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Purpose: Due 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., Methods: Ninety (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., Results: In 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., Conclusion: The 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., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

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., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

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., Competing Interests: Q.F. has a financial interest at Nurotron Biotechnology Co. Ltd., a medical device company that designs, develops, and markets CI systems. The remaining authors have no conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Cochlear ossification (CO) after meningitis can cause profound sensorineural hearing loss (SNHL). Cochlear implantation (CI) is the ideal treatment strategy for CO., Aims: To explore the strategy for CI in patients with CO after meningitis., Materials and Methods: In this retrospective study, the medical records of patients diagnosed with profound SNHL due to CO after meningitis and who underwent CI in our department between September 2010 and September 2021 were collected and reviewed. Their imaging and surgical findings were analyzed., Results: The data of 26 patients with unilateral CI were reviewed. All patients underwent preoperative temporal high-resolution computed tomography (HRCT) and 22 patients magnetic resonance imaging (MRI). The sensitivity of HRCT was 61.5% (10/26), whereas that of MRI was 81.8% (18/22). Combined HRCT and MRI achieved a detection rate of 92.3% (24/26). Twenty-two and four patients underwent complete and partial electrode implantations, respectively., Conclusions and Significance: Preoperative temporal bone HRCT and MRI are essential for determining whether a patient is suitable for CI and surgical planning. A false-negative diagnosis is possible when diagnosing CO, but combined HRCT and MRI can improve the positive rate of preoperative diagnosis of CO post meningitis. Early CI is recommended.

Background: The aim of this study was to find out how candidacy criteria have evolved differently across the globe., Methods: Candidacy criteria and outcome measurements applied in 19 HEARRING clinics were analyzed., Results: Candidacy criteria vary between clinics. Overall, both bilateral implantation and cochlear implantation in patients with single-sided deafness are becoming more frequent., Conclusion: Standardized outcome measurement instruments need to be applied to provide access to the hearing world to all patients with hearing loss who would benefit from cochlear implantation.

Purpose: We aimed to analyse the reasons behind the need for cochlear implant revision surgeries, as well as the rate at which they occur, to reduce the revision surgery rate for non-device failures. We also aimed to elucidate the cumulative survival and device survival rates in different age groups., Methods: This retrospective single cohort study reviewed 4563 cochlear implant surgeries and 119 revision surgeries performed at a tertiary referral hospital in China between 1996 and 2019. Kaplan-Meier curves were used to calculate the cumulative survival and device survival rates., Results: The revision surgery rate was 2.61%. The reasons for revision included device (73.1%) and non-device (26.9%) failures. The most common reasons were hard device (47.1%) and non-device failure (28.6%). The 10- and 20-year cumulative survival rates were 96.8% and 96.7%, respectively. Younger children were more likely to undergo a second surgery., Conclusion: This study is the longest study about revision surgery in China. Cochlear implantation is a reliable treatment. It has a low complication rate in patients with sensorineural hearing loss. Children have a higher revision rate than adults. Doctors should be aware of each complication and perform the appropriate procedure., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Generally, cochlear implants (CIs) are effective in helping patients improve their hearing performance; however, some patients have poor hearing performance owing to facial nerve stimulation (FNS), which is often associated with cochlear anomalies. We report a case with a normal cochlea and severe and persistent FNS owing to cochlear-facial dehiscence (CFD) that affected the CI outcomes. Preoperatively, a careful review of the computed tomography images before CI surgery is necessary not only for patients with otosclerosis and inner ear malformations but also for patients with normal cochlear structures because facial nerve anomalies could be present.

Mandarin is a tonal language, and it is important to preserve lexical tone information in synthesized speech. With natural speech, Chinese cochlear implant (CI) users have difficulty perceiving voice pitch cues important for lexical tone perception; it is unclear whether this difficulty persists in Mandarin synthesized speech. In this study, intelligibility of naturally produced and synthesized Mandarin speech was measured in Chinese CI listeners; intelligibility was also measured in a control group of normal-hearing (NH) listeners. Five synthesized voices were selected to represent different talker genders (male, female, child), speaking rates (normal, slow), and speaking styles (emotional, accent). The data showed that while modern Mandarin text-to-speech (TTS) systems can provide perfect speech intelligibility for NH listeners, overall intelligibility was much poorer for CI than for NH listeners. CI performance was significantly poorer with synthesized speech than with natural speech (p < 0.001). CI listeners were highly sensitive to the "extra-atypical" synthesized emotional and accented speech. Performance with each of the synthesized speech types was significantly correlated with performance with natural speech in CI users (p < 0.01 in all cases). While modern TTS systems offer educational and communication benefits to CI users and hearing-impaired individuals, the selection of synthesized voices should be carefully considered in education applications of TTS for hearing-impaired individuals, especially CI children, since poor intelligibility performance may affect language learning.

Objectives: Speech materials validated with normal-hearing listeners may not be appropriate for clinical assessment of cochlear implant (CI) users. The aim of this study was to validate list equivalency of the Mandarin Speech Perception (MSP) sentences, disyllables, and monosyllables in Mandarin-speaking CI patients., Design: Recognition of MSP sentences, disyllables, and monosyllables each were measured for all 10 lists., Study Sample: 67 adult and 32 pediatric Mandarin-speaking CI users., Results: There was no significant difference between adult and pediatric subject groups for all test materials. Significant differences were observed among lists within each test. After removing one or two lists within each test, no significant differences were observed among the remaining lists. While there was equal variance among lists within a given test, the variance was larger for children than for adults, and increased from monosyllables to disyllables to sentences., Conclusions: Some adjustment to test lists previously validated with CI simulations was needed to create perceptually equivalent lists for real CI users, suggesting that test materials should be validated in the targeted population. Differences in mean scores and variance across test materials suggest that CI users may differ in their ability to make use of contextual cues available in sentences and disyllables.

Conclusion: This study indicates that Mandarin-speaking subjects acquire significant benefit from a cochlear implant (CI) and audio processor with the fine structure processing (FSP) coding strategy. It can take adult Mandarin CI users time to become accustomed to their CI and appreciate the benefits., Objectives: This study aimed to determine speech perception outcomes in Mandarin-speaking adults who received the FSP coding strategy., Methods: This was a prospective study. Ten adults who were implanted unilaterally with a PULSARCI(100), and received an OPUS 1 or OPUS 2 speech processor, were included in this study. The mean age at implantation was 31.1 years. We determined the benefit to postlingually deafened Mandarin-speaking adults with a severe to profound hearing loss using the tests: monosyllables in quiet, sentences in quiet and the Mandarin Hearing in Noise Test (MHINT). Tone perception was evaluated using a tone perception test. A visual analog scale (VAS) was used to score sound quality and ease of listening., Results: The results showed a significant improvement between first fitting and 6 months in all speech tests and in tone perception. The VAS scores showed a significant improvement over 6 months in sound quality and an immediate benefit in ease of listening.

Objective: The full benefit of bilateral cochlear implants may depend on the unilateral performance with each device, the speech materials, processing ability of the user, and/or the listening environment. In this study, bilateral and unilateral speech performances were evaluated in terms of recognition of phonemes and sentences presented in quiet or in noise., Design: Speech recognition was measured for unilateral left, unilateral right, and bilateral listening conditions; speech and noise were presented at 0° azimuth. The 'binaural benefit' was defined as the difference between bilateral performance and unilateral performance with the better ear., Study Sample: Nine adults with bilateral cochlear implants participated., Results: On average, results showed a greater binaural benefit in noise than in quiet for all speech tests. More importantly, the binaural benefit was greater when unilateral performance was similar across ears. As the difference in unilateral performance between ears increased, the binaural advantage decreased; this functional relationship was observed across the different speech materials and noise levels even though there was substantial intra- and inter-subject variability., Conclusions: The results indicate that subjects who show symmetry in speech recognition performance between implanted ears in general show a large binaural benefit.

Cochlear implant (CI) users' speech understanding may be influenced by different speaking styles. In this study, speech recognition was measured in Mandarin-speaking CI and normal-hearing (NH) subjects for sentences produced according to four styles: slow, normal, fast, and whispered. CI subjects were tested using their clinical processors; NH subjects were tested while listening to a four-channel CI simulation. Performance gradually worsened with increasing speaking rate and was much poorer with whispered speech. CI performance was generally similar to NH performance with the four-channel simulation. Results suggest that some speaking styles, especially whispering, may negatively affect Mandarin-speaking CI users' speech understanding., (© 2011 Acoustical Society of America)

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. [ABSTRACT FROM AUTHOR]

Cochlear nerve deficiency (CND) is often associated with variable outcomes of cochlear implantation (CI). We assessed previous investigations aiming to identify the main factors that determine CI outcomes, which would enable us to develop predictive models. Seventy patients with CND and normal cochlea who underwent CI surgery were retrospectively examined. First, using a data-driven approach, we collected demographic information, radiographic measurements, audiological findings, and audition and speech assessments. Next, CI outcomes were evaluated based on the scores obtained after 2 years of CI from the Categories of Auditory Performance index, Speech Intelligibility Rating, Infant/Toddler Meaningful Auditory Integration Scale or Meaningful Auditory Integration Scale, and Meaningful Use of Speech Scale. Then, we measured and averaged the audiological and radiographic characteristics of the patients to form feature vectors, adopting a multivariate feature selection method, called stability selection, to select the features that were consistent within a certain range of model parameters. Stability selection analysis identified two out of six characteristics, namely the vestibulocochlear nerve (VCN) area and the number of nerve bundles, which played an important role in predicting the hearing and speech rehabilitation results of CND patients. Finally, we used a parameter-optimized support vector machine (SVM) as a classifier to study the postoperative hearing and speech rehabilitation of the patients. For hearing rehabilitation, the accuracy rate was 71% for both the SVM classification and the area under the curve (AUC), whereas for speech rehabilitation, the accuracy rate for SVM classification and AUC was 93% and 94%, respectively. Our results identified that a greater number of nerve bundles and a larger VCN area were associated with better CI outcomes. The number of nerve bundles and VCN area can predict CI outcomes in patients with CND. These findings can help surgeons in selecting the side for CI and provide reasonable expectations for the outcomes of CI surgery. [ABSTRACT FROM AUTHOR]

Object: To investigate the long-term development of auditory and speech in patients with common cavity deformity (CCD) after cochlear implantation (CI) and its relationship to imaging characteristics. Methods: Twenty-three CCD patients and 59 age- and sex-matched CI children with normal inner ear structure were recruited. The auditory and speech development of these two groups were evaluated at 0, 1, 3, 6, 12, and 18 months after CI activation using four parent reports questionnaires [Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), Meaningful Auditory Integration Scale/Infant-Toddler Meaningful Auditory Integration Scale (MAIS/ITMAIS), and Meaningful Use of Speech Scale (MUSS)]. Computed tomography-based 3-dimensional reconstruction of the surgical side of 18 CCD children was performed, the volume and surface area were calculated. Correlation analysis was performed on the imaging performance and post-operative outcomes. Results: The percentages of MAIS/IT-MAIS scores and CAP scores at different evaluation time points are significantly different (p < 0.05). When comparing SIR results across time points, significant growth was observed in most of the comparisons. In addition, significant differences (p < 0.05) are observed among the percentages of MUSS scores at different time points except the comparison between 0 and 1 month after CI activation. Patients in the CCD group had poorer auditory and speech performances at different stages after CI compared with those in the control group. According to the reconstruction of CCD patients, the volume ranged from 12.21 to 291.96 mm3; the surface area ranged from 27.81 to 284.7 mm2. When the lumen surface area was <190.45 mm2 or the volume was <157.91 mm3, the survival time for CCD children to achieve a CAP score of 4 after CI was significantly shorter. Conclusion: Cochlear implantation are less effective in CCD patients than in patients with normal inner ear structures, but they can still achieve significant improvement post-operatively. The morphology and size of the inner ear vary in CCD patients, which reflects the degree of inner ear development influences the outcome after CI surgery. [ABSTRACT FROM AUTHOR]

Purpose: Owing to the characteristic anatomy, cochlear implantation (CI) for common cavity deformity (CCD) has resulted in varied outcomes and frequent facial and vestibular nerve stimulation. The current study analyzed the correlation among the distance between each electrode and cavity wall (abbreviation, D), programming parameters, and performances outcomes. Materials and Methods: The current, retrospective study included 25 patients (27 ears) with CCD underwent CI. The multiplanar volume reconstruction (MPVR) techniques were employed to reconstruct and evaluate the postoperative temporal bone CT. The D and maximum comfortable level (MCL) 6 months after CI, facial and vestibular nerve stimulation, and outcomes 1, 2, and 3 years after CI pertaining to the questionnaires were documented and analyzed. Results: The patients were divided into symptomatic (10, 37%) and asymptomatic (17, 63%) groups according to with or without facial and vestibular nerve stimulation. The MCL pertaining to the symptomatic group was significantly lower than asymptomatic group, but Categories of Auditory Performance (CAP) scores 1 year after surgery was better (p < 0.05). The subjects were divided into flat (12, 44.4%) and curved (15, 55.6%) groups based on the contour of MCL map. The MCL and D were lower and shorter in the curved group than the flat group, and CAP score 1 year after surgery and Speech Intelligibility Rating (SIR) 3 years after surgery were better (p < 0.05). Conclusion: Although abnormal reactions such as facial and vestibular nerve stimulation were observed to be more frequent, lower MCL and better outcomes were observed in relation to the shorter D. [ABSTRACT FROM AUTHOR]

Cochlear implantation is safe for simple case presentations. However, facial nerve (FN) aberrations may impede insertion of the electrode through a round window and increase the risk of iatrogenic FN injury. We developed a novel method of cochlear implantation, a present the case of a child with FN aberrations. Inferior displacement of a horizontal segment of FN lay on the surface of th e promontory, obscuring the round window. The oval window was used and the electrode successfully inserted. No postoperative complications occurred, and significant improvement in speech perception was achieved. This novel approach is suitable for patients with FN aberrations. [ABSTRACT FROM AUTHOR]

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.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.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.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.Methods: 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.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.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.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.Results: 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.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.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.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.Conclusion: 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.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.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.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. [ABSTRACT FROM AUTHOR]

Objective To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation. Methods Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS). Results All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition. Conclusion Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months. [ABSTRACT FROM AUTHOR]