better monaural and bilateral speech recognition, likely due to better spectral representation of the speech signal. Individualizing the map using imaging and behavioral findings may improve performance for malpositioned arrays when revision surgery is not pursued.

Összefoglaló. Bevezetés: A cochlearis implantátum egy műtétileg behelyezett elektromos eszköz, amely az akusztikus hanghullámokat elektromos jelekké alakítja, közvetlenül a hallóideget stimulálja, így segíti a súlyos fokú hallássérüléssel vagy teljes hallásvesztéssel élők életét. Cochlearis implantációt követően a legjobb rehabilitációs eredmény elérésének technikai feltétele többek között az esetre szabott elektródaválasztás és az elektródasor teljes, kontrollált, szövődménymentes bejuttatása a scala tympaniba, miközben a cochlea belső struktúrája a lehető legkisebb mértékben sérül. A rutin intraoperatív elektrofiziológiai tesztek fontos információt adnak a készülék működőképességéről és a hallóideg stimulációjáról, azonban nem hagyatkozhatunk rájuk az elektródasor cochleán belüli helyzetének igazolásában. Mivel előfordulhat, hogy a rendelkezésre álló elektrofiziológiai vizsgálatok eredménye megfelelő, és mégis rendellenes helyzetbe kerül az elektróda, az arany standardot a képalkotó vizsgálatok jelentik. Módszer: Közleményünkben egy modern, hibrid műtő által nyújtott technológiai háttér új alkalmazási területét mutatjuk be. Szimultán kétoldali cochlearis implantációt végeztünk Cochlear Nucleus Slim Modiolar típusú perimodiolaris elektródasorral, a belső fül fejlődési rendellenességével rendelkező betegen. Az intraoperatív képalkotást Siemens Artis pheno C-karos robot digitális szubtrakciós angiográfiás rendszer biztosította valós idejű átvilágító és volumentomográfiás funkcióval. Eredmények: Az intraoperatív képalkotás által dinamikusan követhető az elektródasor bevezetésének folyamata, ellenőrizhető az elektródasor statikus helyzete, így kiváltható a rutinnak számító posztoperatív képalkotó vizsgálat. A rendellenes helyzetbe kerülő elektródasor pozíciója egy ülésben korrigálható, az újból bevezethető, így elkerülhető az újabb altatással járó, bizonytalan kimenetelű revíziós műtét. Következtetés: A hibrid műtő jól kontrollált, minimálisan invazív eljárások elvégzését biztosítja. Különösen a hallószerv fejlődési rendellenessége vagy egyéb, az elektródának a cochleába vezetését nehezítő rendellenesség esetén javasolt a műtői képalkotó diagnosztika. Orv Hetil. 2021; 162(22): 878–883. Summary. Introduction: The cochlear implant is a surgically inserted electrical device that converts acoustic sound waves into electrical signals to stimulate the cochlear nerve, thus helps the rehabilitation of people with severe to total hearing loss. One of the most important technical conditions for achieving the best rehabilitation result after cochlear implantation is the personalized choice of electrodes. Additionally, it is vital that there is a complete, controlled, uncomplicated delivery of the electrode array to the scala tympani while minimizing damage to the inner structures of the cochlea. Routine electrophysiological tests provide important information about device functionality and auditory nerve stimulation. However, they probably do not show an abnormal position of the electrode array within the cochlea. Thus, imaging studies remain the gold standard. Method: In our paper, we present a novel application field of the modern technological background provided by a hybrid operating room. Simultaneous bilateral cochlear implantation was performed with cochlear implants with perimodiolar electrode array (Nucleus Slim Modiolar) in a patient with cochlear malformation. Intraoperative imaging was provided by a Siemens Artis pheno C-arm robot digital subtraction angiography system with real-time fluoroscopy and volume tomography function. Results: Intraoperative imaging ensures dynamic follow-up of the introduction and static determination of the position of the electrode array and replaces routine postoperative imaging. If the electrode array was inserted in an abnormal position, the revision can be performed in the same sitting. Also, the revision surgery with a potential risk of uncertain outcome, alongside additional anaesthesia, can be prevented. Conclusion: The hybrid operating room ensures that well-controlled, minimally invasive procedures are performed. Intraoperative imaging can be imperative in malformed cochleae and conditions that may complicate electrode insertion. Orv Hetil. 2021; 162(22): 878–883.

Cochlear nerve deficiency is one of the known causes of congenital sensorineural hearing loss. Management of hearing loss in children with cochlear nerve deficiency poses a multidimensional challenge. The absent or hypoplastic cochlear nerve may prevent electrical stimulation from reaching the brainstem and the auditory cortex. A deficient cochlear nerve can be associated with other inner ear malformations, which may diminish the success of cochlear implantation in those children. Promising results in adults after auditory brainstem implantation led to the expansion of candidacy to include the pediatric populations who were contraindicated for CIs. To review the outcomes of cochlear implantation versus that of auditory brainstem implantation in children with various conditions of the auditory nerve. This retrospective chart review study comprised two pediatric groups. The first group consisted of seven ABI recipients with cochlear nerve aplasia and the second group consisted of another seven children with cochlear nerve deficiencies who underwent CI surgery. The participants’ auditory skills and speech outcomes were assessed using different tests selected from the Evaluation of Auditory Responses to Speech (EARS) test battery. There were some individual variations in outcomes depending on the status of the auditory nerve. The mean CAP score of the ABI group was 2.87, while the mean SIR score was 0.62. On the other hand, the mean CAP score of the CI group was 1.29, while the mean SIR score was 0.42. Our results are in good agreement with the reported auditory perception and speech and language development outcomes of pediatric auditory brainstem implantation. We added to the growing body of literature on the importance of verifying and identifying the status of the cochlear nerve in the decision-making process of the surgical management of those pediatric groups.