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Traditional approaches to the human cochlear nerve have been impeded by its bony encasement deep inside the skull base. We present an innovative, minimally invasive, therapeutic pathway for direct access to the nerve to deliver novel regenerative therapies. Neuroanatomical studies on 10 cadaveric human temporal bones were undertaken to identify a potentially safe therapeutic pathway to the cochlear nerve. Simulations based on three-dimensional delineation of anatomical structures obtained from synchrotron phase-contrast imaging were analyzed. This enabled the identification of an approach to the nerve in the fundus of the internal auditory meatus by trephining the medial modiolar wall of the cochlea via the round window for a median depth of 1.48 mm (range 1.21–1.91 mm). The anatomical access was validated on 9 additional human temporal bones using radio-opaque markers and contrast injection with micro-computed tomography surveillance. We thus created an effective conduit for the delivery of therapeutic agents to the cochlear nerve. [ABSTRACT FROM AUTHOR]

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Abstract Traditional approaches to the human cochlear nerve have been impeded by its bony encasement deep inside the skull base. We present an innovative, minimally invasive, therapeutic pathway for direct access to the nerve to deliver novel regenerative therapies. Neuroanatomical studies on 10 cadaveric human temporal bones were undertaken to identify a potentially safe therapeutic pathway to the cochlear nerve. Simulations based on three-dimensional delineation of anatomical structures obtained from synchrotron phase-contrast imaging were analyzed. This enabled the identification of an approach to the nerve in the fundus of the internal auditory meatus by trephining the medial modiolar wall of the cochlea via the round window for a median depth of 1.48 mm (range 1.21–1.91 mm). The anatomical access was validated on 9 additional human temporal bones using radio-opaque markers and contrast injection with micro-computed tomography surveillance. We thus created an effective conduit for the delivery of therapeutic agents to the cochlear nerve.

Background: Quick and appropriate diagnostics and the use of intraoperative monitoring (IM) of hearing during vestibular schwannoma (VS) resection increase the likelihood of hearing preservation. During surgery, various methods of IM can be used, i.e., auditory brainstem responses (ABRs), transtympanic electrocochleography (TT-ECochG), and direct cochlear nerve action potentials. The aim of the study was to evaluate the prognostic values of IM of hearing using ABR and TT-ECochG in predicting postoperative hearing preservation and to evaluate relationships between them during various stages of surgery. Methods: This retrospective study presents the pre- and postoperative audiological test results and IM of hearing records (TT-ECochG and ABR) in 75 (43 women, 32 men, aged 18–69) patients with diagnosed VS. Results: The preoperative pure tone average hearing threshold was 25.02 dB HL, while after VS resection, it worsened on average by 30.03 dB HL. According to the American Academy of Otolaryngology–Head and Neck Surgery (AAO—HNS) Hearing Classification, before and after (pre/post) surgery, there were 47/24 patients in hearing class A, 9/8 in B, 2/1 in C, and 17/42 in D. In speech audiometry, the average preoperative speech discrimination score at an intensity of 60 dB SPL was 70.93%, and after VS resection, it worsened to 38.93%. The analysis of electrophysiological tests showed that before the tumor removal the I–V ABR interlatencies was 5.06 ms, and after VS resection, it was 6.43 ms. Conclusions: The study revealed correlations between worse postoperative hearing and changes in intraoperatively measured ABR and TT-ECochG. IM of hearing is very useful in predicting postoperative hearing in VS patients and increases the chance of postoperative hearing preservation in these patients. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this retrospective study is to compare the results of electrically evoked compound action potential (ECAP) measurements using automatic auditory response telemetry (AutoART) with those obtained by ART in adults. The study also aimed to evaluate the predictive value of intraoperative ART and AutoART ECAPs for speech intelligibility (SI) and hearing success (HS), and to determine if cochlear nerve (CN) cross-sectional area (CSA) obtained preoperatively by magnetic resonance imaging (MRI) scans could predict ART and AutoART ECAPs and SI and HS outcome. Methods: The study analyzed and correlated ART and AutoART ECAP thresholds at electrodes E2, E6, and E10, as well as averaged ECAP thresholds over electrodes E1–E12, using data from 32 implants. Correlations were also examined for ART and AutoART ECAP slopes. In addition, averaged ART and AutoART ECAP thresholds and slopes over all 12 electrodes for each participant were correlated with CN CSA measured from MRI sequences. SI of the monosyllabic Freiburg Speech Test at 65 dB sound pressure level was examined along with averaged ART and AutoART thresholds and slopes over all 12 electrodes. A parallel analysis was performed for HS, derived from the difference between baseline and 6-month SI. Finally, correlations between CN CSA and SI, as well as CN CSA and HS were examined. Results: The results of the study showed a significant positive correlation between ART and AutoART ECAP thresholds and as well as slopes for E2, E6, E10 and averaged thresholds and slopes of E1–E12. However, no significant correlation was observed between ART and AutoART averaged ECAP thresholds and slopes and either SI and HS or CN CSA. Furthermore, no significant correlation was found between CN CSA and SI and HS. Conclusion: While AutoART is a reliable and safe program for measuring ECAPs in adults, the study found no preoperative prognostic information on intraoperative ECAP results using parameters extracted from current MRI sequences or pre-/intraoperative information on subsequent hearing outcome using ECAP and CN CSA. [ABSTRACT FROM AUTHOR]

Background and Objectives: Idiopathic sudden sensorineural hearing loss (ISSHL) is a rapid loss of hearing, exceeding 30 dB in at least 3 consecutive frequencies within 3 days, without any identifiable cause despite thorough investigations. Currently, the etiology and pathogenesis of ISSHL have not been fully elucidated. This study aimed to assess the size of the cochlear nerve in patients with ISSHL and explore its relationship with pretreatment audiograms and treatment response. Subjects and Methods: A total of 125 patients (59 [47.2%] women; mean age 47.7±13.8 years [minimum-maximum: 21-76]) and 60 healthy participants (27 [45%] women; mean age 45.7±16.8 years [minimum-maximum: 20-76]) as a control group were included in this study. The size of the cochlear nerve was assessed on the affected side, compared to the control group, as well as on the unaffected side. Pretreatment and posttreatment audiological values were also analyzed. Results: The cross-sectional area (CSA), vertical diameter (VD), and horizontal diameter (HD) of the CN were found to be smaller on the affected side of ISSHL patients compared to the control group (p<0.01; p=0.04; p=0.02, respectively). In the study group (affected side of ISSHL patients), there were no significant differences in VD, HD, and CSA values between pretreatment audiogram types (p=0.23; p=0.53; p=0.39, respectively), and initial hearing levels (p=0.16; p=0.22; p=0.23, respectively). Furthermore, there were no significant differences in VD, HD, and CSA values between the recovery groups according to Furuhashi criteria (p=0.18; p=0.37; p=0.27, respectively). Conclusions: The size of the CN may be a risk factor for ISSHL, but it does not affect the type of audiogram curves and was not prognostic in terms of treatment response. [ABSTRACT FROM AUTHOR]

Background: Cochlear implantation (CI) outcomes in patients with auditory neuropathy (AN) are variable, which hampers patients' decisions on CI. Objective: This study aims to assess the outcomes of CI in individuals diagnosed with AN and to examine the various factors that may influence the effectiveness of this intervention. Methods: A total of 75 patients diagnosed with AN were included in the study. The hearing threshold, the score of categories of auditory performance (CAP), speech intelligibility rating (SIR), and speech audiometry were tested. Genetic testing was conducted by medical exome sequencing in 46 patients. Results: After CI, the average aided hearing threshold for patients with prelingual and post-lingual onset was 38.25 ± 6.63 dB and 32.58 ± 9.26 dB, respectively; CAP score improved to 5.52 ± 1.64 (p < 0.001) and 6.00 ± 0.96 (p < 0.001), respectively; SIR score increased to 3.57 ± 1.22 (p < 0.001) and 4.15 ± 0.95 (p < 0.001), respectively. Maximum speech recognition ranged from 58 to 93% for prelingual onset patients and 43 to 98% for those with post-lingual onset. Speech outcomes of CI in cases with cochlear nerve (CN) deficiency were significantly poorer (p = 0.008). Molecular etiologies, including TWIST1, ACTG1, m.A7445G, and a copy-number variant (CNV) carrying ACTB, were related to AN here. Conclusion: CI is a viable therapy option for patients with AN; CN deficiency might impact outcomes of CI. [ABSTRACT FROM AUTHOR]

The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved using intraoperative EMG monitoring, hearing preservation remains challenging, with the recent use of cochlear nerve action potential (CNAP) monitoring. This prospective longitudinal series of VS with useful hearing operated on using a retrosigmoid approach included 37 patients with a mean largest extrameatal VS. diameter of 25 ± 8.7 mm (81% of Koos stage 4). CNAP was detected in 51% of patients, while auditory brainstem responses (ABR) were present in 22%. Patients were divided into two groups based on the initial intraoperative CNAP status, whether it was present or absent. FN function was preserved (grade I–II) in 95% of cases at 6 months. Serviceable hearing (class A + B) was preserved in 16% of the cases, while 27% retained hearing with intelligibility (class A–C). Hearing with intelligibility (class A–C) was preserved in 42% of cases when CNAP could be monitored in the early stages of VS resection versus 11% when it was initially absent. Changes in both the approach to the cochlear nerve and VS resection are mandatory in preserving CNAP and improve the rate of hearing preservation. [ABSTRACT FROM AUTHOR]

Background: Giant vestibular schwannoma (VS) represents one of the most challenging interventions in skull base surgery. Preserving facial and cochlear nerves requires effective surgical planning and nuanced techniques. Objectives: The present study evaluates the role of intraoperative neuromonitoring (IONM) in predicting the outcome of facial and cochlear nerves. Methods: This retrospective cohort comprised 34 patients with a giant (Samii IV/IVb grade) VS during 2016 - 2019. The retrosigmoid approach was used for tumor resection. Pre- and postoperative facial exams were graded according to the House-Brackmann Scale. Hearing ability was also classified according to the Hannover Hearing Classification. Intraoperative findings included the location of the nerve complex, tumor consistency, the surgical plane of the tumor from the facial nerve/brainstem, and the level of internal auditory canal (IAC) decompression. Intraoperative neuromonitoring was used for all surgeries. The amplitude required for a positive response was recorded during the facial nerve's direct electrical stimulation (DES). All patients were followed 6 and 12 months postoperative. Results: The preoperative facial nerve function was normal in 91.2% of patients, and 8.8% had slight facial nerve impairment (FNI). Considering preoperative hearing status, 9 cases (26.5%) had moderate hearing loss or impairment, while 25 patients (73.5%) complained of severe hearing impairment. The mean intraoperative direct facial nerve stimulation threshold was 1.38 ± 0.89. Hearing impairment was moderately severe in 7 (20.6%) and severe in 25 patients (73.5%) postoperation. In addition, postoperative assessment revealed 13 (38.2%) cases with moderate FNI and 12 (35.2%) with moderately severe FNI. The tumor size did not affect the postoperative hearing loss, and the postoperative hearing loss did not improve significantly (P = 0.32). There was no statistically significant correlation between the intensity of intraoperative DES and postoperative facial nerve function (P> 0.05). Nosignificant correlation was observed between tumor consistency and postoperative hearing status (P > 0.05). Conclusions: The retrosigmoid approach combined with IONM appears to be associated with favorable facial and cochlear nerve outcomes in giant VS. The postoperative facial nerve function may improve, but sensorineural hearing status may not improve significantly. Moreover, the intensity of intraoperative DESmaynot predict the postoperative facial function. Therefore, the findings of IONM should be interpreted carefully. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to evaluate the relationship between the cochlear nerve and the anatomical structures of the cochlea and internal acoustic canal in patients with congenital hearing loss. Materials and Methods: Temporal tomography and magnetic resonance images of 44 patients (88 ears) with non-syndromic congenital hearing loss were retrospectively analyzed between 2018 and 2021. Patients were divided into 2 groups according to cochlear nerve hypoplasia. Cochlear nerve canal width, cochlear basal/ middle turn widths, and internal auditory canal widths were examined. Results: Cochlear nerve hypoplasia was detected in 18.2% (n = 16) of the patients and all of the patients with cochlear nerve hypoplasia had severe hearing loss. A statistically significant difference was found between the structures' widths in patients with and without cochlear nerve hypoplasia, in cochlear nerve canal and coronal width of the internal auditory canal. When stenosis is accepted as <1.4 mm for cochlear nerve canal and <3.80 mm for coronal width of the internal auditory canal, cochlear nerve hypoplasia differs statistically between the groups in measurements (respectively; P < .001, P = .018). Conclusions: In patients with sensorineural hearing loss, cochlear nerve hypoplasia may accompany. Anatomical structures are important in predicting cochlear nerve hypoplasia from temporal computed tomography. Cochlear nerve hypoplasia should be suspected if the cochlear nerve canal and coronal width of the internal auditory canal are less than 1.4 mm and 3.8 mm, respectively, on temporal computed tomography. [ABSTRACT FROM AUTHOR]

Introduction:Incomplete partition (IP) type and accompanying cochlear nerve (CN) anomalies affect the patient’s management. We revealed the cochlear nerve area (CNA), cochlear nerve canal width (CNCW), and inner auditory canal width (IACW) of IP types.Methods:We retrospectively scanned patients with IP. There were 88 IP ears (26 IP type 1, 54 IP type 2, 8 IP type 3) and 54 controls. The CNCW and IACW were measured in axial temporal computed tomography sections. The CNA and facial nerve area (FNA) were measured in the distal IAC on the sagittal-oblique plane of 3D constructive interference steady-state T2-weighted magnetic resonance images.Results:CNA and CNA/FNA values for each IP type differed significantly compared with the control group. However, the CNCW and IACW values did not differ significantly. The CNA was the least in IP 1 cases. Five CN aplasia were detected, and all were associated with IP type 1 anomaly (3.5% of all, 5.6% of IP types, and 19.2% of IP type 1 cases). CN hypoplasia was observed in 10 IP type 1 (38.5% of IP 1), 6 IP type 2 (6.8% of IP type 2), and 1 IP type 3 (12.5% of IP type 3) ears. None of the CN hypoplasia had a CNC hypoplasia.Conclusion:CN aplasia and hypoplasia most frequently accompanied with IP type 1 in our study. Therefore, they need an extra interest in CN evaluation. CNCW and IACW are not very useful in predicting CN dysplasia in IP cases.

BACKGROUND: Idiopathic sudden sensorineural hearing loss is a disabling condition that lowers the quality of life specifically in older adults living alone. It is crucial to determine the outcome of the disease and to offer early treatment to prevent isolation caused by hearing impairment in this population. The objective of our study was to investigate whether the initial cochlear nerve thickness may predict the outcome of hearing recovery in older adults with idiopathic sudden sensorineural hearing loss. METHODS: The study population was composed of older adults that were referred with idiopathic sudden sensorineural hearing loss in 1 ear. Long-term audiological data of the cohort were analyzed according to Siegel's criteria on hearing recovery and were grouped according to complete recovery or treatment failure. Cochlear nerve diameters of the diseased and safe ears of each group, measured on reformatted images on magnetic resonance imaging, at the fundus, in the mid-internal acoustic canal, and at the entry point into the Pons were compared in each group and between groups. RESULTS: Mean cochlear nerve diameter was significantly larger in the recovered older adults (1.11 ± 0.27 mm) than in the non-recovered adults (0.94 ± 0.21 mm) at the mid-internal acoustic canal (Student's t-test, P < .05). Cochlear nerve thickness at mid-internal acoustic canal (≤0.8 mm) sensitivity for recovery failure was 89% and displayed an odds ratio 5.333, 95% CI (1.000-28.435). CONCLUSION: Cochlear nerve thickness in mid-internal acoustic canal in non-recovered older adults with idiopathic sudden sensorineural hearing loss is significantly thinner than the completely recovered group. Older adults with mid-internal acoustic canal cochlear nerve greatest diameter cutoff level of ≤0.8 mm are 5.33 times more exposed to recovery failure. [ABSTRACT FROM AUTHOR]

Despite the absence of tympanic middle ears, snakes can hear. They are thought to primarily detect substrate vibration via connections between the lower jaw and the inner ear. We used the western rat snake (Pantherophis obsoletus) to determine how vibration is processed in the brain. We measured vibration‐evoked potential recordings to reveal sensitivity to low‐frequency vibrations. We then used tract tracing combined with immunohistochemistry and Nissl staining to describe the central projections of the papillar branch of the VIIIth nerve. Applications of biotinylated dextran amine to the basilar papilla (homologous to the organ of Corti of mammals) labeled bouton‐like terminals in two first‐order cochlear nuclei, a rostrolateral nucleus angularis (NA) and a caudomedial nucleus magnocellularis (NM). NA formed a distinct dorsal eminence, consisted of heterogenous cell types, and was parvalbumin positive. NM was smaller and poorly separated from the surrounding vestibular nuclei. NM was distinguished by positive calbindin label and included fusiform and round cells. Thus, the atympanate western rat snake shares similar first‐order projections to tympanate reptiles. Auditory pathways may be used for detecting vibration, not only in snakes but also potentially in atympanate early tetrapods. [ABSTRACT FROM AUTHOR]

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination.

Objective To explore the application of membranous concept in vestibular schwannoma surgery. Methods A total of 39 patients with vestibular schwannoma who received membranous surgical treatment in The First Affiliated Hospital of Anhui Medical University from May 2020 to June 2021 were enrolled. Through the retrosigmoid approach, the intraoperative electrophysiological monitoring was performed, and the membranous concept was used to separate the brain stem of the tumor and the interface between the tumor and the facial nerve and the cochlear nerve. Facial nerve function was evaluated by the House⁃Brackmann (H⁃B) grade and hearing was evaluated by the American Academy of Otolaryngology⁃Head and Neck Surgery（AAO⁃HNS）scale at one day, one week and one year after surgery. Results Total resection was performed in 31 cases (79.49%) and nearly total resection in 8 cases (20.51%). Facial nerve anatomical preservation was achieved in all patients, and facial nerve function returned to normal or nearly normal (H⁃B grade Ⅰ-Ⅱ) 84.62% (33/39), moderate facial paralysis (H⁃B grade Ⅲ) 10.27% (4/39), moderate and severe facial paralysis (H⁃B grade Ⅳ) 5.13% (2/39) on the first day after surgery. One week after surgery, 71.79% (28/39) of patients with normal or nearly normal facial nerve function, 15.38% (6/39) of moderate facial paralysis, 10.27% (4/39) of moderate and severe facial paralysis, and 2.56% (1/39) of severe facial paralysis (H⁃B grade Ⅴ). One year after surgery, 92.31% (36/39) of patients with facial nerve function returned to normal or nearly normal, 5.13% (2/39) of patients with moderate facial paralysis, and 2.56% (1/39) of patients with moderate and severe facial paralysis. Among the 10 patients (25.64%) who retained effective hearing before surgery (AAO⁃HNS grade≥C), there were 4, 3 and 6 cases at one day, one week and one year after surgery, respectively. Those who did not retain effective hearing before surgery did not recover effective hearing after surgery. According to the comparison of tumor volume and nature, the proportion of patients with normal facial nerve function (H⁃B grade Ⅰ) in the tumor volume≤3 cm group was higher than that in＞3 cm group [12/18 vs. 19.05% (4/21); Fisher's exact probability: P=0.006], the proportion of patients with normal facial nerve function in the solid tumor group was higher than that in cystic group [71.43% (15/21) vs. 1/10; Fisher's exact probability: P=0.010] at one year after surgery. Conclusions The membranous concept in vestibular schwannoma surgery can protect facial nerve function well, and has a positive effect on the protection of cochlear nerve and brain stem.

Introduction: Incomplete partition (IP) type and accompanying cochlear nerve (CN) anomalies affect the patient's management. We revealed the cochlear nerve area (CNA), cochlear nerve canal width (CNCW), and inner auditory canal width (IACW) of IP types. Methods: We retrospectively scanned patients with IP. There were 88 IP ears (26 IP type 1, 54 IP type 2, 8 IP type 3) and 54 controls. The CNCW and IACW were measured in axial temporal computed tomography sections. The CNA and facial nerve area (FNA) were measured in the distal IAC on the sagittal-oblique plane of 3D constructive interference steady-state T2-weighted magnetic resonance images. Results: CNA and CNA/FNA values for each IP type differed significantly compared with the control group. However, the CNCW and IACW values did not differ significantly. The CNA was the least in IP 1 cases. Five CN aplasia were detected, and all were associated with IP type 1 anomaly (3.5% of all, 5.6% of IP types, and 19.2% of IP type 1 cases). CN hypoplasia was observed in 10 IP type 1 (38.5% of IP 1), 6 IP type 2 (6.8% of IP type 2), and 1 IP type 3 (12.5% of IP type 3) ears. None of the CN hypoplasia had a CNC hypoplasia. Conclusion: CN aplasia and hypoplasia most frequently accompanied with IP type 1 in our study. Therefore, they need an extra interest in CN evaluation. CNCW and IACW are not very useful in predicting CN dysplasia in IP cases. [ABSTRACT FROM AUTHOR]

The Trans-labrynthine approach, through Otic capsule gives direct approach to the cerebellopontine angle (CPA) and internal auditory meatus (IAM) with preservation of the facial nerve. Mastering the approach to IAM in a cadaver with anatomical landmarks is important to the budding Otologist & Neurotologist to approach the CPA with functional preservation of the Facial nerve in patients with Vestibular Schwannoma & other procedures. Transitioning surgical skills and anatomic knowledge from surgical anatomy textbooks and laboratory training to the operative room is challenging. 30 adult wet human cadaveric temporal bones were studied by performing Trans-labrynthine approach to the IAM by using ZEISS microscope in a temporal bone dissection lab. Photographs were taken by HD phone camera, imported into computer & labeled the anatomical landmarks. Wide exposure & 3D visualization of complex anatomical landmarks were noted in each step by step approach from basic to advanced procedure of Trans-labrynthine approach to IAM. The step by step approach to the IAM from basic to advanced procedure in a cadaveric temporal bone offers great orientation & opportunity to become a master of the complex surgical anatomy of IAM and in acquiring a 3D orientation of the critical structures. [ABSTRACT FROM AUTHOR]

Local and privileged expression of dendritic proteins allows segregation of distinct functions in a single neuron but may represent one of the underlying mechanisms for early and insidious presentation of sensory neuropathy. Tangible characteristics of early hearing loss (HL) are defined in correlation with nascent hidden hearing loss (HHL) in humans and animal models. Despite the plethora of causes of HL, only two prevailing mechanisms for HHL have been identified, and in both cases, common structural deficits are implicated in inner hair cell synapses, and demyelination of the auditory nerve (AN). We uncovered that Na+-activated K+ (KNa) mRNA and channel proteins are distinctly and locally expressed in dendritic projections of primary ANs and genetic deletion of KNa channels (Kcnt1 and Kcnt2) results in the loss of proper AN synaptic function, characterized as HHL, without structural synaptic alterations. We further demonstrate that the local functional synaptic alterations transition from HHL to increased hearing-threshold, which entails changes in global Ca2+ homeostasis, activation of caspases 3/9, impaired regulation of inositol triphosphate receptor 1 (IP3R1), and apoptosis-mediated neurodegeneration. Thus, the present study demonstrates how local synaptic dysfunction results in an apparent latent pathological phenotype (HHL) and, if undetected, can lead to overt HL. It also highlights, for the first time, that HHL can precede structural synaptic dysfunction and AN demyelination. The stepwise cellular mechanisms from HHL to canonical HL are revealed, providing a platform for intervention to prevent lasting and irreversible age-related hearing loss (ARHL).

Noninvasive transcranial electric stimulation is increasingly being used as an advantageous therapy alternative that may activate deep tissues while avoiding drug side-effects. However, not only is there limited evidence for activation of deep tissues by transcranial electric stimulation, its evoked human sensation is understudied and often dismissed as a placebo or secondary effect. By systematically characterizing the human sensation evoked by transcranial alternating-current stimulation, we observed not only stimulus frequency and electrode position dependencies specific for auditory and visual sensation but also a broader presence of somatic sensation ranging from touch and vibration to pain and pressure. We found generally monotonic input-output functions at suprathreshold levels, and often multiple types of sensation occurring simultaneously in response to the same electric stimulation. We further used a recording circuit embedded in a cochlear implant to directly and objectively measure the amount of transcranial electric stimulation reaching the auditory nerve, a deep intercranial target located in the densest bone of the skull. We found an optimal configuration using an ear canal electrode and low-frequency (

Objectives: The presence of a functional cochlear nerve is a key issue in the preoperative evaluation of pediatric candidates for cochlear implants. Correlations between cochlear nerve deficiency (CND) and bony abnormalities of the labyrinth or bony canal of the cochlear nerve are not yet well understood. The aim of this study was to determine whether the width of the bony cochlear canal (BCNC) can serve as a reliable predictive factor for the existence of a CND. Materials and methods: A total of 11 children with a confirmed diagnosis of prelingual, severe sensorineural hearing loss were included in this study. In all patients, indication for CI was confirmed and according to the preoperative protocol, high-resolution CT and MR were performed. Reconstructions at a distance of 0.6 mm of the axial plane and images from the HRCT of temporal bones were used for measuring the width of the BCNC. The cochlear nerves were evaluated on axial and sagittal – oblique T2 – MRI images and classified as normal, hypoplastic or aplastic. Two factors were reviewed retrospectively: the presence of inner ear anomalies and the relationship between BCNC stenosis and the existence of CND. Results: From a total of 22 temporal bones analyzed (22 ears in 11 patients), inner ear malformations were detected in 6 ears from 3 patients (27.27%). All three children had a bilateral malformation, in one it was Michel deformity and in two it was IP2 (incomplete partition 2). The BCNC diameter ranged from 0.1mm to 2.33mm with a mean value of 1.46±0.6mm. CND was recorded in 4 of 22 ears and all were associated with stenosis of the BCNC. In a total of three ears with a stenotic canal, we obtained a normal finding for the cochlear nerve on MR. Conclusion: Children with BCNC stenosis have a high incidence of CND. A narrowed BCNC on CT can be an indicator for the selection of children with sensorineural hearing loss who will need to be additionally referred for MRI in order to definitively assess the status of the cochlear nerve. [ABSTRACT FROM AUTHOR]

Objective:To reveal the correlation between implantation age, the internal acoustic canal (IAC) width, bony cochlear nerve canal (BCNC) width, and auditory performance in primary school children with bilateral cochlear implantation (CI).Methods:Preoperative IAC and BCNC widths of 57 pre-lingually deaf children aged 7–11 years who had previously undergone bilateral CI in our institution were reviewed and cut-off values were calculated. Twenty-four patients who had additional problems and could not attend school and those who refused to participate in the study were excluded. The remaining 33 were invited to the hospital, and their speech perception tests, and language development scores were analyzed (16 of 33 patients had been operated on before the age of 24 months).Results:The cut-off values calculated from the 114 ears of 57 patients were 3.86 mm for IAC width and 1.56 mm for BCNC width. The auditory performances of the 33 patients after CI were not significantly different in the narrow and normal width groups. However, speech perception test results, and language development scores of patients implanted before the age of 24 months were significantly higher.Conclusion:There are some studies showing that children with bilateral sensorineural hearing loss have narrower IAC and BCNC widths. However, we concluded that the widths of the IAC and the bone cochlear nerve canal did not affect auditory performance. We found that implantation age is the single most important determinant of speech-language development after CI.

Electrical stimulation of the auditory nerve with a penetrating intraneural (IN) electrode in acutely deafened cats produces much more restricted spread of excitation than is obtained in that preparation with a conventional cochlear implant (CI) as reported by Middlebrooks and Snyder (J Assoc Res Otolaryngol 8:258-279, 2007). That suggests that a future auditory prosthesis employing IN stimulation might offer human patients greater frequency selectivity than is available with a present-day CI. Nevertheless, it is a concern that the electrical field produced by an IN electrode might be too restricted to produce adequate stimulation of the partially depopulated auditory nerve of a deaf patient. We evaluated this by testing responses to IN and CI stimulation in adult-deafened cats. Activation of the auditory pathway was monitored by recording from the central nucleus of the inferior colliculus (ICC). Cats deaf for 153-277 days exhibited a ~ 30 % loss of auditory nerve fibers compared to cats deaf for

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to qualitatively evaluate the variations in nerve bundles between patients with normal anatomy and those with inner-ear anomalies., Methods: Magnetic resonance imaging (MRI) scans of the temporal bones of candidates for cochlear implants (CIs) enrolled at a tertiary center were retrospectively reviewed from the clinical database. The 3.0-Tesla MRI scans were analyzed using a three-dimensional slicer to visualize the nerve bundles in the internal auditory canal., Results: A total of 49 ears were analyzed. Twenty ears exhibited normal inner ear anatomy, whereas 29 ears had various inner-ear malformations. The cochlear nerve (CN) was visible on all 20 scans with normal inner-ear anatomy. In addition, the CN was visualized in 18 scans with inner ear malformations. Furthermore, the CN was identified in six of the eight scans with IP type I, whereas in two scans, the CN and vestibular nerve were unclear. Three scans with a common cavity showed only two nerve bundles., Conclusion: The findings of this study show that the CN can be visualized in most inner-ear anatomical types. Even in severely malformed inner ears, the common nerve bundle that represents the cochlear and vestibular nerves can be visualized. The MRI is highly recommended for CN assessment before CI surgery., Level of Evidence: Level IV., Competing Interests: Anandhan Dhanasingh is employed by MED‐EL as the Head of Electrodes Research & Inner Ear Malformations within R&D department. The remaining authors declare no conflict of interest., (© 2024 The Author(s). Laryngoscope Investigative Otolaryngology published by Wiley Periodicals LLC on behalf of The Triological Society.)

Objectives: This study investigated the relationships between the cochlear nerve (CN) health and sentence-level speech perception outcomes measured in quiet and noise in postlingually deafened adult cochlear implant (CI) users., Design: Study participants included 24 postlingually deafened adult CI users with a Cochlear ® Nucleus ™ device. For each participant, only one ear was tested. Neural health of the CN was assessed at three or four electrode locations across the electrode array using two parameters derived from results of the electrically evoked compound action potential (eCAP). One parameter was the phase locking value (PLV) which estimated neural synchrony in the CN. The other parameter was the sensitivity of the eCAP amplitude growth function (AGF) slope to changes in the interphase gap (IPG) of biphasic electrical pulses (i.e., the IPGE slope ). Speech perception was tested using AzBio sentences in both quiet and a ten-talker babble background noise with +5 dB and +10 dB signal-to-noise ratios (SNR). IPGE slope and PLV values were averaged across electrodes for each subject, both with and without weighting by the frequency importance function (FIF) of the AzBio sentences. Pearson and Spearman correlations were used to assess the pairwise relationships between the IPGE slope , the PLV, and age. Multiple linear regression models with AzBio score as the outcome and the PLV and the IPGE slope as predictors were used to evaluate the associations between the three variables while controlling for age., Results: The correlation between the IPGE slope and the PLV was negligible and not statistically significant. The PLV, but not the IPGE slope , differed significantly across electrodes, where the apical electrodes had larger PLVs (better neural synchrony) than the basal electrodes. The IPGE slope , but not the PLV, was significantly correlated with participant's age, where smaller IPGE slope values (poorer CN health) were associated with more advanced age. The PLV, but not the IPGE slope , was significantly associated with AzBio scores in noise, where larger PLVs predicted better speech perception in noise. Neither the PLV nor the IPGE slope was significantly associated with AzBio score in quiet. The result patterns remained the same regardless of whether the mean values of the IPGE slope and the PLV were weighted by the AzBio FIF., Conclusions: The IPGE slope and the PLV quantify different aspects of CN health. The positive association between the PLV and AzBio scores suggests that neural synchrony is important for speech perception in noise in adult CI users. The lack of association between age and the PLV indicates that reduced neural synchrony in the CN is unlikely the primary factor accounting for the greater deficits in understanding speech in noise observed in elderly, as compared to younger, CI users., Competing Interests: Conflict of Interest: None.

Objective: Hearing outcomes following Gamma Knife radiosurgery (GKRS) for vestibular schwannoma (VS) are multifactorial and poorly characterized in prior literature. In this study the authors evaluated hearing outcomes chronologically to identify prognostic factors of serviceable hearing preservation (HP) rates following GKRS for VS., Methods: Six medical databases were queried according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Eligible studies reported VS treated with single-fraction GKRS and included the HP rate following GKRS. HP was defined as a postoperative Gardner-Robertson score ≤ 2 among patients with preoperative serviceable hearing. A meta-analysis with random-effects modeling was performed for variables of interest., Results: Data from 42 articles with a total of 6582 patients were analyzed; the average age of patients was 54 years and the average follow-up time was 68 months. The pooled proportion of preoperative serviceable hearing was 76%, and the pooled HP rate was 60% at the last follow-up visit. At < 5 years after GKRS, age was significantly correlated with HP on both continuous and categorical analyses (p = 0.001 and p = 0.011, respectively). Between 5 and < 10 years of follow-up, HP was associated with a radiation dose of 12.5 Gy, but not with age or tumor volume. At ≥ 10 years after radiosurgery, a significant negative correlation was seen between marginal radiation dose and HP on both continuous and categorical analyses (p = 0.001 and p = 0.021, respectively)., Conclusions: This meta-analysis identifies age and radiation dose as independent prognostic factors for HP. Age-related hearing deterioration appears to be concentrated in the first 5 years after GKRS, whereas radiation dose was associated with HP at last follow-up, between 5 and < 10 years, and at ≥ 10 years after radiosurgery. This meta-analysis offers an objective overview of the literature and a framework for clinical decision-making, with applications for treatment planning and patient counseling.

Cochlear implants (CIs) are the treatment of choice for hearing rehabilitation in children with congenital or acquired profound hearing loss or deafness in order to ensure appropriate speech development and avoid social deprivation. However, in the case of a radiologically detectable malformation of the inner ear structures and potentially associated hypo- or aplasia of the vestibulocochlear nerve, application of a CI is either not possible, or the functional outcome may be of limited predictability. In addition, the risk of surgical complications is also increased in these patients. Counseling parents and developing an appropriate individual therapeutic decision can therefore be a major challenge for the medical team. The current paper is intended to provide support in this regard. It presents criteria for various inner ear malformations and discusses possible treatment options., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Cochlear nerve deficiency (CND) is a rare cause of congenital sensorineural hearing loss, often leading to serious communication challenges if not diagnosed and treated early. We present this case of a three-year-old male child with congenital hearing loss and sensory aphasia. Despite normal cochlear anatomy on computed tomography, magnetic resonance imaging (MRI) revealed the absence of the cochlear nerve on the right side. The diagnosis of right-sided CND was confirmed, and the patient was advised to undergo cochlear implantation and speech therapy. This case underscores the importance of timely imaging, particularly using MRI with high-resolution sequences, in diagnosing CND and facilitating early intervention to improve auditory and developmental outcomes., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Khaladkar et al.)

Introduction: Electrically evoked auditory brainstem responses provide reliable clinical information to assist professionals in the auditory rehabilitation of cochlear implant users. Objective: This study aimed to investigate intraoperative evoked auditory brainstem response recordings in Evo®-cochlear implant electrode array recipients and its correlation with their behavioral levels and auditory performance. Methods: : This is a retrospectivey study. Intraoperative evoked auditory brainstem responses were recorded in adult Evo®-cochlear implant electrode array recipients. Wave V latencies, amplitudes and interpeak III–V intervals were recorded in three different electrode locations and compared to the sentence recognition scores obtained from subjects after six months of device use. Evoked auditory brainstem responses thresholds were also recorded and compared to the behaviorally determined levels of the subjects in the sound processor activation. Results: Evoked auditory brainstem responses thresholds were significantly correlated with both, behavioral T- and C-levels and they were recorded at audible electrical stimulation levels in all subjects. There was a significant correlation between interpeak III–V interval recorded in the apical electrode and sentence recognition scores of the subjects. Conclusions: Intraoperative evoked auditory brainstem responses can be used to establish audible levels for fitting the sound processor in Evo®-cochlear implant recipients and it could help professionals to plan further actions aiming to improve their auditory performance.

Artificial cochlea or cochlear implant is the name for neurosensory prosthesis, which is a current surgical treatment for severe hearing loss or deafness.The surgical procedure is cochlear implantation During the cochlear implantation one of the methods of neural response measuring of the auditory nerve to electrical stimulation is telemetry. It is an integral part of the cochlear implantation procedure. Telemetry determines audibility thresholds, stimulus discomfort thresholds, and dynamic range. With objective parameters, in telemetry we also estimate psychoacoustic parameters, which are the basis of simulation maps for the future hearing rehabilitation.

Introduction:To investigate the relationship between morphometric variants of the cochlear nerve (CN) and the hearing level. To investigate the confounding effects of age, sex, and audiometric variables on the relationship between CN morphometrics and hearing loss, if present.Methods:Audiological data and magnetic resonance imaging findings of 127 patients with asymmetrical sensorineural hearing loss (ASNHL) were reviewed retrospectively. According to pure tone average (PTA) frequencies of each ear, difference above 25 dB were accepted as ASNHL. The vertical (Vd) and horizontal diameters (Hd) and the cross-sectional area (CSA) of the CN were measured.Results:The difference in CSA and Vd between ears showed a negative correlation with age. The mean CSA was 1.57 in AE and 1.60 in NE. Mean Hd was 1.00 in AE and 1.00 NE. Mean Vd was 1.40 in AE and 1.50 in NE. There was no correlation between the difference ratios of thresholds and CN diameter parameters. There was no significant correlation of CSA, Vd, and Hd with PTA thresholds at each frequency.Conclusion:Aging is associated with losses in Vd diameter and CSA. The degree of hearing loss was not correlated with the morphometric features of the CN. The morphometry of the CN should be interpreted together with the etiology of hearing loss.

Objective: To reveal the correlation between implantation age, the internal acoustic canal (IAC) width, bony cochlear nerve canal (BCNC) width, and auditory performance in primary school children with bilateral cochlear implantation (CI). Methods: Preoperative IAC and BCNC widths of 57 pre-lingually deaf children aged 7-11 years who had previously undergone bilateral CI in our institution were reviewed and cut-off values were calculated. Twenty-four patients who had additional problems and could not attend school and those who refused to participate in the study were excluded. The remaining 33 were invited to the hospital, and their speech perception tests, and language development scores were analyzed (16 of 33 patients had been operated on before the age of 24 months). Results: The cut-off values calculated from the 114 ears of 57 patients were 3.86 mm for IAC width and 1.56 mm for BCNC width. The auditory performances of the 33 patients after CI were not significantly different in the narrow and normal width groups. However, speech perception test results, and language development scores of patients implanted before the age of 24 months were significantly higher. Conclusion: There are some studies showing that children with bilateral sensorineural hearing loss have narrower IAC and BCNC widths. However, we concluded that the widths of the IAC and the bone cochlear nerve canal did not affect auditory performance. We found that implantation age is the single most important determinant of speech-language development after CI. [ABSTRACT FROM AUTHOR]

Vestibular schwannoma is the most common intracranial schwannoma. The treatment concept is changing from simple surgical resection to the integrated strategy of observation and follow⁃up, stereotactic radiotherapy (SRT) and surgical resection. Higher requirements are put forward for the function perservation of the facial nerve and the cochlear nerve. Neurosurgeons should pay attention to the protection of nerve function and actively develop modern vestibular schwannoma surgery.

Sensorineural hearing impairment is the most common form of hearing loss, and encompasses pathologies of the cochlea and the auditory nerve. Hearing impairment caused by abnormal neural encoding of sound stimuli despite preservation of sensory transduction and amplification by outer hair cells is known as 'auditory neuropathy'. This term was originally coined for a specific type of hearing impairment affecting speech comprehension beyond changes in audibility: patients with this condition report that they "can hear but cannot understand". This type of hearing impairment can be caused by damage to the sensory inner hair cells (IHCs), IHC ribbon synapses or spiral ganglion neurons. Human genetic and physiological studies, as well as research on animal models, have recently shown that disrupted IHC ribbon synapse function--resulting from genetic alterations that affect presynaptic glutamate loading of synaptic vesicles, Ca(2+) influx, or synaptic vesicle exocytosis--leads to hearing impairment termed 'auditory synaptopathy'. Moreover, animal studies have demonstrated that sound overexposure causes excitotoxic loss of IHC ribbon synapses. This mechanism probably contributes to hearing disorders caused by noise exposure or age-related hearing loss. This Review provides an update on recently elucidated sensory, synaptic and neural mechanisms of hearing impairment, their corresponding clinical findings, and discusses current rehabilitation strategies as well as future therapies.

With the development of medicine, surgery has also experienced the development and evolution from traditional surgery to minimally invasive surgery, and then to super minimally invasive surgery (SMIS). Meanwhile, reducing surgical trauma and preserving and reconstructing nerve function have gradually become new goals of modern vestibular schwannoma (VS) resection surgery. The surgery of VS can be divided into hearing-preserving surgery (retrosigmoid approach and middle fossa approach) and non-hearing-preserving surgery (traditional translabyrinthine approach), according to whether the patient has practical hearing before operation. Improving the hearing preservation rate of hearing-preserving surgery and reconstructing the hearing of patients with non-hearing-preserving surgery are major challenges and hotspots. The traditional translabyrinthine approach has the highest proportion in the Department of Otolaryngology-Head and Neck Surgery, with the advantages of high facial nerve preservation rate and few intracranial complications. However, due to the resection of the cochlear nerve and labyrinth, the cochlea develops fibrosis, and patients lose the opportunity to reconstruct hearing through cochlear implantation. The new modified translabyrinthine approach can preserve the cochlear nerve and effectively reduce cochlear fibrosis, providing an opportunity for cochlear implantation to reconstruct the hearing. This is another important breakthrough in vestibular schwannoma surgery.

Background: Magnetic resonance imaging (MRI) is used to determine whether cochlear nerve development is normal in infants and adults, but it has not yet been used to evaluate cochlear nerve development or measure cochlear nerve-related structures in the fetus. This study sought to provide imaging data for clinical evaluations concerning cochlear nerve development in the fetus using MRI., Methods: Postmortem 3.0-Tesla MRI of inner ear was performed in 51 fetuses with normal temporal bones at 25 to 40 weeks of gestation. The continuous scanning protocol incorporated axial three-dimensional (3D) sampling perfection with application-specific contrasts using different flip angle evolution sequences. The images were evaluated to measure the structures of the cochlear aperture (CA), internal auditory canal (IAC), and vestibulocochlear and facial nerves in the cerebellopontine angle (CPA), which have been reported to be associated with cochlear nerve development. We also calculated the ratio between the diameters of the vestibulocochlear and facial nerves. The measurable parameters were compared between the right and left sides. The threshold for statistical significance was set at P<0.05., Results: The inner ear anatomy was discernible on MRI in all the fetal specimens, and growth of the CA, IAC, vestibulocochlear nerve, and facial nerve in the CPA was observed as fetal age increased. There was no significant difference in the measurements of these structures between the right and left sides (all P>0.05)., Conclusions: MRI can be used to help evaluate the anatomy and development of the cochlear nerve in the fetus. These normative measurements could be valuable for clinical evaluations of the cochlear nerve., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-24-626/coif). C.A.M. serves as an unpaid editorial board member of Quantitative Imaging in Medicine and Surgery. The other authors have no conflicts of interest to declare., (2024 Quantitative Imaging in Medicine and Surgery. All rights reserved.)

&lt;b&gt;Introduction:&lt;/b&gt; The choice of treatment of vestibular schwannoma (VS) depends on several factors, including the tumor size, the patient's age and overall health, and the presence and severity of symptoms.&lt;b&gt;Aim:&lt;/b&gt; The aim of the study was to assess the effectiveness of intentional subtotal resection (STR) of tumor followed by Gamma Knife surgery (GKS) in patients with larger VS (Koos 3 and 4).&lt;b&gt;Materials and methods:&lt;/b&gt; The retrospective analysis was performed on 18 patients. Data of VS volumes measured in MRI, the facial nerve function assessed in the House-Brackmann scoring system (HB), and the results of audiological examination expressed on the Gardner-Robertson scale (GR) were collected preoperatively, postoperatively, and post-GKS.&lt;b&gt;Results:&lt;/b&gt; Preoperatively, the main symptom was hearing loss observed in 13 out of 18 patients. The facial nerve function was assessed as HB 1 in 16, whereas HB 2 in 2 patients. The mean volume of the tumor in the initial MRI amounted to 16.81 cm&lt;sup&gt;3&lt;/sup&gt; . Postoperatively, the facial nerve was assessed as HB 1 or 2 in 16, whereas HB 3 in 2 patients. Serviceable hearing was presented by only 4 persons. The Mean diameter of the tumor after subtotal surgery amounted to 3.16 cm&lt;sup&gt;3&lt;/sup&gt; , 1.83 cm&lt;sup&gt;3&lt;/sup&gt; after GKS, and 1.58 cm&lt;sup&gt;3&lt;/sup&gt; at the last follow-up. The facial nerve function and hearing level remained the same as before GKS in all patients.&lt;b&gt;Conclusions:&lt;/b&gt; STR followed by GKS can be a safe and effective method of treatment of large VS concerning the functional outcome of the facial nerve and the tumor volume growth control.

Vestibular schwannomas (VS) are slow-growing intracranial extraaxial benign tumors, developing from the vestibular part of the eight cranial nerves. Stereotactic radiosurgery (SRS) has now a long-term scientific track record as first intention treatment for small- to medium-sized VS. Though its success rate is very high, SRS for VS might fail to control tumor growth in some cases. However, the literature on repeat SRS after previously failed SRS remains scarce and reported in a low number of series with a limited number of cases. Here, we aimed at performing a systematic review and meta-analysis of the literature on repeat SRS for VS. Using PRISMA guidelines, we reviewed manuscripts published between January 1990 and October 2020 and referenced in PubMed. Tumor control and cranial nerve outcomes were evaluated with separate meta-analyses. Eight studies comprising 194 patients were included. The overall rate of patients treated in repeat SRS series as per overall series with first SRS was 2.2% (range 1.2–3.2%, p < 0.001). The mean time between first and second SRS was 50.7 months (median 51, range 44–64). The median marginal dose prescribed at first SRS was 12 Gy (range 8–24) and at second SRS was 12 Gy (range 9.8–19). After repeat SRS, tumor stability was reported in 61/194 patients, i.e., a rate of 29.6% (range 20.2–39%, I2 = 49.1%, p < 0.001). Tumor decrease was reported in 83/194 patients, i.e., a rate of 54.4% (range 33.7–75.1%, I2 = 89.1%, p < 0.001). Tumor progression was reported in 50/188 patients, i.e., a rate of 16.1% (range 2.5–29.7%, I2 = 87.1%, p = 0.02), rarely managed surgically. New trigeminal numbness was reported in 27/170 patients, i.e., a rate of 9.9% (range 1.4–18.3%, p < 0.02). New facial nerve palsy of worsened of previous was reported in 8/183 patients, i.e., a rate of 4.3% (range 1.4–7.2%, p = 0.004). Hearing loss was reported in 12/22 patients, i.e., a rate of 54.3% (range 24.8–83.8%, I2 = 70.7%, p < 0.001). Repeat SRS after previously failed SRS for VS is associated with high tumor control rates. Cranial nerve outcomes remain favorable, particularly for facial nerve. The rate of hearing loss appears similar to the one related to first SRS. [ABSTRACT FROM AUTHOR]

Introduction Good hearing in pilots, including central auditory skills, is critical for flight safety and the prevention of aircraft accidents. Pure tone audiometry alone may not be enough to assess hearing in the members of this population who, in addition to high noise levels, routinely face speech recognition tasks in non-ideal conditions. Objective To characterize the frequency-following response (FFR) of a group of military pilots compared with a control group. Methods Twenty military pilots in the Study Group and 20 non-pilot military personnel, not exposed to noise in their work, in the Control Group, all with normal hearing, aged between 30 and 40 years old, completed a questionnaire to assess their hearing habits, and their FFRs were measured with a /da/ syllable (duration 40 milliseconds, speed 10.9/s), at 80 dB NA in the right ear. All procedures were approved by the ethical committee of the institution. Statistical analysis was performed using the t-Student or Mann-Whitney tests for quantitative variables, and the Fisher or chi- squared tests for qualitative variables, and a value of p<0.05 was considered to be statistically significant. Results There was no significant difference between the groups regarding auditory habits. In the FFR, wave amplitudes A (p=0.01) and C (p=0.04) were significantly lower in the Study Group. Conclusion Working as a military pilot can be a crucial factor in determining an individual’s typical FFR pattern, demonstrated in the present study by statistically significant reductions in the amplitudes of the A and C waves. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To investigate prevalence, radiological characteristics, and functional correlates of arachnoid cysts (AC) of the internal auditory canal (IAC) region, including associations of nerve compression with auditory/vestibular symptoms and asymmetrical audiogram or vestibular testing. Study Design: Retrospective study. Methods: T2‐weighted magnetic resonance imaging (MRI) studies of IACs were retrospectively analyzed from 1247 patients with asymmetric auditory or vestibular symptoms. Patients with radiological findings of AC of the IAC were identified. Multiplanar analysis was used to analyze cyst position in the IAC and assess nerve displacement or compression. Size, position, and presence of nerve compression were correlated with symptoms. Results: Twenty‐four patients had a cyst in the middle or fundus in the IAC. Diameter (P =.04) and position (P =.002) of AC were associated with symptoms. Sagittal analyses identified displacement versus compression (P =.003) more reliably than axial imaging. Symptom laterality was associated with the site of radiological abnormality. Vestibular nerve compression was associated with vertigo (P =.0001), and cochlear nerve compression was associated with auditory symptoms (P <.0001). Conclusions: In a retrospective series of patients undergoing MRI of IACs for asymmetric auditory or vestibular impairment, clinical symptom profile correlated with blinded assessment of IAC lesions. Level of Evidence: 4 Laryngoscope, 131:2323–2331, 2021 [ABSTRACT FROM AUTHOR]

Mutations in the OTOF gene encoding otoferlin result in a disrupted function of the ribbon synapses with impairment of the multivesicular glutamate release. Most affected subjects present with congenital hearing loss and abnormal auditory brainstem potentials associated with preserved cochlear hair cell activities (otoacoustic emissions, cochlear microphonics [CMs]). Transtympanic electrocochleography (ECochG) has recently been proposed for defining the details of potentials arising in both the cochlea and auditory nerve in this disorder, and with a view to shedding light on the pathophysiological mechanisms underlying auditory dysfunction. We review the audiological and electrophysiological findings in children with congenital profound deafness carrying two mutant alleles of the OTOF gene. We show that cochlear microphonic (CM) amplitude and summating potential (SP) amplitude and latency are normal, consistently with a preserved outer and inner hair cell function. In the majority of OTOF children, the SP component is followed by a markedly prolonged low-amplitude negative potential replacing the compound action potential (CAP) recorded in normally-hearing children. This potential is identified at intensities as low as 90 dB below the behavioral threshold. In some ears, a synchronized CAP is superimposed on the prolonged responses at high intensity. Stimulation at high rates reduces the amplitude and duration of the prolonged potentials, consistently with their neural generation. In some children, however, the ECochG response only consists of the SP, with no prolonged potential. Cochlear implants restore hearing sensitivity, speech perception and neural CAP by electrically stimulating the auditory nerve fibers. These findings indicate that an impaired multivesicular glutamate release in OTOF-related disorders leads to abnormal auditory nerve fiber activation and a consequent impairment of spike generation. The magnitude of these effects seems to vary, ranging from no auditory nerve fiber activation to an abnormal generation of EPSPs that occasionally trigger a synchronized electrical activity, resulting in high-threshold CAPs.