[Cochlear implantation through retro-facial approach with congenital microtia malformation with facial nerve deformity: a case report].

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.

Machine learning-based prediction of the outcomes of cochlear implantation in patients with inner ear malformation.

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.

Cochlear implantation programming characteristics and outcomes of cochlear nerve deficiency.

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.

A 3-Year Follow-Up and Radiological Analysis of Cochlear Implantation Patients with Cochlear Nerve Deficiency and Modiolar Deficiency-Type Malformations.

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.

Assessment of the correlation between residual hearing and audiologic outcomes after cochlear implantation in patients with cochlear nerve deficiency.

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.

Machine Learning-Based Prediction of the Outcomes of Cochlear Implantation in Patients With Cochlear Nerve Deficiency and Normal Cochlea: A 2-Year Follow-Up of 70 Children.

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.

Analysis of Long-Term Cochlear Implantation Outcomes and Correlation With Imaging Characteristics in Patients With Common Cavity Deformity.

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.

Hearing preservation/rehabilitation surgery for small vestibular schwannoma: preliminary experience with the presigmoid retrolabyrinthine approach.

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.

A new phenomenon of cochlear otosclerosis: an acquired or congenital disease? - A clinical report of cochlear otosclerosis.

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.

Application of Multiplanar Volume Reconstruction Technique for the Assessment of Electrode Location and Analysis of the Correlation to Cochlear Programming and Performance in Common Cavity Deformity.

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.

Tympanum reconstruction using a sternocleidomastoid flap in patients with lateral skull base lesions: surgical technique and clinical report.

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.

Simultaneous bilateral cochlear implantation in children aged 12‒18 months is safe and can be performed using standard cochlear implant surgical techniques.

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.

Functional Outcomes Following Temporomandibular Joint Dissection Performed During Surgery for Skull Base Tumors.

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.

Combined Endoscopic and Microscopic Surgery for Perilabyrinthine Lesions: Feasibility and Technique.

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.

Slotted labyrinthotomy approach with customized electrode for patients with common cavity deformity.

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.