Clinical characteristics of persistent postural-perceptual dizziness and its visual subtype in Korean patients: A multicenter cross-sectional study.

OBJECTIVES: Persistent postural-perceptual dizziness (PPPD) is a chronic functional vestibular disorder for which the Bárány Society has established diagnostic criteria. This nationwide multicenter study aims to investigate the clinical features of individuals with definite PPPD and clinical variant PPPD who do not fully meet the diagnostic criteria, with a particular focus on visual exaggeration. METHODS: Between September 2020 and September 2021, a total of 76 individuals with definite PPPD and 109 individuals with clinical variant PPPD who did not meet all three exacerbating factors outlined in Criterion B were recruited from 18 medical centers in South Korea. The study gathered information on demographic factors, clinical manifestations, balance scales, and personality assessments. RESULTS: Comparative analysis between groups with definite PPPD and clinical variant with visual exacerbation revealed no significant differences in sociodemographic characteristics, clinical course, dizziness impact, and specific precipitants. Only disease duration was significantly longer in definite PPPD compared with variant with visual exacerbation. However, the variant without visual exacerbation displayed significantly reduced rates of panic disorder, diminished space-motion discomfort, lesser impact of dizziness, and decreased prevalence of depression when compared with the definitive PPPD. CONCLUSION: This is the first comprehensive nationwide study examining clinical features of both definite PPPD patients and its clinical variants, considering visual exacerbating factors. Differences in dizziness and personality traits emerged between definite PPPD and its potential variant without visual issues. Our results highlight the possibility of a distinct clinical variant of PPPD influenced by visual dependency.

Findings of Intravenous Gadolinium Inner Ear Magnetic Resonance Imaging in Patients With Acute Low-Tone Sensorineural Hearing Loss.

OBJECTIVES: Acute low-tone sensorineural hearing loss (ALHL) is thought to have a different etiology from that of idiopathic sudden sensorineural hearing loss. We hypothesized that endolymphatic hydrops (EH) in the inner ear organ contributes to ALHL, even in patients without vertigo. This study investigated the presence of EH in ALHL and compared the clinical characteristics of patients with or without EH. METHODS: We retrospectively reviewed 38 patients diagnosed with ALHL without vertigo from January 2017 to March 2022. EH was measured in all patients using inner ear magnetic resonance imaging (MRI). In addition, we selected patients who showed only mid- or high-frequency hearing loss and had available MRI data as a control group and compared the ALHL and control groups. RESULTS: After treatment, the pure-tone average at low frequencies significantly improved compared to the initial hearing (P<0.001). Hearing recovery was observed in 63.1% of patients; however, the recovery rate did not differ based on the treatment method. During the follow-up period, six patients (15.8%) progressed to Meniere's disease, and 18 (47.4%) experienced recurrence. In the ALHL group, the cochlear hydrops ratio on the affected side (0.34±0.09) was significantly higher than on the contralateral side (0.29±0.12) (P=0.005), and most patients showed hydrops in the apex area of the cochlea. Compared with the control group (0.25±0.15), the ALHL group showed a significantly higher cochlear hydrops ratio (P=0.043). The correlation analysis showed a tendency for hearing thresholds at low frequencies to increase as the hydrops ratio increased, albeit without statistical significance. CONCLUSION: The cochlear hydrops ratio, especially in the apex area on the affected side, was significantly higher in patients with ALHL, suggesting that EH in the cochlea contributes to the pathogenesis of ALHL.

Treatment Efficacy of Various Maneuvers for Lateral Canal Benign Paroxysmal Positional Vertigo With Apogeotropic Nystagmus: A Randomized Controlled Trial.

OBJECTIVES: The aim of this study was to determine the most effective treatment approach by comparing the impacts of various otolith reduction techniques in patients with apogeotropic lateral semicircular canal benign paroxysmal positional vertigo (LC-BPPV). METHODS: We performed a multicenter randomized prospective study from January to December 2015, involving 72 consecutive patients with apogeotropic LC-BPPV. The patients were divided into three treatment groups: therapeutic head-shaking (group A), the Gufoni-Appiani maneuver (group B), and the cupulolith repositioning maneuver (CuRM; group C). Each group underwent evaluation and treatment up to the fourth week. Treatment success was defined as the disappearance of positional vertigo and nystagmus. RESULTS: This study included 72 patients (49 male and 23 female), with a mean (±standard deviation) age of 55.4±13.5 years. The mean duration of vertigo experienced prior to treatment was 3.9±4.4 days. The mean latency and duration of nystagmus were 2.7±3.0 seconds and 47.9±15.8 seconds, respectively. The overall treatment frequency was 2.0±0.9. The number of treatments differed significantly among the three groups (P<0.05). After 4 weeks, the success rates for groups A, B, and C were 90.5%, 92.3%, and 100%, respectively. No significant difference was observed in the success rate across treatment. METHODS: and periods (P>0.05). However, CuRM was the only method with a 100% treatment success rate. CONCLUSION: While no clear difference was observed among the three treatments for LC-BPPV, CuRM was found to be superior to the other approaches in the long term.

Round-window delivery of lithium chloride regenerates cochlear synapses damaged by noise-induced excitotoxic trauma via inhibition of the NMDA receptor in the rat.

Noise exposure can destroy the synaptic connections between hair cells and auditory nerve fibers without damaging the hair cells, and this synaptic loss could contribute to difficult hearing in noisy environments. In this study, we investigated whether delivering lithium chloride to the round-window can regenerate synaptic loss of cochlea after acoustic overexposure. Our rat animal model of noise-induced cochlear synaptopathy caused about 50% loss of synapses in the cochlear basal region without damaging hair cells. We locally delivered a single treatment of poloxamer 407 (vehicle) containing lithium chloride (either 1 mM or 2 mM) to the round-window niche 24 hours after noise exposure. Controls included animals exposed to noise who received only the vehicle. Auditory brainstem responses were measured 3 days, 1 week, and 2 weeks post-exposure treatment, and cochleas were harvested 1 week and 2 weeks post-exposure treatment for histological analysis. As documented by confocal microscopy of immunostained ribbon synapses, local delivery of 2 mM lithium chloride produced synaptic regeneration coupled with corresponding functional recovery, as seen in the suprathreshold amplitude of auditory brainstem response wave 1. Western blot analyses revealed that 2 mM lithium chloride suppressed N-methyl-D-aspartate (NMDA) receptor expression 7 days after noise-exposure. Thus, round-window delivery of lithium chloride using poloxamer 407 reduces cochlear synaptic loss after acoustic overexposure by inhibiting NMDA receptor activity in rat model.

Results of Active Middle Ear Implantation in Patients With Mixed Hearing Loss After Middle Ear Surgery: A Prospective Multicenter Study (the ROMEO Study).

OBJECTIVES: This study was conducted to evaluate the user satisfaction, efficacy, and safety of round window (RW) vibroplasty using the Vibrant Soundbridge (VSB) in patients with persistent mixed hearing loss after mastoidectomy. METHODS: The study included 27 patients (mean age, 58.7 years; age range, 28-76 years; 11 men and 16 women) with mixed hearing loss after mastoidectomy from 15 tertiary referral centers in Korea. The VSB was implanted at the RW. The Korean translation of the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire and the Korean version of the International Outcome Inventory for Hearing Aids (K-IOI-HA) questionnaire were used to evaluate user satisfaction as the primary outcome. The secondary outcome measures were audiological test results and complication rates. RESULTS: The mean scores for ease of communication (61.3% to 29.7% to 30.2%), reverberation (62.1% to 43.1% to 37.4%), and background noise (63.3% to 37.7% to 34.3%) subscales of the APHAB questionnaire significantly decreased after VSB surgery. The mean K-IOI-HA scores at 3 and 6 months after surgery were significantly higher than the mean preoperative score (18.6 to 27.2 to 28.1). The postoperative VSB-aided thresholds were significantly lower than the preoperative unaided and hearing aid (HA)-aided thresholds. There was no significant difference between preoperative unaided, preoperative HA-aided, and postoperative VSB-aided maximum phonetically balanced word-recognition scores. None of the 27 patients experienced a change in postoperative bone conduction pure tone average. One patient developed temporary facial palsy and two developed surgical wound infections. CONCLUSION: RW vibroplasty resulted in improved satisfaction and audiological test results in patients with mixed hearing loss after mastoidectomy, and the complication rate was tolerable.

Validation of inner ear MRI in patients with Ménière's disease by comparing endolymphatic hydrops from histopathologic specimens.

Intravenous gadolinium-enhanced inner-ear magnetic resonance imaging (IV-Gd inner-ear MRI) has been used to visualize endolymphatic hydrops (EH) in clinical diagnosis of Ménière's disease (MD). However, lack of histological validation has led to several concerns regarding how best to interpret the resulting images. Here, we compared hydropic changes in temporal bone specimens with the results of IV-Gd inner-ear MRI in patients with MD. Histopathologic images of temporal bones from 37 patients with MD and 10 healthy controls were collected from the National Temporal Bone Bank of the Massachusetts Eye and Ear Infirmary in the United States. The EH ratios in the vestibule and cochlea were calculated from temporal bones using the methods used for IV-Gd inner-ear MRI, and the degree to which the saccular and utricular hydrops contributed to vestibular hydrops was measured. The presence of hydropic change in each semicircular canal was assessed using temporal bone images and compared with IV-Gd inner-ear MRI scans of 74 patients with MD. Based on human temporal bone imagery, the EH ratios in the cochlea and the vestibule on the affected side were 0.314 and 0.757, respectively. In the healthy control group, the ratio was 0.064 for the cochlea and 0.289 for the vestibule; these values were significantly different from those for the affected side of MD patients. The values for the affected ear were similar to the ratios from the IV-Gd inner-ear MRI scans in MD patients. In the vestibule, saccular hydrops were more common than utricular hydrops. The average EH ratios in the saccule and utricle were 0.513 and 0.242, respectively. No significant hydropic change from each of three semicircular canals was evident in temporal bone histopathology. However, herniation of otolithic organs (saccule or utricle) into the lateral semicircular canal was found in 44.4% of the patients, with saccular herniation (24.8%) more common than utricular herniation (16.7%). Although IV-Gd inner-ear MRI might not reflect fully the results of actual histopathology due to the limited resolution of MRI and image-processing techniques, the measured EH ratios from temporal bone specimens and IV-Gd inner-ear MRI scans were similar. Hydropic change in the three semicircular canals was not significant at either the ampullated or nonampullated end. Canal invasion of vestibular hydrops seen on MRI also appeared in temporal bone histopathology, and saccular invasion was dominant.

A Fully Automated Analytic System for Measuring Endolymphatic Hydrops Ratios in Patients With Ménière Disease via Magnetic Resonance Imaging: Deep Learning Model Development Study.

BACKGROUND: Recently, the analysis of endolymphatic hydropses (EHs) via inner ear magnetic resonance imaging (MRI) for patients with Ménière disease has been attempted in various studies. In addition, artificial intelligence has rapidly been incorporated into the medical field. In our previous studies, an automated algorithm for EH analysis was developed by using a convolutional neural network. However, several limitations existed, and further studies were conducted to compensate for these limitations. OBJECTIVE: The aim of this study is to develop a fully automated analytic system for measuring EH ratios that enhances EH analysis accuracy and clinical usability when studying Ménière disease via MRI. METHODS: We proposed the 3into3Inception and 3intoUNet networks. Their network architectures were based on those of the Inception-v3 and U-Net networks, respectively. The developed networks were trained for inner ear segmentation by using the magnetic resonance images of 124 people and were embedded in a new, automated EH analysis system-inner-ear hydrops estimation via artificial intelligence (INHEARIT)-version 2 (INHEARIT-v2). After fivefold cross-validation, an additional test was performed by using 60 new, unseen magnetic resonance images to evaluate the performance of our system. The INHEARIT-v2 system has a new function that automatically selects representative images from a full MRI stack. RESULTS: The average segmentation performance of the fivefold cross-validation was measured via the intersection of union method, resulting in performance values of 0.743 (SD 0.030) for the 3into3Inception network and 0.811 (SD 0.032) for the 3intoUNet network. The representative magnetic resonance slices (ie, from a data set of unseen magnetic resonance images) that were automatically selected by the INHEARIT-v2 system only differed from a maximum of 2 expert-selected slices. After comparing the ratios calculated by experienced physicians and those calculated by the INHEARIT-v2 system, we found that the average intraclass correlation coefficient for all cases was 0.941; the average intraclass correlation coefficient of the vestibules was 0.968, and that of the cochleae was 0.914. The time required for the fully automated system to accurately analyze EH ratios based on a patient's MRI stack was approximately 3.5 seconds. CONCLUSIONS: In this study, a fully automated full-stack magnetic resonance analysis system for measuring EH ratios was developed (named INHEARIT-v2), and the results showed that there was a high correlation between the expert-calculated EH ratio values and those calculated by the INHEARIT-v2 system. The system is an upgraded version of the INHEARIT system; it has higher segmentation performance and automatically selects representative images from an MRI stack. The new model can help clinicians by providing objective analysis results and reducing the workload for interpreting magnetic resonance images.

Effect of Virtual Reality on Stress Reduction and Change of Physiological Parameters Including Heart Rate Variability in People With High Stress: An Open Randomized Crossover Trial.

Introduction: Although, attempts to apply virtual reality (VR) in mental healthcare are rapidly increasing, it is still unclear whether VR relaxation can reduce stress more than conventional biofeedback. Methods: Participants consisted of 83 healthy adult volunteers with high stress, which was defined as a score of 20 or more on the Perceived Stress Scale-10 (PSS-10). This study used an open, randomized, crossover design with baseline, stress, and relaxation phases. During the stress phase, participants experienced an intentionally generated shaking VR and serial-7 subtraction. For the relaxation phase, participants underwent a randomly assigned relaxation session on day 1 among VR relaxation and biofeedack, and the other type of relaxation session was applied on day 2. We compared the State-Trait Anxiety Inventory-X1 (STAI-X1), STAI-X2, the Numeric Rating Scale (NRS), and physiological parameters including heart rate variability (HRV) indexes in the stress and relaxation phases. Results: A total of 74 participants were included in the analyses. The median age of participants was 39 years, STAI-X1 was 47.27 (SD = 9.92), and NRS was 55.51 (SD = 24.48) at baseline. VR and biofeedback significantly decreased STAI-X1 and NRS from the stress phase to the relaxation phase, while the difference of effect between VR and biofeedback was not significant. However, there was a significant difference in electromyography, LF/HF ratio, LF total, and NN50 between VR relaxation and biofeedback. Conclusion: VR relaxation was effective in reducing subjectively reported stress in individuals with high stress.

Clinical predictors of cybersickness in virtual reality (VR) among highly stressed people.

The use of virtual reality (VR) in the treatment of psychiatric disorders is increasing, and cybersickness has emerged as an important obstacle to overcome. However, the clinical factors affecting cybersickness are still not well understood. In this study, we investigated clinical predictors and adaptation effect of cybersickness during VR application in highly stressed people. Eighty-three healthy adult participants with high stress level were recruited. At baseline, we conducted psychiatric, ophthalmologic, and otologic evaluations and extracted physiological parameters. We divided the participants into two groups according to the order of exposure to VR videos with different degrees of shaking and repetitively administered the Simulator Sickness Questionnaire (SSQ) and the Fast Motion sickness Scale (FMS). There was no significant difference in changes in the SSQ or the FMS between groups. The 40-59 years age group showed a greater increase in FMS compared to the 19-39 years age group. Smoking was negatively associated with cybersickness, and a high Positive Affect and Negative Affect Schedule score was positively associated with cybersickness. In conclusion, changing the intensity of shaking in VR did not affect cybersickness. While smoking was a protective factor, more expression of affect was a risk factor for cybersickness.

Significance of Pseudo-Conductive Hearing Loss and Positional Nystagmus for Perilymphatic Fistula: Are They Related to Third-Window Effects?

OBJECTIVES: Patients' clinical presentation is critical for identifying suspected perilymphatic fistula (PLF). The involvement of third-window lesions in the pathomechanism of PLF has been hypothesized. This study investigated the clinical features of PLF and the relationship of the third-window effect with PLF. METHODS: Sixty patients underwent surgical exploration for suspected PLF and the oval and round windows were reinforced. Clinical features including demographics, pure-tone audiometry (PTA), and videonystagmography were evaluated preoperatively and 1 month postoperatively. Surgical outcomes were analyzed according to the improvement of hearing and vestibular symptoms and signs. The conductive components of PTA (air-bone gap [ABG]) were measured, and the relationship between ABG closure after surgery and hearing improvement was analyzed. In addition, postoperative subjective dizziness was assessed by clinical interviews. Changes in positional nystagmus were analyzed according to ABG closure and hearing improvement. RESULTS: ABG at lower frequencies (LFABG; 250 Hz, 500 Hz, 1,000 Hz) was present in 27 patients (45%). Postoperatively, PTA significantly improved after surgical repair. Among the patients with preoperative LFABG (n=27), 15 (55.5%) showed postoperative ABG closure and significant improvement in PTA at all frequencies compared with the patients without ABG closure (P=0.012). Subjective dizziness improved in 57 patients (93.3%). Positional nystagmus was found in 45 of 49 patients. Multiple canal involvement was more common than single canal involvement (67% vs. 33%). The horizontal semicircular canal was most commonly involved, followed by the posterior and anterior canals. Postoperatively, positional nystagmus disappeared, or the number of involved canals decreased in 22 of 34 patients (64.7%). CONCLUSION: Pseudo-conductive hearing loss at lower frequencies and positional nystagmus originating from multiple semicircular canals were common findings in PLF. Surgical reinforcement of the oval and round windows improved the hearing threshold accompanied by closure of ABG. A third-window lesion might explain these clinical features of PLF.

Factors Contributing to the Severity and Laterality of Pisa Syndrome in Parkinson's Disease.

Objective: Pisa syndrome (PS) is a disabling postural deformity in Parkinson's disease (PD). We aimed to elucidate clinical factors determining the severity and laterality of PS in PD. Methods: In 54 PD patients with PS, we measured the clinical factors that are previously known to contribute to the occurrence of PS as follows: asymmetry of motor symptoms for the evaluation of asymmetric basal ganglia dysfunction, the degree and direction of subjective visual vertical (SVV) tilt for the misperception of body verticality, the canal paresis for unilateral peripheral vestibulopathy, and the tonic electromyographic (EMG) hyperactivity of paraspinal muscles for dystonia. Multivariable linear and logistic regression analyses were conducted to identify the clinical factors associated with the degree of truncal tilt, for the quantification of the severity of PS, and PS tilting to the less affected side, respectively. Results: The multivariable linear regression analyses revealed that the larger degree of SVV tilt (ß = 0.29, SE = 0.10, p = 0.005), right-sided SVV tilt (ß = 2.32, SE = 0.82, p = 0.007), and higher Hoehn and Yahr (HY) stage (ß = 4.01, SE = 1.29, p = 0.003) significantly increased the severity of PS. In the multivariable logistic regression analyses, greater asymmetry of motor symptoms [odds ratio (OR) = 2.01, 95% CI = 1.34-3.49] was significantly associated with PS tilting to the less affected side, while right-sided SVV tilt (OR = 0.02, 95% CI = 0.001-0.21), unilateral canal paresis (OR = 0.06, 95% CI = 0.003-0.79), and higher HY stage (OR = 0.04, 95% CI = 0.002-0.46) were associated with PS tilting to the more affected side. Conclusion: Misperception of verticality, asymmetric basal ganglia dysfunction, unilateral peripheral vestibulopathy, and motor disability are the clinical factors associated with the severity and laterality of PS in patients with PD.

Automated measurement of hydrops ratio from MRI in patients with Ménière's disease using CNN-based segmentation.

Ménière's Disease (MD) is difficult to diagnose and evaluate objectively over the course of treatment. Recently, several studies have reported MD diagnoses by MRI-based endolymphatic hydrops (EH) analysis. However, this method is time-consuming and complicated. Therefore, a fast, objective, and accurate evaluation tool is necessary. The purpose of this study was to develop an algorithm that can accurately analyze EH on intravenous (IV) gadolinium (Gd)-enhanced inner-ear MRI using artificial intelligence (AI) with deep learning. In this study, we developed a convolutional neural network (CNN)-based deep-learning model named INHEARIT (INner ear Hydrops Estimation via ARtificial InTelligence) for the automatic segmentation of the cochlea and vestibule, and calculation of the EH ratio in the segmented region. Measurement of the EH ratio was performed manually by a neuro-otologist and neuro-radiologist and by estimation with the INHEARIT model and were highly consistent (intraclass correlation coefficient = 0.971). This is the first study to demonstrate that automated EH ratio measurements are possible, which is important in the current clinical context where the usefulness of IV-Gd inner-ear MRI for MD diagnosis is increasing.

Evaluating Reasons for Revision Surgery and Device Failure Rates in Patients Who Underwent Cochlear Implantation Surgery.

Importance: Understanding the reasons for cochlear implant (CI) revision surgery and device failure rates is important for clinicians when counseling patients who are considering CI. Objectives: To analyze the revision surgery rate, reasons for revision surgery, and device failure and survival rates of different device models in recipients of CIs. Design, Setting, and Participants: In this cohort study, cochlear implants at Samsung Medical Center, a tertiary referral center, were retrospectively reviewed. Patients who underwent CI surgery from October 2001 to March 2019 were included. In the device survival analysis, the first revision surgery was considered the primary event, and the end point of observation was June 1, 2019. Interventions: Therapeutic and rehabilitative CI surgery. Main Outcomes and Measures: The revision surgery rate, reasons for revision surgery, and the failure and survival rates of different device models were analyzed. The Kaplan-Meier method and the log-rank test were used to present both the device survival and cumulative survival curves with rates. Results: In this study, 43 of 925 patients with CIs (4.6%) underwent a revision surgery. Device failure was the most common reason (28 of 43 patients [65%]). Flap-associated problems and migration of the inner device were the next most important reasons (4 of 43 [9.3%] each). Overall, the 10-year cumulative survival rate of CI surgery was 94.4%, and the device survival rate was 96.0%. Thirteen different CI devices from 4 different manufacturers were implanted, and no meaningful differences in device failure were found among CI manufacturers or devices (hazard ratios for cumulative survival: Cochlear, 1.67 [95% CI, 0.72-3.88]; Advanced Bionics, 1.67 [95% CI, 0.61-4.53]; Med-El, reference; hazard ratios for device survival: Cochlear, 1.65 [95% CI, 0.55-4.99]; Advanced Bionics, 1.93 [95% CI, 0.56-6.74]; Med-El, reference). Several recalls were issued by manufacturers during the study period, and after excluding the recalled devices, the device survival rates for 5, 10, and 15 years were 98.2%, 97.7%, and 94.9%, respectively. Conclusions and Relevance: Generally, implanted devices remain safe and stable for a long time, and no significant differences in survival rates were found between device types or manufacturers. Device failure was the main reason for CI revision, followed by flap-associated problems and migration of the inner device.

Auditory Characteristics in Patients With Mucopolysaccharidosis.

OBJECTIVE: The purpose of the study was to evaluate audiologic findings according to mucopolysaccharidosis (MPS) subtypes and to estimate hearing changes as the disease progressed, as well as the therapeutic effect of enzyme replacement therapy on the hearing apparatus. METHODS: A total of 124 patients who were diagnosed with MPS between September 1994 and December 2016 were retrospectively analyzed. Play audiometry or pure-tone audiometry was performed for hearing assessment, and auditory brainstem response was conducted in patients with poor compliance. RESULTS: In total 124 patients were identified, ranging in age at diagnosis from 0 to 33 years. Fourteen of the patients had been diagnosed with type I, while 91 had type II, 2 had type III, 14 had type IV, and 3 had type VI. Mean bone conduction and air conduction for the better ear were 26.13±16.95 dB and 34.77 ±â€Š20.00 dB in all patients, and 34.20±7.64 dB and 40.70±9.67 dB in patients with MPS II. The average auditory brainstem response threshold was 68.96 ±21.93 dB nHL. The most common type of hearing loss was pure sensorineural hearing loss in all subtypes, and the degree of hearing loss was variable mostly within the mild to severe range. The increase in the hearing threshold was also significantly correlated with the disease duration. However, the change in hearing level was not correlated with the duration of enzyme replacement therapy. CONCLUSIONS: Hearing impairment in MPS patients is common and is aggravated as the disease progresses. Thus, adequate intervention and hearing rehabilitation might play an important role in managing hearing disabilities in MPS patients.

Benefit and predictive factors for speech perception outcomes in pediatric bilateral cochlear implant recipients / Benefícios e fatores preditivos para os resultados de percepção da fala em pacientes pediátricos com implantes cocleares bilaterais

Abstract Introduction: Despite recent advancement in the prediction of cochlear implant outcome, the benefit of bilateral procedures compared to bimodal stimulation and how we predict speech perception outcomes of sequential bilateral cochlear implant based on bimodal auditory performance in children remain unclear. Objectives: This investigation was performed: (1) to determine the benefit of sequential bilateral cochlear implant and (2) to identify the associated factors for the outcome of sequential bilateral cochlear implant. Methods: Observational and retrospective study. We retrospectively analyzed 29 patients with sequential cochlear implant following bimodal-fitting condition. Audiological evaluations were performed; the categories of auditory performance scores, speech perception with monosyllable and disyllables words, and the Korean version of Ling. Audiological evaluations were performed before sequential cochlear implant with the bimodal fitting condition (CI1 + HA) and one year after the sequential cochlear implant with bilateral cochlear implant condition (CI1 + CI2). The good performance group (GP) was defined as follows; 90% or higher in monosyllable and bisyllable tests with auditory-only condition or 20% or higher improvement of the scores with CI1 + CI2. Age at first implantation, inter-implant interval, categories of auditory performance score, and various comorbidities were analyzed by logistic regression analysis. Results: Compared to the CI1 + HA, CI1 + CI2 provided significant benefit in categories of auditory performance, speech perception, and Korean version of Ling results. Preoperative categories of auditory performance scores were the only associated factor for being GP (odds ratio = 4.38, 95% confidence interval - 95% = 1.07-17.93, p = 0.04). Conclusions: The children with limited language development in bimodal condition should be considered as the sequential bilateral cochlear implant and preoperative categories of auditory performance score could be used as the predictor in speech perception after sequential cochlear implant.

Resumo Introdução: Apesar dos avanços recentes na predição do resultado do implante coclear, ainda não está claro o benefício do procedimento bilateral em comparação com a estimulação bimodal e como podemos prever resultados de percepção da fala com implante coclear bilateral sequencial com base no desempenho auditivo bimodal em crianças. Objetivos: Este estudo foi realizado para: 1) Determinar o benefício do implante coclear bilateral sequencial e 2) Identificar os fatores associados com o resultado do implante bilateral sequencial. Método: Estudo observacional e retrospectivo. Analisamos retrospectivamente 29 pacientes com implantes sequenciais após a adaptação bimodal. Avaliações audiológicas foram realizadas; os escores das categorias de desempenho auditivo, a percepção da fala com palavras monossílabas e dissílabas e a versão coreana de Ling. As avaliações audiológicas foram realizadas antes do implante sequencial com o ajuste bimodal (IC1 + AH) e um ano após o implante coclear sequencial com implante bilateral (IC1 + IC2). O grupo com bom desempenho (BD) foi definido da seguinte forma: 90% ou mais em testes com monossílabos e dissílabos com condição apenas auditiva ou melhoria de 20% ou mais dos escores com IC1 + IC2. A idade no primeiro implante, o intervalo interimplante, o escore categorias de desempenho auditivo e as diversas comorbidades foram analisadas através de análise de regressão logística. Resultados: Em comparação com o IC1 + AA, IC1 + IC2 demonstraram benefícios significativos nos escores categorias de desempenho auditivo, percepção da fala e a versão coreana de Ling. Os escores de categorias de desempenho auditivo pré-operatórios foram o único fator associado para ser do grupo BD (odds ratio - OR = 4,38, intervalo de confiança de 95% - IC 95% = 1,07-17,93, p = 0,04). Conclusões: As crianças com desenvolvimento limitado de linguagem em condição bimodal devem ser consideradas, pois o implante coclear bilateral sequencial e o escore pré-operatório das categorias de desempenho auditivo poderiam ser usados como preditores na percepção da fala após implante sequencial.

Predicting cochlear dead regions in patients with hearing loss through a machine learning-based approach: A preliminary study.

We propose a machine learning (ML)-based model for predicting cochlear dead regions (DRs) in patients with hearing loss of various etiologies. Five hundred and fifty-five ears from 380 patients (3,770 test samples) diagnosed with sensorineural hearing loss (SNHL) were analyzed. A threshold-equalizing noise (TEN) test was applied to detect the presence of DRs. Data were collected on sex, age, side of the affected ear, hearing loss etiology, word recognition scores (WRS), and pure-tone thresholds at each frequency. According to the cause of hearing loss as diagnosed by the physician, we categorized the patients into six groups: 1) SNHL with unknown etiology; 2) sudden sensorineural hearing loss (SSNHL); 3) vestibular schwannoma (VS); 4) Meniere's disease (MD); 5) noise-induced hearing loss (NIHL); or 6) presbycusis or age-related hearing loss (ARHL). To develop a predictive model, we performed recursive partitioning and regression for classification, logistic regression, and random forest. The overall prevalence of one or more DRs in test ears was 20.36% (113 ears). Among the 3,770 test samples, the overall frequency-specific prevalence of DR was 6.7%. WRS, pure-tone thresholds at each frequency, disease type (VS or MD), and frequency information were useful for predicting DRs. Sex and age were not associated with detecting DRs. Based on these results, we suggest possible predictive factors for determining the presence of DRs. To improve the predictive power of the model, a more flexible model or more clinical features, such as the duration of hearing loss or risk factors for developing DRs, may be needed.

Surgical Outcomes on Hearing and Vestibular Symptoms in Barotraumatic Perilymphatic Fistula.

OBJECTIVE: To analyze surgical results on hearing and vestibular symptoms in patients with barotraumatic perilymphatic fistula (PLF) according to diagnostic criteria. METHODS: A total of 39 patients (41 ears) who underwent surgery on suspicion of barotraumatic PLF from January 2005 to December 2017 were included. Pure tone audiometry and videonystagmography (VNG) recording for spontaneous nystagmus and positional tests were performed preoperatively and postoperatively at 1 week and 1 month. Surgical outcomes were analyzed based on hearing results, subjective dizziness, and change of nystagmus. RESULTS: Preoperative hearing level was 75.5 ±â€Š28.7 dB for definite PLF and 88.5 ±â€Š22.8 dB for probable PLF, and levels were not significantly different between groups. Preoperatively, subjective dizziness was present in 18 (94.7%) and 19 (95%) in each group. Among 39 patients, 24 had VNG recordings. Positional nystagmus was recorded in 87.5% (7/8) and 87.5% (14/16) of the definite and probable PLF groups, respectively. Postoperatively, hearing was improved in 65% (13/20 ears) of definite PLF and 61.9% (13/21 ears) of probable PLF. There was no significant difference between the two groups. Hearing gain was significantly correlated with the time interval between symptom onset and surgical timing. Subjective dizziness was improved immediately after surgical repair in 97.4% (17/18) of definite PLF patients and 100% (19/19) of probable PLF patients. Even though dizziness was improved in most patients, some had persistent positional nystagmus and recurrent dizziness. CONCLUSIONS: Surgical sealing of both windows in suspected barotraumatic PLF is an effective treatment to improve hearing and subjective dizziness.

Benefit and predictive factors for speech perception outcomes in pediatric bilateral cochlear implant recipients.

INTRODUCTION: Despite recent advancement in the prediction of cochlear implant outcome, the benefit of bilateral procedures compared to bimodal stimulation and how we predict speech perception outcomes of sequential bilateral cochlear implant based on bimodal auditory performance in children remain unclear. OBJECTIVES: This investigation was performed: (1) to determine the benefit of sequential bilateral cochlear implant and (2) to identify the associated factors for the outcome of sequential bilateral cochlear implant. METHODS: Observational and retrospective study. We retrospectively analyzed 29 patients with sequential cochlear implant following bimodal-fitting condition. Audiological evaluations were performed; the categories of auditory performance scores, speech perception with monosyllable and disyllables words, and the Korean version of Ling. Audiological evaluations were performed before sequential cochlear implant with the bimodal fitting condition (CI1+HA) and one year after the sequential cochlear implant with bilateral cochlear implant condition (CI1+CI2). The good performance group (GP) was defined as follows; 90% or higher in monosyllable and bisyllable tests with auditory-only condition or 20% or higher improvement of the scores with CI1+CI2. Age at first implantation, inter-implant interval, categories of auditory performance score, and various comorbidities were analyzed by logistic regression analysis. RESULTS: Compared to the CI1+HA, CI1+CI2 provided significant benefit in categories of auditory performance, speech perception, and Korean version of Ling results. Preoperative categories of auditory performance scores were the only associated factor for being GP (odds ratio=4.38, 95% confidence interval - 95%=1.07-17.93, p=0.04). CONCLUSIONS: The children with limited language development in bimodal condition should be considered as the sequential bilateral cochlear implant and preoperative categories of auditory performance score could be used as the predictor in speech perception after sequential cochlear implant.

Usefulness of Intravenous Gadolinium Inner Ear MR Imaging in Diagnosis of Ménière's Disease.

This study aimed to investigate the usefulness of the intravenous gadolinium enhanced inner ear magnetic resonance imaging (IV-Gd inner ear MRI) in diagnosing Ménière's disease(MD) and find a correlation between the degree of endolymphatic hydrops(EH) and the audiovestibular tests. Total 29 patients diagnosed with unilateral definite MD were enrolled. All patients underwent IV-Gd inner ear MRI and auditory and vestibular function tests such as pure tone audiometry (PTA), electrocochleography (ECoG), cervical vestibular evoked myogenic potential (cVEMP) and caloric test. The hydrops ratio in the cochlea and vestibule were significantly higher in the affected side than the unaffected side (p < 0.001). Average pure-tone thresholds for 0.5, 1 k, 2 k, and 4 k Hz correlated significantly with cochlear and vestibular hydrops (p < 0.01) in the affected side. When comparing the SP/AP ratio of ECoG with hydrops ratio in the vestibule, the affected and unaffected ears showed a significant difference (p < 0.05). Similarly, the results of the caloric test also showed a significant correlation (p < 0.05) with relative vestibular hydrops. However, the cVEMP response was not related to the hydrops ratio in the cochlea or vestibule. This study presents pertinent data with appropriate correlations with auditory vestibular functional testing which demonstrates the usefulness of IV-Gd inner ear MRI as a diagnostic method for visualizing the endolymphatic hydrops in MD.

Evaluation of Cerebral White Matter in Prelingually Deaf Children Using Diffusion Tensor Imaging.

This study compared white matter development in prelingually deaf and normal-hearing children using a tract-based spatial statistics (TBSS) method. Diffusion tensor imaging (DTI) was performed in 21 prelingually deaf (DEAF group) and 20 normal-hearing (HEAR group) subjects aged from 1.7 to 7.7 years. Using TBSS, we evaluated the regions of significant difference in fractional anisotropy (FA) between the groups. Correlations between FA values and age in each group were also analyzed using voxel-wise correlation analyses on the TBSS skeleton. Lower FA values of the white matter tract of Heschl's gyrus, the inferior frontooccipital fasciculus, the uncinate fasciculus, the superior longitudinal fasciculus, and the forceps major were evident in the DEAF group compared with those in the HEAR group below 4 years of age, while the difference was not significant in older subjects. We also found that age-related development of the white matter tracts may continue until 8 years of age in deaf children. These results imply that development of the cerebral white matter tracts is delayed in prelingually deaf children.