Objectives: To investigate the outcomes of cochlear implantation in Mandarin-speaking cochlear implant (CI) users with single-sided deafness (SSD). Methods: This study was a single-center prospective cohort study. Eleven Mandarin-speaking adult SSD patients who underwent CI implantation at Capital Medical University Beijing Tongren Hospital from August 2020 to October 2021 were recruited, including 6 males and 5 females, with the age ranging from 24 to 50 years old. In a sound field with 7 loudspeakers distributed at 180°, we measured root-mean-square error(RMSE)in SSD patients at the preoperative, 1-month, 3-month, 6-month, and 12-month after switch-on to assess the improvement of sound source localization. The Mandarin Speech Perception (MSP) was used in the sound field to test the speech reception threshold (SRT) of SSD patients under different signal-to-noise locations in a steady-state noise under conditions of CI off and CI on, to reflect the head shadow effect(SSSDNNH), binaural summation effect(S0N0) and squelch effect(S0NSSD). The Tinnitus Handicap Inventory (THI) and the Visual Analogue Scale (VAS) were used to assess changes in tinnitus severity and tinnitus loudness in SSD patients at each time point. The Speech, Spatial and Qualities of Hearing Scale(SSQ) and the Nijmegen Cochlear Implantation Scale (NCIQ) were used to assess the subjective benefits of spatial speech perception and quality of life in SSD patients after cochlear implantation. SPSS 19.0 software was used for statistical analysis. Results: SSD patients showed a significant improvement in the poorer ear in hearing thresholds with CI-on compared with CI-off; The ability to localize the sound source was significantly improved, with statistically significant differences in RMSE at each follow-up time compared with the preoperative period (P<0.05). In the SSSDNNH condition, which reflects the head shadow effect, the SRT in binaural hearing was significantly improved by 6.5 dB compared with unaided condition, and the difference was statistically significant (t=6.25, P=0.001). However, there was no significant improvement in SRT between the binaural hearing condition and unaided conditions in the S0N0 and S0NSSD conditions (P>0.05). The total score of THI and three dimensions were significant decreased (P<0.05). Tinnitus VAS scores were significantly lower in binaural hearing compared to the unaided condition (P<0.001). The total score of SSQ, and the scores of speech and spatial dimensions were significant improved in binaural hearing compared to the unaided condition (P<0.001). There was no statistical difference in NCIQ questionnaire scores between preoperative and postoperative (P>0.05), and only the self-efficacy subscore showed a significant increase(Z=-2.497,P=0.013). Conclusion: CI could help Mandarin-speaking SSD patients restore binaural hearing to some extent, improve sound localization and speech recognition in noise. In addition, CI in SSD patients could suppress tinnitus, reduce the loudness of tinnitus, and improve subjective perceptions of spatial hearing and quality of life.
目的： 评估汉语普通话成人单侧聋（single-sided deafness，SSD）患者人工耳蜗（CI）植入术后1年的康复效果。 方法： 本研究为单中心前瞻性队列研究，选取2020年8月至2021年10月在首都医科大学附属北京同仁医院接受CI植入的11例母语为汉语普通话的成人SSD患者作为研究对象，其中男性6例、女性5例，年龄24~50岁。在180°平均分布7个扬声器的声场中，测试患者术前、术后CI开机1、3、6和12个月时的声源定位角度均方根误差（root-mean-square error，RMSE），评估患者声源定位能力的改善情况。在声场中采用“空间言语评估系统”，测试患者在稳态噪声环境中不同信噪方位下裸耳（CI off）和助听（CI on）条件下的言语接受阈（speech reception threshold，SRT），以反映患者头影效应、双耳总和效应和静噪效应的获益情况。采用耳鸣致残量表（Tinnitus Handicap Inventory，THI）和视觉模拟评分（Visual Analogue Scale，VAS）评估SSD患者在术前、术后1个月、开机1个月、开机3个月、开机6个月和开机12个月时的耳鸣困扰和耳鸣响度的变化情况。采用言语、空间和音质听觉量表（The Speech，Spatial and Qualities of Hearing Scale，SSQ）和Nijmegen人工耳蜗植入量表（Nijmegen Cochlear Implant Questionnaire，NCIQ）评估患者CI植入后空间言语感知和生活质量等多方面主观获益情况。采用SPSS19.0软件进行相应统计学分析。 结果： SSD患者佩戴CI后，患耳助听听阈较裸耳听阈有明显改善；声源定位能力明显改善，各随访时间点RMSE与术前相比，差异均有统计学意义（P值均<0.05）。在反映头影效应的SSSDNNH测试条件下，患者在助听情况下的SRT较裸耳时平均降低6.5 dB，差异具有统计学意义（t=6.25，P=0.001）；在反映双耳总和效应的S0N0和反映静噪效应的S0NSSD测试条件下，SSD患者在裸耳和助听情况下的SRT差异无统计学意义（P值均>0.05）。术后各时间点THI问卷总分及3个维度得分与术前相比，差异均有统计学意义（P值均<0.05）；助听时VAS评分显著低于裸耳时VAS评分，差异有统计学意义（P<0.001）；助听下，SSQ问卷总分、言语理解和空间听觉分类得分均显著提高，差异均有统计学意义（P值均<0.01），NCIQ问卷总分与裸耳相比，差异无统计学意义（P>0.05），仅自信心分项得分较裸耳有显著增加，差异均有统计学意义（Z=-2.497，P=0.013）。 结论： CI植入可在一定程度上帮助汉语普通话SSD患者重建双耳听觉，改善声源定位能力和噪声环境下的言语识别能力，同时有助于减轻耳鸣困扰和耳鸣响度，提高空间听觉的主观感受。.

UNASSIGNED: Follow-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.
UNASSIGNED: Nurotron (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.
UNASSIGNED: From 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.
UNASSIGNED: All 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.
UNASSIGNED: Nurotron Venus CI System provides long-term, stable results in hearing speech assistance capabilities and can improve the quality of life of CI recipients.

本文报告1个伴视神经萎缩的综合征型听神经病家系，家系中2例患者（先证者及其父亲）临床表现为不同程度的感音神经性听力损失，同时伴有双眼视力下降。听力学检查提示纯音听阈与听性脑干反应（ABR）、畸变产物耳声发射（DPOAE）、耳蜗微音器电位（CM）不相符；眼眶及内耳MRI提示双侧视神经及听神经萎缩。基因检测发现其携带OPA1：c.1226 G＞A，并在家系中共分离。先证者在确诊后1年行右侧人工耳蜗植入，术后随访21个月，CI助听听阈（250 Hz-500 Hz-1 000 Hz-2 000 Hz-4 000 Hz）为30-40-45-45-40 dBHL，最大单音节词言语识别率44%。文献回顾12例OPA1基因变异患者人工耳蜗植入术后疗效，其助听听阈均接近正常值，其中，11例患者言语识别率明显改善，仅1例患者术后1年言语识别率无改善。结合本病例及文献回顾，OPA1变异所致综合征型听神经病患者可受益于人工耳蜗植入。.

Objective: To investigate the auditory and speech abilities of children with congenital auditory neuropathy (AN) after cochlear implant (CI), and to analyze the role of genetic testing in predicting the postoperative outcomes of CI in AN patients. Methods: Fourteen children diagnosed with AN by audiological battery test and underwent CI surgery in Xijing Hospital of the Air Force Medical University from 2002 to 2021 were included in this study (9 males and 5 females), with an implantation age of (3.1±1.7) years (mean±standard deviation, the same as follows). The preoperative audiological results and deafness gene results were analyzed. Another 52 children with ordinary sensorineural hearing loss (SNHL) were selected as the control group (30 males and 22 females), with an implantation age of (2.2±0.9) years. The demographic factors such as age and gender were matched with those of the AN group. The modified Category Auditory Performance (CAP-Ⅱ) and Speech Intelligence Rate (SIR) were used to evaluate the development of postoperative auditory and speech abilities in two groups. The Mandarin Speech Test System was used to test the speech recognition rate of monosyllabic and disyllabic words and sentences. Matlab 2022 software was used to analyze the data. Results: The results of gene in 14 children with AN showed that 6 cases had OTOF gene mutations, 2 cases (siblings) were confirmed to have TNN gene mutations through whole exome sequencing, and the remaining 6 cases were not find any clear pathogenic gene mutations. All subjects underwent CI surgery with electrodes implanted into the cochlea smoothly, and there were no postoperative complications. After surgery, all AN children had improved auditory and speech abilities, but only 64% (9/14) of AN children with CI had auditory ability scores comparable to the control group of SNHL children (including 2 children with TNN gene mutations), and 36% (5/14) of AN children had lower scores than the control group of SNHL children.The average speech recognition rate of two children with TNN gene mutations was 86.5%, and of two children with OTOF gene mutations was 83.2%. Conclusions: AN children achieved varying degrees of auditory and speech abilities after CI, but the postoperative effects varied greatly. Some children achieved similar results as ordinary SNHL children, but there were still some children whose effects were worse than those of ordinary SNHL children. The postoperative efficacy of CI in two children with AN caused by TNN pathogenic genes were comparable to that of ordinary SNHL in children. Genetic testing had certain reference value for predicting the postoperative effect of CI in AN children.
目的： 探讨先天性听神经病（auditory neuropathy，AN）儿童人工耳蜗（cochlear implant，CI）植入术后的听觉言语能力，并分析基因检测在AN患者CI术后效果预测中的作用。 方法： 研究对象为2002—2021年在西京医院经听力学检查诊断为AN并行CI手术的14例患儿，其中男9例、女5例，植入年龄（3.1±1.7）岁（均数±标准差，下同），分析其术前听力学特征及耳聋基因检测结果。另选52例行CI手术的普通感音神经性听力损失（sensorineural hearing loss，SNHL）患儿作为对照组，其中男30例，女22例，植入年龄（2.2±0.9）岁，其年龄、性别等人口统计学因素与AN组相匹配。使用改良版听觉能力分级（Category Auditory Performance，CAP-Ⅱ）和言语可懂度分级（Speech Intelligibility Rate，SIR）评价两组患者术后听觉言语能力的发展情况，采用心爱飞扬言语测听系统测试单双音节词和语句的言语识别率。使用Matlab 2022软件对数据进行分析和处理。 结果： 14例AN患儿耳聋基因检测显示6例为OTOF基因变异，2例（姐弟二人）经全外显子组测序证实为TNN基因变异，其余6例未发现明确致病性基因变异。所有患者术中电极均顺利植入耳蜗，术后未出现相关并发症。术后所有AN患儿的听觉和言语能力均有提高，但仅64%（9/14）的AN患儿CI术后听觉能力得分与对照组SNHL患儿相当（包括2例TNN基因变异的患儿），36%（5/14）的AN患儿听觉及言语能力得分明显低于对照组SNHL患儿。2例TNN基因变异患儿的言语识别率平均为86.5%，2例OTOF基因变异患儿的言语识别率平均为83.2%。 结论： AN患儿CI术后可获得不同程度的听觉和言语能力改善，但个体差异较大，部分患儿可达到与普通SNHL患儿相当的效果，但仍有部分患儿效果较差。TNN致病基因变异导致的AN患儿CI术后效果与普通SNHL儿童的效果相当，基因检测对预测AN患儿CI术后效果有一定参考价值。.

OBJECTIVE: This study aimed to explore the diagnostic sensitivity of 3D heavily weighted T2-weighted MRI (T2MRI) and high-resolution computed tomography (HRCT) in patients with cochlear fibrosis associated with Streptococcus suis (S. suis) meningitis and the practicality of Cochlear implantation (CI) treatments.
METHODS: Between January 2020 and December 2022, we enrolled four patients with rare cochlear S. suis meningitis with associated hearing loss despite aggressive or non-aggressive follow-up antibiotic treatment. Clinical imaging data, surgical performances and post-surgical-electrode impedance were evaluated.
RESULTS: Combined with HRCT and T2MRI, the cochlea had varying degrees of fibrosis and ossification in different cases. However, the electrodes were successfully and wholly inserted after intraoperative removal of the ossified and fibrotic foci. Post-surgical electrode impedance values of MP1 + 2 mode were normal in all 4 cases at initial activation.
CONCLUSIONS: In patients with S. suis meningitis and associated cochlear fibrosis, T2MRI examination of the inner ear was more sensitive than HRCT. This research highlights the feasibility of CI treatment in S. suis meningitis patients with severe cochlear fibrosis.

UNASSIGNED: 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.
UNASSIGNED: To investigate the short-term dynamic changes in hearing and cochlear histopathology in minipigs after electrode array insertion.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

OBJECTIVE: To evaluate a system for otomicrosurgery based on 4K three-dimensional (3D) exoscope technology and apply it to cochlear implantation.
METHODS: An open stereoscopic vision-based surgical system, which differs from traditional surgical microscopes, was created by utilizing 4K stereo imaging technology and combining it with low-latency 4K ultra-high-definition 3D display. The system underwent evaluation based on 57 cochlear implantation operations, three designed microscopic manipulations, and a questionnaire survey.
RESULTS: The surgical images displayed by the 4K-3D exoscope system (4K-3D-ES) are stereoscopic, clear, and smooth. The use of 4K-3D-ES in cochlear implantation is not inferior to traditional microscopes in terms of intraoperative bleeding and surgical complications, and the surgical duration is not slower or may even be faster than when using traditional microscopes. The results of micromanipulation experiments conducted on 16 students also confirmed this and demonstrated that 4K-3D-ES can be easily adapted. Furthermore, additional advantages of 4K-3D-ES were gathered. Significantly enlarged and high-definition stereoscopic images contribute to the visualization of finer anatomical microstructures such as chordae tympani, ensuring safer surgery. Users feel more comfortable in their necks, shoulders, waists, and backs. Real-time shared stereoscopic view for multiple people, convenient for collaboration and teaching. The ear endoscope and 4K-3D-ES enable seamless switching on the same screen. High-definition 3D images and videos can be saved with just one click, making future publication and communication convenient.
CONCLUSIONS: The feasibility and safety of 4K-3D-ES for cochlear implantation surgery have been demonstrated. The 4K-3D-ES also offers numerous unique advantages and holds clinical application and promotional value.

UNASSIGNED: Reliable predictors for rehabilitation outcomes in patients with congenital sensorineural hearing loss (CSNHL) after cochlear implantation (CI) are lacking. The purchase of this study was to develop a nomogram based on clinical characteristics and neuroimaging features to predict the outcome in children with CSNHL after CI.
UNASSIGNED: Children with CSNHL prior to CI surgery and children with normal hearing were enrolled into the study. Clinical data, high resolution computed tomography (HRCT) for ototemporal bone, conventional brain MRI for structural analysis and brain resting-state fMRI (rs-fMRI) for the power spectrum assessment were assessed. A nomogram combining both clinical and imaging data was constructed using multivariate logistic regression analysis. Model performance was evaluated and validated using bootstrap resampling.
UNASSIGNED: The final cohort consisted of 72 children with CSNHL (41 children with poor outcome and 31 children with good outcome) and 32 healthy controls. The white matter lesion from structural assessment and six power spectrum parameters from rs-fMRI, including Power4, Power13, Power14, Power19, Power23 and Power25 were used to build the nomogram. The area under the receiver operating characteristic (ROC) curve of the nomogram obtained using the bootstrapping method was 0.812 (95 % CI = 0.772-0.836). The calibration curve showed no statistical difference between the predicted value and the actual value, indicating a robust performance of the nomogram. The clinical decision analysis curve showed a high clinical value of this model.
UNASSIGNED: The nomogram constructed with clinical data, and neuroimaging features encompassing ototemporal bone measurements, white matter lesion values from structural brain MRI and power spectrum data from rs-fMRI showed a robust performance in predicting outcome of hearing rehabilitation in children with CSNHL after CI.

Unilateral deafness will lead to the decline of children\'s speech recognition rate, language development retardation and spatial positioning ability, which will have many adverse effects on children\'s life and study. Cochlear implantation can help children rebuild binaural hearing, and systematic audiological evaluation after operation is particularly important for clinicians to evaluate the hearing recovery of children. In this study, a variety of commonly used audiological evaluation, testing processes and methods after cochlear implantation in children with unilateral deafness are described in detail, and the related research status and results are summarized.
摘要: 单侧耳聋会导致儿童的言语识别率下降、语言发育迟缓、空间定位能力减退，对儿童的生活及学习产生多种不良影响。人工耳蜗植入可帮助患儿重建双耳听力，术后系统的听觉功能测评对临床医生掌握患儿听觉恢复情况尤为重要。本文综述了单侧聋患儿人工耳蜗植入术后多种常用的听觉功能测评方法，总结归纳了相关的研究结果和展望。.

Objective:To investigate the predictive value of temporal bone high-resolution CT（HRCT） multiplanar reconstruction（MPR） for cerebrospinal fluid（CSF） gusher during cochlear implantation in patients with inner ear malformation. Methods:The clinical data of 33 patients（36 ears） with inner ear malformation who underwent cochlear implantation were retrospectively analyzed. The predictive value of HRCT for cerebrospinal fluid gusher during cochlear implantation was evaluated. Results:The width of the cochlear foramen（P=0.024, OR=1.735） and the diameter of the inner auditory meatus（P=0.022, OR=6.119） were independent risk factors for CSF gusher during cochlear implantation. The area under the curve（AUC） of cochlear foramen width in predicting intraoperative gusher was 0.851, the sensitivity was 93.33%, and the specificity was 61.90%. The AUC of the upper and lower diameter of the internal auditory canal for predicting intraoperative gusher was 0.848, the sensitivity was 80.00%, and the specificity was 80.95%. The AUC of cochlear foramen width combined with the upper and lower diameters of the internal auditory meatus for predicting intraoperative gusher was 0.930, the sensitivity was 80.00%, and the specificity was 95.24%. Conclusion:Based on temporal bone HRCT, the prediction model of cochlear foramen width combined with the upper and lower diameter of the internal auditory canal has crucial predictive value for the \"gusher\" during cochlear implantation in patients with inner ear malformation.
目的：探讨颞骨高分辨率CT（HRCT）多平面重组对内耳畸形患者人工耳蜗植入术中脑脊液井喷的预测价值。 方法：回顾性分析33例（36耳）行CI内耳畸形患者的临床资料，评估其对CI中脑脊液井喷的预测价值。 结果：蜗孔宽度（P=0.024，OR=1.735）、内听道底上下径（P=0.022，OR=6.119）是内耳畸形患者CI中脑脊液井喷的独立危险因素。蜗孔宽度预测术中井喷的AUC=0.851，敏感度为93.33%，特异度为61.90%；内听道底上下径预测术中井喷的AUC=0.848，敏感度为80.00%，特异度为80.95%；蜗孔宽度联合内听道底上下径预测术中井喷的AUC=0.930，敏感度为80.00%，特异度为95.24%。 结论：基于颞骨HRCT的蜗孔宽度联合内听道底上下径预测模型对内耳畸形患者CI中“井喷”具有重要预测价值。.