Objectives To investigate the imaging characteristics, surgical approaches, and outcomes of cochlear implantation (CI) in patients with special inner ear malformations (IEMs) that show transitional forms between cochlear hypoplasia (CH) and common cavity (CC). Methods Twelve children (eight males, four females), aged 10 to 43 months, with special IEMs were enrolled, and their inner ear structures were analyzed using detailed segmentation. Two surgical approaches were employed: the transmastoid slot labyrinthotomy approach (TSLA) for cases requiring customized electrodes, and the round window or cochleostomy approach for the remaining cases. Outcomes were evaluated using Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), and Meaningful Auditory Integration Scale (MAIS/IT-MAIS) at 12 months post-implantation. Results Two main types of malformed cochleae were identified: common cavity-like and primitive CH types. All patients exhibited cochlear nerve deficiency and significant bilateral differences in their inner ear structures. Four patients underwent TSLA with customized electrodes, while the remaining patients received lateral wall electrodes via the round window or cochleostomy approach. Most patients showed improvement in auditory and speech capabilities following implantation. Conclusion Inner ear malformations with transitional forms between CH and CC present unique challenges, requiring detailed preoperative evaluation and customized surgical plans. Even in severe cases, carefully planned surgery can lead to meaningful auditory rehabilitation.

OBJECTIVE:This study aimed to investigate the long-term auditory and speech outcomes of sequential bilateral cochlear implantation (BiCI) in children with cochlear nerve deficiency (CND). METHODS:Sixteen CND children who underwent sequential BiCI were retrospectively reviewed. Auditory and speech abilities were evaluated using questionnaire-based rating scales, including the Categories of Auditory Performance (CAP), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and the Speech Intelligibility Rating (SIR). Closed-set and open-set speech recognition abilities were also measured. RESULTS:The first cochlear implantation (CI) was performed at a median age of 12 months. The mean age at contralateral CI was 43 months, and the mean inter-implant interval time was 26 months. All patients showed continuous auditory and speech improvement over time. Of the sixteen patients, nine completed closed-set and/or open-set speech recognition assessments. After contralateral CI, all nine patients showed improved closed-set speech recognition scores. Four patients achieved closed-set test scores of more than 90% and open-set disyllable recognition scores ranging from 44 to 85%. In addition, two patients demonstrated better performance with bilateral CI than with unilateral CI. CONCLUSIONS:CND children showed favorable long-term auditory and speech outcomes after sequential BiCI, and some demonstrated bilateral benefit in speech perception. These findings suggest that BiCI may be a viable option for selected CND children.

Objective:To quantitatively characterize the spatial relationships between intratemporal facial nerve segments and key cochlear implantation (CI) landmarks in cochlear hypoplasia (CH), and to compare these features with age-matched CI recipients with normal inner ear anatomy, thereby informing preoperative planning and facial nerve preservation strategies. Methods:CI recipients were reviewed retrospectively, and high-resolution temporal bone CT datasets were reconstructed. The measured parameters comprised cochlear-facial partition width (CFPW) of the labyrinthine segment, the distance between the tympanic facial nerve and oval window (FN‑OW), the distance between the mastoid facial nerve and round window (FN‑RW), the angle between the FN‑RW line and the anteroposterior axis (α), and the distance from the mastoid facial nerve to the posterior external auditory canal wall (FN‑EAC). Results:The CH cohort included 48 patients (91 ears: 40 CH‑Ⅱ, 43 CH‑Ⅲ, 8 CH‑Ⅳ); controls comprised 45 patients (90 ears). The median CFPW was larger in CH patients than in controls (0.7 vs. 0.57 mm; p < 0.01). Cochlear-facial dehiscence occurred at similar rates (CH 4.40% vs. controls 4.44%). FN-OW did not differ significantly between the two groups (2.33 vs. 2.37 mm; p = 0.198). A low‑lying tympanic segment was observed in 23.07% of CH ears but in none of the control ears. For the mastoid (vertical) segment, the CH group exhibited a shorter FN-RW (5.74 vs. 6.08 mm; p < 0.05) and a larger angle α (60.4° vs. 57.8°; p < 0.05). FN-EAC was slightly smaller in the CH group without reaching statistical significance (4.08 vs. 4.35 mm; p = 0.087). Conclusion:In CH, intratemporal facial nerve segments exhibit substantial positional variability. Anterior displacement of the vertical segment is common and may impede round window exposure via the facial recess. Preoperative HRCT should systematically assess nerve courses to reduce intra- and postoperative facial nerve complications.

BACKGROUND:Cochlear hypoplasia type I (CH-I) is a severe inner ear malformation often deemed a relative contraindication for cochlear implantation (CI), posing unique challenges for patients. OBJECTIVES:This study aimed to evaluate surgical techniques, challenges, and postoperative auditory outcomes in CH-I patients undergoing CI, identifying key prognostic factors. MATERIAL AND METHODS:A retrospective analysis of six CH-I patients (11-222 months old) who underwent CI between 2012 and 2023 was conducted. Five patients used the transmastoid facial nerve recess approach (TFNRA) with a short straight electrode, while one employed the transmastoid slot labyrinthotomy approach (TSLA) with a customized electrode. Outcomes were assessed via Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), and both closed-set and open-set speech tests, including the Mandarin Lexical Neighborhood Test (MLNT). RESULTS:Five patients achieved useable auditory function, with three demonstrating good open-set speech recognition. No significant differences were observed between unilateral and bilateral implantation. CONCLUSIONS AND SIGNIFICANCE:CI is viable for CH-I patients when TFNRA are combined with systematic auditory training. Preoperative cochlear morphology assessment and short straight electrodes are crucial for optimizing outcomes, emphasizing the need for long-term rehabilitation.

Objective:The aims of this study were to investigate the clinical applicability of 3D segmentation in measuring cochlear anatomical parameters, explore factors that influence the insertion angle of cochlear implant electrodes in patients with inner ear malformations, and determine the value of 3D segmentation in predicting cochlear implant electrode insertion depth by simulating electrode implantation in a reconstructed 3D model. Methods:Data from 208 temporal bone CT scans of patients with a variety of inner ear malformations (including the CH, IP-I, IP-II, and IP-III types) who underwent cochlear implantation at our center were retrospectively analyzed. Preoperative temporal bone CT data were subjected to three-dimensional (3D) segmentation of the cochlea with a 3D slicer. Results:Cochlear malformation types, including IP types I (42 ears), II (278 ears), III (20 ears), and CH (65 ears), were diagnosed and measured in 208 preoperative CT datasets. Cochlear anatomical parameters and electrode length were correlated, which partially explained the variations in electrode insertion angle. The mean angle of implantation among the enrolled patients was 564.33°, and the mean implantation angle prediction error in the 3D segmentation was |23.74|°. Conclusion:Three-dimensional segmentation from temporal bone CT is valuable for surgeons, especially in treating patients with inner ear malformation. Such insights will help surgeons understand overall anatomical variations, predict electrode implantation depth, and complete preoperative imaging assessments for cochlear implant insertion depth in patients with inner ear malformations.

Objective To assess the effectiveness of machine learning in automating the prediction of vestibular abnormalities after cochlear implantation (CI) in patients with sensorineural hearing loss (SNHL), with the goal of developing a practical model that can accurately predict long-term vestibular function outcomes and identify associated risk factors. Methods Clinical data, including imaging, vestibular evoked myogenic potentials (VEMPs), and auditory information, were collected from patients with sensorineural hearing loss (SNHL) before and after CI. The decision tree algorithm was employed to address missing values and screen pre-CI clinical features. Six machine learning methods were subsequently utilized to predict the relationships between the extracted features and post-CI vestibular dysfunction. The best-performing method determined the ranking of feature importance, which was regarded as risk factors for predicting symptoms and VEMPs results after CI. Results Logistic regression models effectively predicted both post-CI vestibular dysfunction and abnormal cervical VEMP (cVEMP), with accuracies of 80% and 78%, respectively. The relative importance of the features, in descending order, was as follows: cVEMP latency, cVEMP amplitude, and residual hearing threshold. Moreover, the support vector machine (SVM) model attained an accuracy of 88% in predicting abnormal ocular VEMP (oVEMP) post-CI. For the SVM model, the feature importance ranking was as follows: oVEMP latency, oVEMP amplitude, and residual hearing threshold. Conclusions This study successfully leverages machine learning techniques, specifically support vector machines (SVM) and logistic regression models, to predict the impact of CI on vestibular function. These predictive models provide valuable insights for presurgical planning and decision-making in CI procedures. Moreover, the findings highlight the critical risk factors associated with vestibular dysfunction, offering a robust reference for guiding vestibular rehabilitation strategies.

BACKGROUND:Head trauma often leads to bilateral sensorineural hearing loss (SNHL), and determining the optimal side for cochlear implantation (CI) remains a critical issue that requires further investigation. Objective: To evaluate the role of preoperative aided threshold (AT) testing in selecting the optimal CI side for patients with bilateral SNHL caused by head trauma. METHODS:This study included 9 patients with bilateral SNHL resulting from head trauma. Preoperative audiological and radiological evaluations were performed. The correlation between preoperative AT, duration of deafness (DoD), radiological findings, and Nijmegen Cochlear Implant Questionnaire (NCIQ) scores was analyzed. RESULTS:Preoperative AT showed a tendency for a negative correlation with advanced sound perception scores (r = -0.740, p = 0.057), while DoD exhibited a significant negative correlation with auditory self-confidence (r = -0.803, p = 0.029). Imaging combined with AT provides a foundational framework for selecting the implantation side; however, the limited sample size restricts the generalizability of the findings. CONCLUSION:Lower preoperative AT and shorter DoD may predict better auditory rehabilitation outcomes following CI. It is recommended to integrate imaging and audiological evaluations for individualized decision-making. Future studies with larger sample sizes are necessary to validate these findings and explore the clinical implications of early CI.

Background: With the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended. Objectives: To identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis. Methods: This study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared. Results: The cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X-2 = 18.228, P<0.001) (oVEMP: X-2 = 7.528, P = 0.023). Significant intergroup differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups. Conclusion: Patients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training.

Objective:To evaluate the effects of cochlear implantation in patients with single-sided deafness（SSD） and asymmetrical hearing loss（AHL）. Methods:Seventeen Mandarin-speaking CI patients diagnosed as SSD/AHL were recruited in our study. The Tinnitus Handicap Inventory（THI） and the Visual Analogue Scale（VAS） were used to assess changes in tinnitus distress and tinnitus loudness in SSD patients at each time point（pre-operation and post-operation）. Results:The THI score and all 3 dimensions were significant decreased with CI-on than pre-operation（P<0.05）. Tinnitus VAS scores were also decreased, and VAS scores were lower with CI-on than with CI-off, and were both significantly different at each time point after CI switch-on（P<0.05）. Conclusion:CI could help SSD/AHL patients to suppress tinnitus and reduce the loudness of tinnitus. However, CI should not be a treatment of tinnitus.

OBJECTIVE:The aim of this study was to conduct a bibliometric and visualization analysis of research on cochlear implantation (CI) for inner ear malformations (IEMs) from 1986 to 2024. METHODS:A comprehensive literature search was performed using the Web of Science Core Collection Database, resulting in the identification of 431 relevant publications. Various data analysis and visualization tools, including VOSviewer, CiteSpace, and Bibliometrix, were utilized to analyze annual publication outputs, countries/regions and institutions, authors, journals and studies, keywords, and theme evolution. RESULTS:The study revealed an overall increasing trend in research output on CI for IEMs, with significant contributions from countries such as the United States, China, Turkey, Germany, and Italy. The analysis also identified key authors, research teams, journals, and studies that have made substantial contributions to the field. Furthermore, the study highlighted important research hotspots and trends, such as the classification of IEMs, outcomes of CI for IEMs, and the management of pediatric patients with IEMs. CONCLUSION:The findings of this study provide a comprehensive overview of the research landscape surrounding CI for IEMs. The results serve as a basis for future research topic selection and emphasize the need for enhanced international collaboration and the publication of high-impact research to further advance this field.

Objectives: In recent years, with the incidence of bilateral cochlear implantation (CI) increasing, understanding the impact of CI on otolith function is of greater necessity. This study aims to investigate the development of gross motor and otolith function in patients with inner ear malformations (IEMs) by vestibular evoked myogenic potentials (VEMP). Materials and Methods: A total of 78 patients with sensorineural hearing loss (SNHL) (age 5.7±4.1 years) were divided into two groups based on the presence (IEM group, n=39) or absence (control group, n=39) of IEMs. VEMP was conducted before and 1–3 months after CI, and gross motor development assessed. Results: The mean ages of head control and independent walking were delayed in the IEM group compared with control group ( p=0.02). The preoperative cVEMP and oVEMP response rates were higher in the control groups (60% and 86.95%) than in the IEM group (57.69% and 74.35%) ( p<0.05). Additionally, abnormal cVEMP was associated with delayed acquisition of independent walking ( p=0.017). Saccular and utricular functions after CI were lost by 40% and 31.75%, respectively, in group of patients present preoperatively VEMPs waveform (n=25). Conclusions: Balance development is more reduced in patients with SNHL and IEMs than in patients without IEMs. The otolith-vestibular nerve conduction pathway can be affected by CI and lead to otolith function impairment. As such, evaluating the otolith and balance functions before CI is necessary and should be considered in clinical practice.

Purpose: The purpose of this study was to evaluate the auditory performance and speech perception of 104 children with isolated large vestibular aqueduct syndrome (LVAS) and 523 children with no inner ear malformation (IEM) for 5 years after cochlear implantation, in order to explore whether isolated LVAS can affect the long-term hearing and speech rehabilitation of deaf children after cochlear implantation. Method: A cohort study was established consisting of 627 children who underwent cochlear implantation at Beijing Tongren Hospital from 1999 to 2016. The children were examined at 0, 6, 12, 24, 36, 48, and 60 months after cochlear implantation to assess their auditory performance and speech perception using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) questionnaires. Results: The CAP scores of the isolated LVAS group increased significantly during the baseline to the 24th month, after which they gradually rose until reaching the plateau during the 24th to the 60th month. The CAP scores of the non-IEM group increased significantly during the baseline to the 36th month and then increased steadily. The SIR scores went up significantly during the baseline to the 48th month, and increased in a gradual manner in other stages of isolated LVAS evaluation. In comparison, The SIR scores of non-IEM group grew remarkably from the baseline to the 60th month. There were no significant differences in the CAP or SIR scores between isolated LVAS and non-IEM groups in each stage of evaluation, with the only exception being the CAP score at baseline month after cochlear implantation. Conclusions: The CAP and SIR questionnaires are helpful tools for quantifying the early hearing and speech skills of younger prelingually deafened cochlear implant recipients. This long-term follow-up study shows that the speech and hearing development of children in isolated LVAS and non-IEM groups follow similar patterns, and isolated LVAS does not affect the long-term rehabilitation of deaf children after cochlear implantation.

OBJECTIVE:The objectives of this study are twofold: first, to visualize the structure of malformed cochleae through image reconstruction; and second, to develop a predictive model for postoperative outcomes of cochlear implantation (CI) in patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation. METHODS:The clinical data from patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation who underwent cochlear implantation (CI) at Beijing Tongren Hospital between January 2016 and August 2020 were collected. Radiological features were analyzed through 3D segmentation of the cochlea. Postoperative auditory speech rehabilitation outcomes were evaluated using the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR). This study aimed to investigate the relationship between cochlear parameters and postoperative outcomes. Additionally, a predictive model for postoperative outcomes was developed using the K-nearest neighbors (KNN) algorithm. RESULTS:In our study, we conducted feature selection by using patients' imaging and audiological attributes. This process involved methods such as the removal of missing values, correlation analysis, and chi-square tests. The findings indicated that two specific features, cochlear volume (V) and cochlear canal length (CDL), significantly contributed to predicting the outcomes of hearing and speech rehabilitation for patients with inner ear malformations. In terms of hearing rehabilitation, the KNN classification achieved an accuracy of 93.3%. Likewise, for speech rehabilitation, the KNN classification demonstrated an accuracy of 86.7%. CONCLUSION:The measurements obtained from the 3D reconstruction model hold significant clinical relevance. Despite the considerable variability in cochlear morphology across individuals, radiological features remain effective in predicting cochlear implantation (CI) prognosis for patients with inner ear malformations. The utilization of 3D segmentation techniques and the developed predictive model can assist surgeons in conducting preoperative cochlear structural measurements for patients with inner ear malformations. This, in turn, can offer a more informed perspective on the anticipated outcomes of cochlear implantation.

目的 探讨听神经发育不良(cochlear nerve deficiency,CND)儿童人工耳蜗植入(cochlear implant,CI)术后听觉能力、言语能力的变化特点及发展规律,为此类儿童的诊治和康复提供参考.方法 使用婴幼儿有意义听觉整合量表(infant toddler-meaningful auditory integration scale,IT-MAIS)、有意义使用言语量表(meaningful use of speech scale,MUSS)、听觉能力分级(categories of auditory performance,CAP)和言语可懂度分级(speech in-telligibility rating,SIR)四个问卷量表,对42例内耳结构正常伴CND的CI儿童(A组)和189例内耳结构正常且听神经发育正常的CI儿童(B组)分别在术前、开机后1、3、6、9、12、24和36个月进行听觉言语能力评估.结果 两组患儿4个问卷得分均随着开机时间的延长而逐渐提高,且B组比A组增长趋势明显.IT-MAIS评估结果显示,在开机后6、9个月时两组得分差异有统计学意义(P＜0.05);MUSS评估结果显示,在开机后24、36个月时两组得分差异有统计学意义(P＜0.05);CAP结果显示,在开机后6、9、12、24个月时两组得分差异有统计学意义(P＜0.05);SIR结果显示,在术前(开机)、开机后9、12、24和36个月时两组得分差异有统计学意义(P＜0.05).结论 CND儿童人工耳蜗植入术后听觉和言语能力均随术后时间的延长呈增长趋势,且发展趋势与听神经发育正常的CI儿童相同,但是发展速度滞后.CND的CI儿童听觉能力在开机后36个月可以接近或达到听神经发育正常的CI儿童,但言语能力与听神经发育正常CI儿童仍有较大差距.

PurposeDue to the specificity of cochlear implantation (CI) programming parameters and outcomes in cochlear nerve deficiency (CND) patients, this study aimed to investigate the correlation between programming parameters and outcomes and further compare the difference between normal and CND groups.MethodsNinety (95 ears) CND patients (normal cochlea, 39; malformed cochlea, 56) and seventy-nine (81 ears) normal cochlea patients who underwent CI surgery with either Med-El or Cochlear devices were included. The programming parameters and outcomes evaluated by the questionnaires were collected and compared among the normal CND, malformed CND, and normal groups in the two device groups, and their correlation was analyzed.ResultsIn the CND group, a reduced stimulation rate, higher pulse width, and triphasic pulse were needed in some cases. The stimulus levels of the CND group were significantly higher than that of the normal group (p < 0.05), but the outcomes of the CND group were significantly worse than that of the normal group (p < 0.05), and the stimulus level was significantly correlated with the outcomes (p < 0.05). However, there was no difference between normal and malformed CND groups. The non-auditory response was observed in the CND group, especially the ones with malformations.ConclusionThe CI programming parameters of some CND patients need to be adjusted, and a slower stimulation rate and higher pulse width are required sometimes. CND patients need a higher stimulus level than normal patients but their outcomes are poorer. Non-auditory response should be noticed in CND patients during programming.

Different from normal-hearing (NH) listeners, speech recognition thresholds (SRTs) in cochlear implant (CI) users are typically poorer with dynamic maskers than with speech-spectrum noise (SSN). The effectiveness of different masker types may depend on their acoustic and linguistic characteristics. The goal of the present study was to evaluate the effectiveness of different masker types with varying acoustic and linguistic properties in CI and NH listeners. SRTs were measured with nine maskers, including SSN, dynamic nonspeech maskers, and speech maskers with or without lexical content. Results showed that CI users performed significantly poorer than NH listeners with all maskers. NH listeners were much more sensitive to masker type than were CI users. Relative to SSN, NH listeners experienced significant masking release for most maskers, which could be well explained by the glimpse proportion, especially for maskers containing similar cues related to fundamental frequency or lexical content. In contrast, CI users generally experienced negative masking release. There was significant intercorrelation among the maskers for CI users' SRTs but much less so for NH listeners' SRTs. Principal component analysis showed that one factor explained 72% of the variance in CI users' SRTs but only 55% in NH listeners' SRTs across all maskers. Taken together, the results suggest that SRTs in SSN largely accounted for the variability in CI users' SRTs with dynamic maskers. Different from NH listeners, CI users appear to be more susceptible to energetic masking and do not experience a release from masking with dynamic envelopes or speech maskers.

Objectives: Talker sex and spatial cues can facilitate segregation of competing speech. However, the spectrotemporal degradation associated with cochlear implants (CIs) can limit the benefit of talker sex and spatial cues. Acoustic hearing in the nonimplanted ear can improve access to talker sex cues in CI users. However, it’s unclear whether the CI can improve segregation of competing speech when maskers are symmetrically placed around the target (i.e., when spatial cues are available), compared with acoustic hearing alone. The aim of this study was to investigate whether a CI can improve segregation of competing speech by individuals with unilateral hearing loss. Design: Speech recognition thresholds (SRTs) for competing speech were measured in 16 normal-hearing (NH) adults and 16 unilaterally deaf CI users. All participants were native speakers of Mandarin Chinese. CI users were divided into two groups according to thresholds in the nonimplanted ear: (1) single-sided deaf (SSD); pure-tone thresholds <25 dB HL at all audiometric frequencies, and (2) Asymmetric hearing loss (AHL; one or more thresholds > 25 dB HL). SRTs were measured for target sentences produced by a male talker in the presence of two masker talkers (different male or female talkers). The target sentence was always presented via loudspeaker directly in front of the listener (0°), and the maskers were either colocated with the target (0°) or spatially separated from the target at ±90°. Three segregation cue conditions were tested to measure masking release (MR) relative to the baseline condition: (1) Talker sex, (2) Spatial, and (3) Talker sex + Spatial. For CI users, SRTs were measured with the CI on or off. Results: Binaural MR was significantly better for the NH group than for the AHL or SSD groups (P < 0.001 in all cases). For the NH group, mean MR was largest with the Talker sex + spatial cues (18.8 dB) and smallest for the Talker sex cues (10.7 dB). In contrast, mean MR for the SSD group was largest with the Talker sex + spatial cues (14.7 dB), and smallest with the Spatial cues (4.8 dB). For the AHL group, mean MR was largest with the Talker sex + spatial cues (7.8 dB) and smallest with the Talker sex (4.8 dB) and the Spatial cues (4.8 dB). MR was significantly better with the CI on than off for both the AHL (P = 0.014) and SSD groups (P < 0.001). Across all unilaterally deaf CI users, monaural (acoustic ear alone) and binaural MR were significantly correlated with unaided pure-tone average thresholds in the nonimplanted ear for the Talker sex and Talker sex + spatial conditions (P < 0.001 in both cases) but not for the Spatial condition. Conclusion: Although the CI benefitted unilaterally deaf listeners’ segregation of competing speech, MR was much poorer than that observed in NH listeners. Different from previous findings with steady noise maskers, the CI benefit for segregation of competing speech from a different talker sex was greater in the SSD group than in the AHL group.

OBJECTIVE:Cochlear nerve deficiency (CND) is often combined with modiolar deficiency-type inner ear malformations, which cause variable cochlear implantation (CI) outcomes. We aimed to assess the postoperative development of auditory and speech perception in CND patients with modiolar deficiency-type malformations after 3 years of follow-up to determine the factors correlated with CI outcomes.METHODS:Sixty-seven CND patients with modiolar deficiency-type malformations who underwent CI surgery were retrospectively reviewed. Modiolar deficiency-type malformations included common cavity (CC), cochlear hypoplasia (CH) (including CH-I and CH-II) and incomplete partition-I (IP-I). Categorical auditory performance (CAP) and the infant-toddler meaningful auditory integration scale (MAIS) were used to assess auditory ability. The speech intelligibility rating (SIR) and meaningful use of speech scale (MUSS) were used to assess the speech intelligibility of these CI patients. The CI outcomes were evaluated at 0, 12, 24 and 36 months after implant activation.RESULTS:All patients demonstrated improvements in auditory ability and speech intelligibility after CI. There were no significant differences in CI outcomes at any time point according to the malformation type. The number of nerve bundles within the internal auditory canal (IAC) showed significant differences at 12, 24 and 36 months after CI ( p < 0.05). Patients with one nerve bundle had relatively poor CI outcomes.CONCLUSIONS:CND patients with modiolar deficiency-type malformations showed continuous improvement in auditory and speech abilities after CI. Compared with malformations, the number of nerve bundles should be given more attention when selecting the side for CI.

Objective Rudimentary otocyst (RO) is characterized by an otic capsule without an internal auditory canal, which is considered a contraindication to cochlear implantation (CI). In this study, we were the first to report two patients with ROs who underwent CI. Patient Two patients (18 months old and 2 years old) presenting with bilateral congenital hearing loss were diagnosed with ROs. Intervention CI was performed. The transmastoid slotted labyrinthotomy approach was used with customized MED-EL electrode arrays. Main Outcome Measures Categorical auditory performance, infant-toddler meaningful auditory integration of sound, the speech intelligibility rating, and meaningful use of speech scale. Results Both children could understand common phrases and had intelligible, connected speech 2 years after CI. Conclusion With proper indication, surgical approach and postoperative training, a child with an RO may benefit from CI.

目的 分析年龄因素对语前聋人工耳蜗植入儿童术后前语言交流能力的影响,初步获得不同年龄语前聋人工耳蜗植入儿童术后前语言交流能力发展规律.方法 按照人工耳蜗植入年龄将36例儿童分为三组:组1包含13例受试者,植入年龄12～24个月,平均18.4±3.7个月;组2包含13例受试者,植入年龄24～36个月,平均28.7±2.9个月;组3包含10例受试者,植入年龄36～48个月,平均42.8±4.0个月.分别于开机时及开机后1、3、6、9、12个月使用录像分析法对患儿的轮流交流、主动交流、听觉注意和视觉交流四项前语言交流能力进行分析和评估.结果 三组儿童的轮流交流和听觉注意能力都随开机时间的延长呈显著增长趋势;在开机6个月时,组1和组2的轮流交流能力得分较组3得分低,差异有统计学意义(P＜0.05),余评估阶段组间轮流交流能力两两比较差异无统计学意义(P＞0.05);在开机6个月和12个月时,组1和组2的听觉注意能力得分较组3低,差异有统计学意义(P＜0.05),余评估阶段组间听觉注意能力两两比较差异无统计学意义(P＞0.05).三组患儿开机后第一年主动交流和视觉交流这两项前语言交流能力都没有显著变化(P＞0.05),且组间对比无统计学差异(P＞0.05).结论 植入年龄对语前聋人工耳蜗植入儿童术后的前语言能力发展有一定影响;轮流交流和听觉注意能力可以作为汉语言儿童前语言交流能力的主要指标,主动交流和视觉交流作为参考指标.