Background Auditory neuropathy, coupled with internal auditory canal stenosis, presents unique challenges for hearing restoration. Advances in cochlear implantation technology have enabled improved auditory and speech perception outcomes in affected patients. Objective This study evaluates the effectiveness of cochlear implantation in enhancing hearing and speech perception in patients diagnosed with auditory neuropathy and internal auditory canal stenosis. Methods Thirteen patients diagnosed with auditory neuropathy and internal auditory canal stenosis underwent cochlear implantation at our institution. Pre- and post-operative assessments included behavioral or pure-tone audiometry to assess hearing thresholds, supplemented by objective measures including auditory steady-state response (ASSR) and 40 Hz objective threshold testing. Mandarin Early Speech Perception (MESP) testing, and IT-MAIS/MAIS scoring. Assessments were conducted preoperatively and at six months, one year, and two years post-implant activation. Statistical comparisons between pre- and post-operative outcomes were performed using paired t-tests. Results After six months of activation, all patients showed significant improvements in behavioral or pure-tone audiometry and ASSR testing (P < 0.05). MESP testing revealed that all patients passed Level 1, with 53.8 % reaching Level 2, and higher levels achieved progressively over two years. Notably, the proportion of patients passing Level 4 increased dramatically from 7.7 % at six months to 53.8 % at one year and 92.3 % at two years, 53.8 % reached Level 5, and 23 % completed Level 6. IT-MAIS/MAIS scores significantly improved at six months post-activation (P < 0.05) and continued to increase after one and two years, demonstrating enhanced auditory and speech perception capabilities over time. Conclusion Technology-driven cochlear implantation substantially enhances auditory and speech perception capabilities in patients with auditory neuropathy and internal auditory canal stenosis. These findings establish the substantial therapeutic efficacy of cochlear implantation for this patient population and its potential for broader application in audiological rehabilitation.

王秋菊: 随着人口老龄化进程的加快,老年性听力损失与认知障碍的共病现象逐渐引起临床与科研领域的高度重视.大量研究提示,听力下降不仅影响老年人沟通能力与生活质量,还可能与认知功能减退呈显著相关,甚至在一定程度上加速痴呆的发生与发展.

Purpose The purpose of the study was to investigate the effect of bimodal beamforming on speech recognition and comfort for cochlear implant (CI) users with the bimodal hearing solution made up by linking a hearing aid to the CI sound processor.Methods 19 subjects participated in this study. Speech tests were conducted in quiet and in noisy environments, with the target speech presented from 0° and the noise signal from 45°. Speech recognition thresholds (SRTs) were compared among the previously used bimodal hearing configuration (baseline, any CI sound processor plus any hearing aid), the Naída Bimodal Hearing Solution with omnidirectional microphone, and with directional microphone (so called StereoZoom) switched on. In addition, the study participants provided subjective feedback on their hearing impressions.Results The SRT results showed no significant difference among the three hearing conditions in the quiet environment. No significant improvement was reported when using Naída bimodal system with omnidirectional microphone in noise compared to the baseline (p=0.27). When applying StereoZoom, SRT in noise showed significant improvements compared to omnidirectional settings (p<0.05). Subjective feedback showed that 13 participants were satisfied with Naída Bimodal Hearing Solution, and wanted to continue using it after the trial.Conclusion The Naída Bimodal Hearing Solution with the same pre-processing algorithm can provide satisfying hearing performance. Beamforming technology can further improve speech perception in noisy environments.

The cochlear implant (CI) is the most successful neural prosthesis and has improved the quality of life for over one million people with profound hearing loss. The number and function of spiral ganglion neurons (SGNs) in the cochlea are crucial to CI performance. Clinically, patients with significant SGN degeneration fail to return to normal life due to unsatisfactory performance of their CIs. This study aims to explore the efficacy of improve auditory function by delivering stem cells into the SGN region using a customized CI (defined as Cochlear-Bioelectrode). Human induced pluripotent stem cells (hiPSCs) with a green fluorescent protein (GFP) marker were delivered cross the osseous spiral lamina via naturally existing openings and congregate in the SGN region by Cochlear-Bioelectrode after Cochlear implantation, electrically evoked auditory brainstem responses (EABR) were compared between those with and without stem cells delivery. In seven days, efficient hiPSC migration into the SGN region was demonstrated with concurrent electric stimulation and stem cell delivery, while hipsc did not migrate into the sgn region with stem cell delivery alone. After two weeks of combined electric stimulation and stem cell delivery treatment, the number of SGNs increased significantly in the 60-day old pig model (mean number = 129.6 ± 1.61/field), compared to untreated deafness pigs of the same age (mean number = 6.2 ± 0.92/field). With the replenished SGNs, CI stimulation thresholds were significantly reduced and hearing sensitivity and dynamic range extended, as demonstrated by electrically evoked auditory brainstem responses. Our results showed that in vivo concurrent cochlear implant stimulation and hipsc delivery increased stem cells in the sgn region and lowered ci stimulation thresholds, paving the way to broad and promising clinical applications.

This systematic bibliometric analysis evaluates the scientific evidence, impact, and trends of photobiomodulation (PBM) for central nervous system (CNS) disorders. Analyzing 150 relevant PubMed-indexed studies (including 46 clinical trials) up to September 2024, we demonstrate PBM's positive effects on the CNS. Key mechanisms include enhanced ATP synthesis, modulated nitric oxide signaling, improved neuronal excitability, suppressed oxidative stress, anti-inflammatory effects, and ion channel modulation. PBM offers therapeutic flexibility: shorter-wavelength, high-irradiance power lasers effectively target deep brain structures, while both modalities suit superficial cortical applications where fine spatial control is nonessential.Crucially, PBM induces long-lasting biological effects, highlighting significant potential for chronic CNS conditions like Alzheimer's disease, Parkinson's disease, and stroke. However, critical challenges hinder translation: optimizing disease-specific wavelengths and light sources (laser vs. LED, continuous vs. pulsed), and establishing standardized, long-term treatment protocols based on robust dose-response relationships. Resolving heterogeneity in parameters (energy density, power, potential thermal effects) is paramount for PBM to become a reliable neuromodulation tool. This analysis provides critical insights for advancing targeted PBM therapies in CNS.

Background: In temporal bone specimens from long-term cochlear implant users, foreign body response within the cochlea has been demonstrated. However, how hearing changes after implantation and fibrosis progresses within the cochlea is unknown. Objectives: To investigate the short-term dynamic changes in hearing and cochlear histopathology in minipigs after electrode array insertion. Material and Methods: Twelve minipigs were selected for electrode array insertion (EAI) and the Control. Hearing tests were performed preoperatively and on 0, 7, 14, and 28 day(s) postoperatively, and cochlear histopathology was performed after the hearing tests on 7, 14, and 28 days after surgery. Results: Electrode array insertion had a significant effect for the frequency range tested (1 kHz-20kHz). Exudation was evident one week after electrode array insertion; at four weeks postoperatively, a fibrous sheath formed around the electrode. At each time point, the endolymphatic hydrops was found; no significant changes in the morphology and packing density of the spiral ganglion neurons were observed. Conclusions and Significance: The effect of electrode array insertion on hearing and intracochlear fibrosis was significant. The process of fibrosis and endolymphatic hydrops seemed to not correlate with the degree of hearing loss, nor did it affect spiral ganglion neuron integrity in the 4-week postoperative period.

Purpose: To analyze the effect of right versus left long-term single-sided deafness (SSD) on sound source localization (SSL), discuss the necessity of intervention and treatment for SSD patients, and analyze the therapeutic effect of long-term unilateral cochlear implantation (UCI) from the perspective of SSL. Methods: This study included 25 patients with SSD, 11 patients with UCI, and 30 participants with normal hearing (NH). Their SSL ability was tested by obtaining their average root mean square (RMS) error values of SSL test. Results: The results showed that the RMS error value of SSD, UCI and NH groups were 52.26 ± 20.25°, 69.84 ± 12.14° and 4.27 ± 2.66°, respectively. The ability of SSL was better in the SSD-L group than that in the SSD-R group, and no significant difference existed in the SSD-R and the UCI group. Conclusion: When bilateral deafness patients select unilateral treatment, right-side cochlear implantation may be more beneficial in terms of SSL, which means that the central auditory cortex in long-term SSD patients is affected differently based on which side their deafness occurs.

Background: Over half of patients with enlarged vestibular aqueducts (EVA) will have an air-bonr gap (ABG), however, current research on audiology has focused on the influencing factors of air-conducted. Objective: To retrospectively analyse the influencing factors and clinical manifestations of the bone-conduction threshold and ABG in patients with EVA. Materials and methods: We included 286 patients with EVA; among them, 126 had full SLC26A4 gene sequence results. We performed a descriptive analysis of the bone-conduction threshold and explored the effect of age. Finally, we analyzed the relationship of ABG and SLC26A4 genes with the degree of vestibular aqueduct (VA) enlargement. Results: Among 555 ears, 312 (57.8%) ears had ABG; approximately 94% of the patients' bone-conduction hearing is almost completely lost at frequencies of 2 and 4 kHz. There was no linear correlation between age and bone-conduction threshold (p > 0.05). ABG did not significantly differ according to the degree of VA enlargement and number of SLC26A4 allele mutations (p > 0.05). Conclusions and significance: Among patients with EVA, ABG is mainly produced at low frequencies and is not significantly correlated with age, size of the VA opening or SLC26A4 genes, which could be attributed to the biomechanical effects.

Cochlear implantation (CI) is currently recognized as the most effective treatment for severe to profound sensorineural deafness and is considered one of the most successful neural prostheses. Since its inception in 1961, cochlear implantation has expanded its range of applications to encompass younger newborns, older people, and individuals with unilateral hearing loss. In addition, it has improved its surgical methods to minimize the occurrence of complications. Furthermore, notable advancements have been made in the design of electrodes, techniques for speech processing, and software for programming. Nevertheless, inflammation, fibrosis, and even ossification are observed in the cochlea of nearly all cochlear implant (CI) patients. These tissue responses might have a negative impact on the performance of the implants, residual hearing, and the results of post-operative CI rehabilitation. Animal models are significant translational tools that offer essential preclinical data for possible therapeutics. Thus, this study concentrates on the existing animal models used for cochlear implantation, highlights the advancements made in research, and offers insights into potential future research areas.

Abstract Background Cochlear implants have helped over one million individuals restore functional hearing globally, but their clinical utility in suppressing tinnitus has not been firmly established. Methods In a decade-long study, we examined longitudinal effects of cochlear implants on tinnitus in 323 post-lingually deafened individuals including 211 with pre-existing tinnitus and 112 without tinnitus. The primary endpoints were tinnitus loudness and tinnitus handicap inventory. The secondary endpoints were speech recognition, anxiety and sleep quality. Results Here we show that after 24 month implant usage, the tinnitus cohort experience 58% reduction in tinnitus loudness (on a 0–10 scale from 4.3 baseline to 1.8 = −2.5, 95% CI: −2.7 to −2.2, p = 3 x 10−6; effect size d’ = −1.4,) and 44% in tinnitus handicap inventory (=−21.2, 95% CI: −24.5 to −17.9, p = 1 x 10−15; d’=−1.0). Conversely, only 3.6% of those without pre-existing tinnitus develop it post-implantation. Prior to implantation, the tinnitus cohort have lower speech recognition, higher anxiety and poorer sleep quality than the non-tinnitus cohort, measured by Mandarin monosyllabic words, Zung Self-rating Anxiety Scale and Pittsburgh Sleep Quality Index, respectively. Although the 24 month implant usage eliminate the group difference in speech and anxiety measures, the tinnitus cohort still face significant sleep difficulties likely due to the tinnitus coming back when the device was inactive at night. Conclusions The present result shows that cochlear implantation can offer an alternative effective treatment for tinnitus. The present result also identifies a critical need in developing always-on and atraumatic devices for tinnitus patients, including potentially those with normal hearing.

OBJECTIVE:To summarize the Solute Carrier Family 26 Member 4 (SLC26A4) mutations and clinical phenotypic characteristics of patients with Pendred syndrome/nonsyndromic enlarged vestibular aqueduct (PS/NSEVA). DESIGN:A retrospective cohort study for the Chinese population was performed to analyze the hearing test results of 406 patients with PS/NSEVA who had a SLC26A4 mutation and the relationship between inner ear imaging and audiology. RESULTS:There was a significant difference in the mean hearing threshold in patients with biallelic mutations (M2), monoallelic mutations (M1), and nonallelic mutations (M0) and between patients with isolated vestibular aqueduct enlargement (IEVA) and patients with IEVA combined with Mondini malformation. There was no significant difference between patients with different gene mutation types or different sexes, or between the width of the vestibular aqueduct (VA) and the mean hearing threshold. The degree of hearing loss was linearly correlated with age. CONCLUSIONS:We propose that the presence and absence of SLC26A4 mutation, whether combined with Mondini malformation and patient age, are essential factors affecting the degree of hearing loss in the Chinese population. However, the number and type of mutations, degree of VA expansion, and sex of the patients did not affect the clinical audiological phenotype. LEVEL OF EVIDENCE:3 Laryngoscope, 135:848-856, 2025.

BackgroundFollow-up of cochlear implant effectiveness is mainly focused on 3 years postoperatively, and studies with more than 5 years of observation are rare, especially for local Chinese brands. ObjectivesNurotron (Chinese domestic cochlear implant brand) CI recipients who participated in the clinical trial in 2009 were followed-up for 10 years prospectively, providing data to guide doctors and patients. Material and MethodsFrom December 2009 to April 2010, 57 subjects underwent Nurotron Venus CI surgery at multiple-centers, and were continued to be followed up and assessed at 1, 2, 3, 4, 5, and 10 years after switch on. ResultsAll recipients were successfully implanted with CIs with no difficulty in subsequent use with one reported case of re-implantation at 9 years after implantation. The aided hearing thresholds were significantly improved at one month after switch on (p < 0.0001) and remained stable afterwards for 10 years. Speech recognition scores were significantly higher than pre-operative results (p < 0.05) and continued to improve till 3 years after switch on. At 10 years post-operation, most subjects had improved QOL scores in most sub-items. Conclusions and SignificanceNurotron Venus CI System provides long-term, stable results in hearing speech assistance capabilities and can improve the quality of life of CI recipients.

Purpose: The purpose of this study was to determine the speech recognition equivalence of Mandarin Bamford-Kowal-Bench (BKB) sentence lists with adults and children with normal hearing. Method: A total of 32 lists, each of nine sentences, were compiled from a corpus of BKB-like sentences with paired babble in Mandarin. Interlist equivalence, critical differences, and sensitivity of performance to signal-to-noise ratio (SNR) were examined. Experiment 1 included 64 native Mandarin-speaking adults with normal hearing. Experiment 2 included 54 native Mandarin-speaking children with normal hearing aged 4–6 years. Results: Among the 32 sentence lists, 28 lists were confirmed to be equivalent in adults, with a mean SNR required for 50% correct (SNR 50 ) of −5.9 ± 0.1 dB, a mean slope of 22.3%/dB ± 1.5%/dB, and a grand 95% critical difference subsequently calculated as 27.2% for score. From the 28 equivalent lists, 27 lists were selected and observed to be equivalent in children, with a mean SNR 50 threshold of −2.0 ± 0.2 dB, a mean slope of 15.8%/dB ± 1.1%/dB, and a grand 95% critical difference of 24.6% for score. Conclusions: The Mandarin BKB sentences in babble noise test offers an opportunity for clinicians and researchers to assess speech understanding in adults and preschool children in an efficient manner. For comparisons of performance in different test conditions, 28 equivalent lists are available for adults and 27 equivalent lists for preschool children. The 95% critical difference values can be used for total percentage correct or SNR for 50% performance. Future work will examine the clinical utility for school-age children and children who are deaf and hard of hearing. Supplemental Material: https://doi.org/10.23641/asha.24400066

Objective To investigate the relationship between the type and site of MITF pathogenic mutations and clinical pheno‐types for genetic diagnosis and understanding of causes for high heterogeneity of clinical manifestations in Waardenburg syndrome（WS） patients.Methods Literatures in the PubMed and CNKI databases between 1995 and November 10,2021 were searched using key words"MITF"and"Waardenburg syndrome".From studies meeting the inclusion/exclusion criteria,clinical phenotypes of pedigree members were collected.The ClinicalVariant database was searched for MITF pathogen‐ic mutation loci to build a MITF pathogenic mutation locus database.MITF mutation types were further grouped based on the changes of MITF proteins and functions.The correlation between mutation types and clinical phenotypes was ana‐lyzed using generalized estimating equations.Results Twenty eight studies met inclusion criteria and were included.A MITF pathogenic mutation database containing 58 pathogenic mutation loci was established based on the information from the literature search and in the ClinicalVariant database.Most MITF pathogenic mutations in the established data‐base were nonsense mutations involving all exons.Missense mutations were concentrated in exons 7 and 8,which main‐ly affect the coding region of bHLH structure and subsequently MITF functions,leading to the manifestation of the dis‐ease.Regarding relations between mutation sites and clinical phenotypes,amino acid （AA） substitution in the nuclear lo‐calization signal domain was correlated with heterochromia iris and hearing loss （P=0,P=0,respectively）,and AA sub‐stitution in the HLH domain was associated with heterochromia iris,freckles,hair color abnormalities and hearing loss（P=0.013,P=0.025,P=0,P=0,respectively）.Conclusion From our preliminary analysis of the relationship between clinical phenotypes and MITF mutation types,missense mutations ultimately cause change of functions of the MITF pro‐tein by affecting the characteristic structural region bHLH-Zip,leading to the disease.Important domains such as nucle‐ar localization signal in bHLH-Zip are of great significance.In particular,nuclear localization signals play an important role.Missense mutations in nuclear localization signals are associated with the appearance of hearing loss in WS.

大龄语前聋者,指习得语言能力前患重度或极重度耳聋并且人工耳蜗植入时年龄偏大的患者.大龄语前聋患者术后康复效果较适龄幼儿患者差,其术后的评估以及康复效果的影响因素是研究重点.所以,本文就大龄语前聋患者人工耳蜗植入术后康复的评估方法和相关影响因素等方面的研究进展加以综述.

Waardenburg综合征是一种常见的综合征性遗传性疾病,其致病机制复杂,致病基因多样,并且具有明显的临床表现异质性,目前常见的相关致病基因包括PAX3、MITF、SOX10、EDNRB/EDN3、SNAI2.对于不同的致病基因而言,具有不同的致病机制以及病变特点,对于表现为重度以及极重度感音神经性聋的患者而言,人工耳蜗植入是唯一的治疗方法,但是目前关于Waardenburg综合征人工耳蜗植入术后康复效果评价不一,可能与WS患者螺旋神经节细胞的数量以及功能相关.本文将就Waardenburg综合征相关临床特征、不同致病基因的致病机制以及治疗、预后进行简述.

人工耳蜗是模拟人耳蜗毛细胞声-电换能机制的一种电子辅听装置,用来替代听损者损伤的耳蜗,以便恢复重度极重度感音神经性聋患者的听觉[1].一般来讲,体内具有铁磁性置入物的患者是不适合接受磁共振(Magnetic resonance imaging, MRI)检查的,人工耳蜗可以耐受的磁场强度为1.5T[2].耳蜗植入(Cochlear implant,CI)患者在1.5-3.0T进行MRI难度较高[3-4],一般需要将植入体中的磁铁手术取出方可行MRI检查 [5].

目的 评价Waardenburg综合征患者人工耳蜗植入后听觉功能改善及言语表达能力变化,以及耳蜗植入时间与术后改善情况之间的关系.方法 通过检索2000年-2021年间Pubmed,Web of Science,EMbase以及CNKI,万方数据库中发表的英文以及中文研究中,依据纳入以及排除标准,筛选文献共15篇,进行系统性评价,同时选取具有可比性的研究纳入meta分析,进一步分析比较WS患者人工耳蜗术后言语康复效果与其他非综合征感音神经性聋患者相比是否存在显著差异.结果 共有15篇文献纳入系统评价,其中从CAP,SIR,PEACH,MAIS,MUSS以及PTA等方面分别进行评价,并分别将3篇文献,共计34名WS患者纳入CAP以及SIR的meta分析中,结果提示WS以及对照组之间的差异,无统计学意义.将2篇文献纳入PEACH评分的meta分析,结果提示二者之间的差异不具有统计学意义.结论 Waardenburg综合征在行人工耳蜗植入后可以获得与非综合征性耳聋患者相似的术后康复效果.

目的 应用全外显子测序对一Waardenburg综合征家系进行基因检测,明确致病基因,探究家系成员表型呈现异质性的原因.方法 对家系成员进行表型收集,包括家系成员的毛发特征、皮肤色素改变、虹膜颜色以及消化系统、骨骼系统的相关临床特征,并对家系成员进行相关听力检测,主观听力检测包括纯音测听,客观听力检测包括听性脑干反应阈值,听性脑干反应潜伏期,40Hz听觉相关电位,畸变耳声发射检查.同时抽取家系成员静脉血进行基因检测.结果 家系中成员的致病基因经验证为MITF(NM_000248.4)第5号外显子c.544A>T,产生无义突变,编码仅包含182个氨基酸的截短蛋白.依据ACMG指南进行致病性分析,明确该突变为该家系致病性突变.结论 该突变为一新验证发现的MITF突变,丰富了MITF突变基因数据库,并为进一步分析MITF突变相关致病机制提供了新的有力证据.

人工听觉植入技术飞速发展,让更多的耳聋患者回到了有声世界.本文报道一例小耳畸形患者一侧耳一期行振动声桥植入(vibrant soundbridge, VSB),对侧耳二期行人工耳蜗植入(cochlear im-plant,CI),以最终恢复患者听觉功能.