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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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: The increase of bilateral cochlear implantation (CI) in recent years has made it essential to comprehend the effects of CI on otolith function. This study aimed to investigate the development of gross motor and otolith function in patients with inner ear malformations (IEMs) using vestibular-evoked myogenic potentials (VEMPs)., Materials and Methods: Overall, 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 testing was performed both before and 1-3 months after CI, and the evaluation of gross motor development was carried out., Results: The mean ages for achieving head control and independent walking were delayed in the IEM group compared with the control group (p = 0.02). The preoperative cervical VEMP (cVEMP) and ocular VEMP (oVEMP) response rates were higher in the control group (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 patients who elicited preoperative VEMPs waveform (n = 25)., Conclusions: Among SNHL patients, balance development is more delayed in patients of IEMs than in patients without IEMs. The cVEMP and oVEMP waveforms differed greatly between the two groups. The otolith-vestibular nerve conduction pathway can be affected by CI, potentially leading to otolith function impairment. Therefore, it is essential to assess otolith and balance functions before CI, and this evaluation should be considered an integral part of clinical practice., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Kenny-Caffey Syndrome type 2 (KCS2) is a genetic disease affecting bone metabolism. However, cochlear implantation (CI) results have yet to be published in detail., Objective: This study presents the gene, clinical characteristics, surgical outcomes, and literature review of 2 patients with sensorineural hearing loss related to KCS2. To enhance diagnostic detection and accuracy, we also compare the differential diagnosis between KCS2, otosclerosis, and Cogan's syndrome (CS)., Methods: Prior to CI, patients with KCS2 and CS underwent comprehensive audiological and radiological evaluations. Postoperative auditory speech outcomes and impedance values were recorded and analyzed statistically. A systematic search of the literature was conducted to summarize clinical characteristics., Results: Patients diagnosed with KCS2 exhibit more pronounced changes in the inner ear. The impedance values in the KCS2 cohort were considerably higher (Mean = 12.13 kΩ) than those with CS (Mean = 8.8 kΩ) one year post-activation. The literature review exhibits the clinical manifestations associated with KCS2., Conclusion: CI is an effective treatment for KCS2 to restore hearing loss. More frequent programming and accurate adjustment of stimulation is of great necessity. A thorough examination, including temporal bone HRCT, 3D-MRI, audiological evaluations, and whole-exome sequencing, is essential for the diagnosis and treatment of KCS2.

The difficulty of cochlear implantation in patients with congenital microtia is usually increased due to the vague anatomical marks and facial nerve malformation. The common types of facial nerve malformation include facial nerve bony cover loss, aberrant position, and bifurcation malformation. Bifurcation malformation may obscure the oval window, press against stapes, and bifurcate in the vestibular window while obscuring the round window. It is important to correctly identify the facial nerve and choose a reasonable surgical approach to avoid postoperative complications. This article describes a case of profound sensorineural hearing loss due to facial nerve malformation in our institution. The patient underwent cochlear implantation through the retro-facial approach. There was no facial nerve injury or dysfunction symptoms such as facial paralysis and hemifacial spasm 2 years after the operation, and the cochlear implant works well. The score of the categories of the auditory performance（CAP） questionnaire was 7, and the score of the speech intelligibility rating（SIR） questionnaire was 4. When the round window cannot be exposed through the facial recess approach during surgery, the retro-facial approach is a feasible method. To avoid facial nerve injury, a thin-section CT of the temporal bone should be performed before the middle and inner ear surgery for patients with facial nerve malformation, and the intraoperative facial nerve monitor should be used to clarify the course of the facial nerve to avoid injury., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

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 inter-group 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., Competing Interests: Declaration of competing interest There are no conflicts of interest, financial or otherwise, to declare., (Copyright © 2023 Elsevier B.V. All rights reserved.)

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.)

Purpose: Air-bone gap (ABG) is an essential indicator of middle ear transfer function after myringoplasty. However, there is still uncertainty about the mechanisms behind unexplained ABGs in patients post-myringoplasty. The present study investigated these mechanisms using cadaveric temporal bone (TB) measurement and finite element (FE) modeling., Methods: Three conditions of tympanic membrane (TM) perforation were modeled with a perforated area of 6%, 24%, and 50% of the total TM area to simulate a small, medium, or large TM perforation of TB model. A piece of paper was used to patch the TM perforation to simulate the situation post-myringoplasty. In the FE model for post-operation, the material properties at the perforation area were changed. Measurement of TM vibration at the umbo was undertaken with a laser Doppler vibrometer (LDV)., Results: As the perforated area increased vibration of the TM at the umbo decreased in both the TB and FE models. But the reduction of TM vibration is more minor in the FE model than in the TB model. After the perforation was repaired, the displacement of TM at the umbo could not be recovered totally in the TB and FE models. In the FE model, the displacement of TM at the umbo decreased markedly when the cone shape of TM flattened, and the reduction was almost the same as that in the TB model in the condition of large perforation., Conclusion: The material properties and the anatomical shape of the repaired TM could influence the TM's modal motion and wave motion. Except for appearance and shape current clinical instruments are unable to resolve factors that affect TM motion. Consequently the ABG seen post-myringoplasty remains unexplained., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: This study aimed to assess the correlation between residual hearing and audiologic outcomes after cochlear implant (CI) surgery in patients with cochlear nerve deficiency (CND)., Methods: This retrospective study included 57 patients with CND who underwent CI surgery. Patients were divided into four groups according to hearing level (80-95, 95-110, 110-120, and >120 dB) and three groups according to residual hearing (entire spectrum hearing, partial spectrum hearing, and no spectrum hearing) based on the measured response at each frequency. Auditory performance (categorical auditory performance [CAP], Infant-Toddler Meaningful Auditory Integration Scale [IT-MAIS]) and speech perception (speech intelligibility rating [SIR] and meaningful use of speech scale [MUSS]) were assessed before and 2 years after the surgery., Results: Forty-seven (82.5%) patients had complete or total hearing loss (≥95 dB) and 17 (29.8%) had no spectrum hearing before CI surgery. Twenty-nine (50.9%) patients did not exhibit residual hearing at 4 kHz. All patients demonstrated an improvement in auditory performance and speech perception: the CAP score in the 80-95 dB group was significantly higher than that in the 110-120 and >120 dB groups, and the entire spectrum hearing group showed significantly higher CAP, SIR, and IT-MAIS scores than the partial spectrum hearing group and significantly higher CAP, SIR, IT-MAIS, and MUSS scores than the no spectrum hearing group., Conclusion: For patients with CND, residual hearing, especially high-frequency residual hearing, was poor and postoperative audiologic outcomes were significantly associated with the range of residual hearing., Level of Evidence: 4., Competing Interests: None of the authors received any other funding and none have financial relationships or conflicts of interest to disclose., (© 2022 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals LLC. on behalf of The Triological Society.)

Introduction: Bilateral cochlear implantation has been shown to be beneficial in terms of sound localization and speech recognition in children with congenital deafness. However, little is known about the benefits of bilateral cochlear implantationin children who communicate in a tonal language such as Mandarin. This study aims to investigate the auditory perception and speech intelligibility of Mandarin-speaking children 1 year after first fitting., Materials and Methods: Twenty-nine children aged between 11.5 and 17.9 months with severe-to-profound bilateral sensorineural hearing loss were recruited; 10 were unilaterally implanted and 19 were bilaterally implanted. A test battery was used to monitor improvements during the first year of cochlear implant use., Results: Bilaterally implanted children scored better in the spatial domain of the Speech, Spatial and Qualities of hearing scale for Parents (SSQ-P) in comparison to unilaterally implanted children. Significant improvements were observed in auditory performance and speech intelligibility at 6 and 12 months after first fitting for both groups of children., Discussion: Young children that speak a tonal language, such as Mandarin, can obtain significant improvements in hearing and speech abilities within the first year of cochlear implant use. Furthermore, bilateral implantation provides users with better spatial hearing in comparison to unilateral implantation., (Copyright © 2022, Otology & Neurotology, Inc.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lu, Xie, Wei, Kong, Chen, Chen, Zhang, Yang, Xue, Shi, Liu, Xu, Dong, Chen, Li and Wang.)

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 mm 3 ; the surface area ranged from 27.81 to 284.7 mm 2 . When the lumen surface area was <190.45 mm 2 or the volume was <157.91 mm 3 , 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., Competing Interests: AD was employed by MED-EL Medical Electronics GmbH. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Zhang, Chen, Kong, Liau, Wei, Shi, Chen, Yang, Dhanasingh and Li.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Wei, Zhang, Lu, Yang, Chen, Chen, Zhang, Liu, Xian, Li and Kong.)

Objective: To study the emergency management of esophageal jujube pit ingestion., Study Design: Retrospective case series., Methods: A retrospective study of 114 consecutive cases of jujube pits esophageal impaction during 3 months was performed., Results: One hundred and fourteen cases were confirmed as jujube pit esophageal impaction using contrast-enhanced radiography. All jujube pits were retrieved using rigid esophagoscopy under general anesthesia as outpatients, except one case where a direct laryngoscope was used. In four cases, esophageal perforation was found, the patients were treated with conservative measures, and none died., Conclusion: Jujube pit esophageal impaction is characterized by a high incidence of perforation. The conservative management of cervical perforation is effective in this study. Rigid esophagoscopy under general anesthesia is safe for jujube pit esophageal impaction., Level of Evidence: 4., Competing Interests: The authors have no personal financial or institutional interests in any of the drugs, materials, or devices described in this article., (© 2021 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals LLC. on behalf of The Triological Society.)

Background: No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported., Aims/objectives: We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs)., Materials and Methods: Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed., Results: The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively., Conclusions and Significance: Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

Background: The presigmoid retrolabyrinthine approach (PRA) for small vestibular schwannoma (VS) has unique advantages. Combination with partial labyrinthectomy further makes up its defect of high demand for anatomy., Objective: Aimed to describe our use of the PRA with partial labyrinthectomy in VS surgery., Methods: We retrospectively analyzed 10 patients diagnosed with VS who underwent PRA between September 2017 and November 2020. Their perioperative data were analyzed., Results: Six tumors were on the left and three were on the right. One case was due to neurofibromatosis type 2 with bilateral involvement and underwent internal auditory canal decompression by PRA without tumor removal; all other patients underwent total tumor resection. Four patients underwent cochlear implantation simultaneously because of hearing loss that was detected during surgery. Three patients underwent partial labyrinthectomy. Five patients achieved hearing preservation, and one experienced hearing improvement. One of the three patients who underwent partial labyrinthectomy maintained hearing. All patients' pre- and postoperative facial nerve functions were House-Brackmann grade I. After a median follow-up time of 13 months, no tumors recurred., Conclusions: PRA for small VS is a safe and effective surgery that can achieve facial nerve and hearing preservation. Combination with partial labyrinthectomy can also preserve hearing.

Background: During surgical treatment of lesions involving the skull base, conductive hearing is often sacrificed due to tympanum destruction. This study aimed to develop a method for tympanum reconstruction using a sternocleidomastoid (SCM) flap to preserve conductive hearing during lateral skull base surgery., Methods: This study included five patients with lateral skull base lesions who underwent surgery including tympanum reconstruction with an SCM flap between July 2015 and November 2017., Results: Three patients seen with facial nerve schwannoma, and two had paraganglioma of the head and neck. All patients' inferior and posterior tympanic walls were resected; the tympanum and ossicular chain were reconstructed with an SCM flap and prosthesis. The mean postoperative air-bone gap was 14 dB. No lesion recurrences were observed until the last follow-up., Conclusion: The SCM flap is effective in preserving conductive hearing through reconstruction of the inferior and posterior tympanic walls in lateral skull base surgery., (© 2020 Wiley Periodicals LLC.)

Purpose: Simultaneous bilateral cochlear implantation is often seen as the gold-standard intervention for many people with bilateral deafness. However, this intervention is not as commonly performed in China. This paper presents the surgical technique and the peri- and postoperative safety results from simultaneous bilateral implantation and compares them with those from sequential bilateral implantation., Methods: Twenty nine children (aged 12‒18 months) met the inclusion/exclusion criteria and took part in this study. 10 participants received a unilateral cochlear implantation; 19 participants received simultaneous bilateral cochlear implantation. The same standard surgical technique (transmastoid facial recess approach with round window insertion) was used in all implantations. Safety was assessed via monitoring peri- and postoperative adverse events. The helpfulness of select surgical tools was assessed via questionnaire., Results: No adverse events were reported for any participants. All queried surgical tools were helpful in all 244 recorded responses., Conclusion: Simultaneous bilateral cochlear implantation can be performed using the same surgical technique as unilateral implantation and poses no increased safety risk for children aged 1‒2 years.

Background: During skull base tumor surgery, temporomandibular joint (TMJ) dissection is commonly performed. The impact of this procedure on patients' postoperative diet and TMJ function is a matter of concern to surgeons., Methods: We reviewed the Craniomandibular Index (CMI) for 32 patients (15 men and 17 women) who underwent TMJ dissection during surgery for skull base tumors between August 2015 and May 2018., Results: Fifteen patients underwent removal of the mandibular condyle, and 17 had the condyle preserved. Twenty-one patients mainly underwent infratemporal fossa approach, and 11 underwent extended temporal bone resection. No significant difference between pre- and postoperative diet was observed in any group. Significant differences in CMI index were seen in all groups. The highest score was 0.115 of Dysfunction Index, observed postoperatively in the group that underwent condyle removal., Conclusions: For skull base tumor surgery, TMJ dissection has no significant impact on postoperative diet. Patients who underwent removal of the mandibular condyle have significantly worse postoperative TMJ function.

Objectives: To determine the feasibility of endoscopic-assisted surgery for lesions involving the perilabyrinthine recesses, and develop and validate a technique for this kind of lesions that increases the possibility of preserving the labyrinth and cochlea., Study Design: Observational study., Setting: Referral center., Cases: Five patients who underwent endoscopy-assisted surgery for lateral skull base pathology involving the perilabyrinthine recesses between July 2010 and March 2016 were reviewed., Interventions: Clinical data of the five patients were collected., Main Outcomes Measures: Hearing level and recurrence., Results: Three of the five patients (three women, two men) had petrous bone cholesteatomas, one a facial neurofibroma, and one a petrous bone cholesterol granuloma. Complete excision with labyrinth and cochlea preservation was achieved using an endoscopic-assisted technique in all patients. Four had preoperative conductive hearing loss and one was totally deaf. In one patient, the postoperative air-bone gap was 30 dB lower than preoperatively levels, one stayed the same, and one was 30 dB because of closing of the external ear canal. After an average follow-up of 62.9 months, no patient had recurrence., Conclusions: Endoscope-assisted surgery is feasible for lesions involving the perilabyrinthine recesses and increases the likelihood to preserve the structure and function of labyrinth/cochlea, which may provide chances for artificial hearing devices implantation.

Objectives/hypothesis: Cochlear implantation for patients with common cavity deformity (CCD) can be difficult, with a higher incidence of intra- and postoperative complications; outcomes with CCD patients are also highly variable. In this study, surgical time was compared between the traditional facial recess approach (TFRA) and the transmastoid slotted labyrinthotomy approach (TSLA). Audiological outcomes and the benefit of using customized electrode arrays for CCD patients are also discussed., Study Design: Retrospective review of 13 cochlear implant (CI) patients with CCD., Methods: Six patients were implanted with the TFRA using traditional electrodes, and seven patients were implanted with the TSLA using customized electrodes. Intra- and postoperative complications were reviewed. Audiological outcomes were measured 3 months to 2 years after CI activation., Results: The mean surgical time for TSLA group was nearly half as long as for the TRFA group (P < .05). Although mean audiological outcomes were better for the TSLA group, there was no significant difference between groups (P > .05)., Conclusions: For CCD patients, TSLA for cochlear implantation is recommended due to shortened surgical time; customized electrode arrays may be additionally advantageous in terms of audiological outcomes., Level of Evidence: 4. Laryngoscope, 128:468-472, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To compare imaging and outcome data in cochlear implant (CI) patients with and without cochlear nerve deficiency (CND)., Study Design: Retrospective study comparing presurgical imaging (via high-resolution computed tomography and three-dimensional magnetic resonance imaging) to postsurgical auditory outcomes in CI patients with and without CND., Patients: Forty-three CI patients with CND diagnosed according to preoperative imaging were included in the CND group. A control group (non-CND; n = 43) was matched to the CND group in terms of age at implantation, CI device type, preoperative hearing threshold, and sex., Results: Across all subjects, internal auditory canal (IAC) diameter, cochlear nerve canal (CNC) diameter, and the number of nerve bundles were significantly correlated with all auditory outcome measures (p < 0.0125 in all cases). Across CND subjects, CNC diameter was significantly correlated with SIR, MUSS, and MAIS-IT/ MAIS scores (p < 0.0125 in all cases). Auditory outcomes were significantly poorer for CND patients with one rather than two nerve bundles. Across non-CND subjects, CNC diameter was significantly correlated only with SIR scores (p < 0.0125). IAC diameter, CNC diameter, and the number of nerve bundles were significantly smaller, and auditory outcomes were significantly poorer for the CND group than for the non-CND group (p < 0.05 in all cases). Results were similar when data only from patients ≤ 5 years old (n = 31) were analyzed., Conclusions: CNC diameter and the number of nerve bundles can significantly predict auditory outcomes for CI patients with CND. The results suggest that presurgical imaging may be useful in predicting CI outcomes for congenitally deaf patients.