Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

Background: Vestibular dysfunction is a complication of cochlear implantation (CI). Reports on the evaluation of vestibular function before and after CI are limited, especially in children. We investigated the effect of CI on vestibular function in pediatric patients.Patients and Methods: We routinely evaluated vestibular function before but not immediately after CI. Therefore, patients who underwent sequential bilateral CI were enrolled in this study. Seventy-three children who underwent sequential CI from 2003 to 2020 at our hospital were included. Since the vestibular function of the first implanted ear was evaluated before the second surgery for the contralateral ear, post-CI evaluation timing differed among the cases. The evaluation included a caloric test, a cervical vestibular-evoked myogenic potential (cVEMP) test, and a damped rotation test. The objective variables included the results of these tests, and the explanatory variables included the age at surgery, cause of hearing loss, electrode type, and surgical approach used. The associations of these tests were analyzed.Results: cVEMP was the most affected after CI (36.1%), followed by the caloric test (23.6%), and damped rotation test (7.8%). Cochleostomy was significantly more harmful than a round window (RW) approach or an extended RW approach based on the results of the caloric test (p = 0.035) and damped rotation test (p = 0.029). Perimodiolar electrodes affected the caloric test results greater than straight electrodes (p = 0.041). There were no significant associations among these tests' results.Conclusions: Minimally invasive surgery in children using a round window approach or an extended round window approach with straight electrodes is desirable to preserve vestibular function after CI.

Objectives: Vibrant Soundbridge (VSB) was developed for treatment of hearing loss, but clinical outcomes vary and prognostic factors predicting the success of the treatment remain unknown. We examined clinical outcomes of VSB for conductive or mixed hearing loss, prognostic factors by analyzing prediction models, and cut-off values to predict the outcomes. Study design: Retrospective chart review. Setting: Tertiary care hospital. Patients: Thirty patients who underwent VSB surgery from January 2017 to December 2019 at our hospital. Intervention: Audiological tests were performed prior to and 3 months after surgery; patients completed questionnaires 3 months after surgery. Main outcome measures: We used a multiregression and the random forest algorithm for predictions. Mean absolute errors and coefficient of determinations were calculated to estimate prediction accuracies. Coefficient values in the multiregression model and the importance of features in the random forest model were calculated to clarify prognostic factors. Receiver operation characteristic curves were plotted. Results: All audiological outcomes improved after surgery. The random forest model (mean absolute error: 0.06) recorded more accuracy than the multiregression model (mean absolute error: 0.12). Speech discrimination score in a silent context in patients with hearing aids was the most influential factor (coefficient value: 0.51, featured value: 0.71). The candidate cut-off value was 36% (sensitivity: 89%, specificity: 75%). Conclusions: VSB is an effective treatment for conductive or mixed hearing loss. Machine learning demonstrated more precise predictions, and speech discrimination scores in a silent context in patients with hearing aids were the most important factor in predicting clinical outcomes.

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Patients with m.3243A>G mutation of mitochondrial DNA develop bilaterally symmetric sensorineural hearing loss. However, it is unclear how fast their hearing loss progresses over time, and whether they experience rapid progression of hearing loss. In the present study, we conducted a long-term hearing evaluation in patients with MELAS or MIDD who harbored the m.3243A>G mutation of mitochondrial DNA. A retrospective chart review was performed on 15 patients with this mutation who underwent pure-tone audiometry at least once a year for more than two years. The mean follow-up period was 12.8 years. The mean progression rate of hearing loss was 5.5 dB per year. Hearing loss progressed rapidly to be profoundly deaf in seven patients during the observation period. Heteroplasmy and age-corrected heteroplasmy levels correlated with the age of onset of hearing loss. These results indicate that patients with m.3243A>G mutation have a gradual progression of hearing loss in the early stages and rapid decline in hearing to be profoundly deaf in approximately half of the patients. Although it is possible to predict the age of onset of hearing loss from heteroplasmy and age-corrected heteroplasmy levels, it is difficult to predict whether and when the rapid hearing loss will occur.