Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Waardenburg syndrome (WS) is caused by autosomal dominant mutations. Since the coexistence of epilepsy and WS type I is rare, the detailed clinical features and treatment of epilepsy, including surgery, have not been fully reported for these patients. We report the first case of an individual with WS type I, who underwent corpus callosotomy (CC) for drug-resistant epilepsy and obtained good seizure outcomes. CASE DESCRIPTION: A boy was diagnosed as having WS type I and developmental delay based on characteristic symptoms and a family history of hearing loss. He underwent cochlear implantation at 18 months of age. At 4 years of age, he developed epileptic seizures with a semiology of drop attack. Electroencephalography (EEG) showed bilateral synchronous high-amplitude spikes and wave bursts, dominant in the right hemisphere. Based on the multimodality examinations, we considered that ictal discharges propagated from the entire right hemisphere to the left, resulting in synchronous discharge and a clinical drop attack; therefore, CC was indicated. At 9 years of age, he underwent a front 2/3(rd) CC. At 1 year, the patient became seizure free, and interictal EEG showed less frequent and lower amplitude spike and wave bursts than before. CONCLUSION: When patients with WS Type I and cognitive impairment show drug-resistant epilepsy, clinicians should consider a presurgical evaluation.

DOI： 10.25259/SNI_228_2021

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES/HYPOTHESIS: The extreme rarity of temporal bone squamous cell carcinoma (TB-SCC) has delayed the accumulation of high-quality clinical evidence. For the purposes of retrospective meta-analysis in the future, a large dataset with information from various institutions would be ideal. Our objective here was to retrospectively review cases of TB-SCC encountered at a single tertiary referral center and explore survival outcomes and prognostic factors. STUDY DESIGN: Retrospective chart review. METHODS: The medical records of all TB-SCC cases were retrospectively reviewed. The resulting dataset contained 71 cases of primary cancer eligible for initial definitive (curative) treatment. RESULTS: T4 status was associated with lower disease-specific 5-year survival than T1 to T3 staging (T1: 100%, T2: 92%, T3: 86%, T4: 51%). Survival was significantly higher in operable than in inoperable cases, even when restricted to advanced (T3/T4) cancers. The tumor extension to the middle ear cavity was observed in 13/17 of T3 cases, but it was not associated with poor survival. In addition, among operable cases, negative surgical margins were associated with significantly higher survival than positive margins. CONCLUSIONS: Definitive treatments can offer disease-specific 5-year survival of over 85% in T1 to T3 cases of TB-SCC. The tumor extension to the middle ear cavity is not associated with poor survival. T4 status, inoperability, nodal invasion, and positive surgical margin are identified as a predictor of poor prognosis. Still, the matter of how to deal with unresectable tumors remains an outstanding issue in the treatment of TB-SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E583-E589, 2021.

DOI： 10.1002/lary.28653

Language：English   Publishing type：Research paper (scientific journal)  

lthough negative pressure wound therapy (NPWT) is widely used, its application to the head and neck region remains challenging due to anatomical complexities. This report presents the case of a female patient presenting with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes, uncontrolled diabetes and severe bilateral sensorineural hearing loss. The patient had undergone cochlear implant surgery and five months later the wound was infected with methicillin-resistant Staphylococcus aureus (MRSA). NPWT was started shortly after removing the internal receiver and was stopped 11 days later. NPWT helped in controlling infection and led to a successful wound closure. In this case, NPWT was effective in treating infectious wounds around the auricle after cochlear implant surgery. Declaration of interest: The authors have no financial support for this article and no conflict of interest directly relevant to the content of this article.

DOI： 10.12968/jowc.2020.29.10.568

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The immune checkpoint inhibitor Nivolumab was approved for the treatment of platinum-refractory head and neck squamous cell carcinoma (SCC), expanding the treatment options for recurrent or advanced head and neck SCC. However, since temporal bone squamous cell carcinoma (TB-SCC) is very rare cancer, the effectiveness of Nivolumab remains unclear. We investigated the effects of Nivolumab for TB-SCC. METHOD: Chart information was collected for all patients who underwent the first administration of Nivolumab for recurrent or residual TB-SCC in our hospital between September 2017 and December 2019. Tumor staging followed the modified Pittsburgh classification. Changes in the tumor burden and survival outcome were examined. RESULTS: We examined 9 patients with recurrent or residual TB-SCC who started administration of Nivolumab. In these cases, recurrent or residual SCC was observed after chemotherapy and/or chemoradiotherapy including platinum. The duration of Nivolumab was 2-54 weeks (median 20.0 weeks). The evaluation of the therapeutic effect according to the RECIST method showed partial response in 1 case, stable disease in 2 cases, progressive disease in 4 cases, and size unevaluated in 2 case. Although the number of cases was small, comparing with 5 cases without Nivolumab, these cases showed longer overall survival (1-year OS 33.3% vs 20.0%). CONCLUSION: We used Nivolumab as palliative chemotherapy in 9 patients with recurrent/residual TB-SCC, and we were able to obtain a certain therapeutic effect on TB-SCC as well as other head and neck SCC.

DOI： 10.1016/j.anl.2020.03.011

Language：English   Publishing type：Research paper (scientific journal)  

External auditory canal squamous cell carcinoma (EACSCC) is an extremely rare and aggressive malignancy. Due to its rarity, the molecular and genetic characteristics of EACSCC have not yet been elucidated. To reveal the genetic alterations of EACSCC, we performed whole exome sequencing (WES) on 11 primary tumors, 1 relapsed tumor and 10 noncancerous tissues from 10 patients with EACSCC, including 1 with a rare case of synchronous bilateral EACSCC of both ears. WES of the primary tumor samples showed that the most frequently mutated gene is TP53 (63.6%). In addition, recurrent mutations in CDKN2A, NOTCH1, NOTCH2, FAT1 and FAT3 were detected in multiple samples. The mutational signature analysis of primary tumors indicated that the mutational processes associated with the activation of apolipoprotein B mRNA-editing enzyme catalytic polypeptide-like (APOBEC) deaminases are the most common in EACSCC, suggesting its similarity to SCC from other primary sites. Analysis of arm-level copy number alterations detected notable amplification of chromosomes 3q, 5p and 8q as well as deletion of 3p across multiple samples. Focal chromosomal aberrations included amplifications of 5p15.33 (ZDHHC11B) and 7p14.1 (TARP) as well as deletion of 9p21.3 (CDKN2A/B). The protein expression levels of ZDHHC11B and TARP in EACSCC tissues were validated by immunohistochemistry. Moreover, WES of the primary and relapsed tumors from a case of synchronous bilateral EACSCC showed the intrapatient genetic heterogeneity of EACSCC. In summary, this study provides the first evidence for genetic alterations of EACSCC. Our findings suggest that EACSCC mostly resembles other SCC.

DOI： 10.1111/cas.14515

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: To develop a registration procedure to achieve a higher degree of registration accuracy in image-guided otological surgery, paying particular attention to the registration centroid. METHODS: A head phantom was used to measure the target registration error (TRE) at measurement points at various depth from the surface of the head. The surface-matching registration was performed using a commercially available surgical navigation system. We registered the phantom using only one ear of either side (right 100% - left 0%, or right 0% - left 100%) or using both ears with variable ratios (right 75% - left 25%, right 50% - left 50%, or right 25% - left 75%). RESULTS: The overall TRE was the smallest when registration was performed equally on both sides. However, the TRE at 20-50 mm from the surface was the smallest when the fiducial points for the registration were collected asymmetrically at a ratio of 75:25 and weighed heavier on the operating side, and this difference was statistically significant. CONCLUSION: The accuracy of image-guided surgery can be improved by carefully planning the registration procedure without changing the procedure itself. Accurate image-guided surgery at the middle and inner ear was achieved using 75% of the point cloud for the operating side and 25% of that for the opposite side for the registration.

DOI： 10.1016/j.anl.2020.01.007

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.heliyon.2020.e03200

Language：English   Publishing type：Research paper (scientific journal)  

Computed DWI (cDWI) is a mathematical technique that calculates arbitrary higher b value images from at least two different lower b values. In addition, the removal of high intensity noise with image processing on cDWI could improve cholesteatoma-background contrast-to-noise ratio (CNR). In the present study, noise reduction was performed by the cut-off values of apparent diffusion coefficient (ADC) less than 0 and 0.4 x 10(-3) s/mm(2). The cholesteatoma to non-cholesteatoma CNR was increased using a noise reduction algorithm for clinical setting.

DOI： 10.2463/mrms.tn.2018-0068

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Ultrahigh-resolution CT (U-HRCT) provides better spatial resolution than conventional multi-detector row CT (ConvCT) and could be expected to identify microstructures with its 0.25-mm collimation, 1792 channels and 160 detector rows, 0.4 x 0.5 mm focus size, and a 1024 matrix. The aim of the study was to evaluate key anatomic structures in temporal bone using U-HRCT comparing it to ConvCT. MATERIALS AND METHODS: A total of 30 patients (14 males and 16 females; age range, 8-82 years; median 49 years) underwent both U-HRCT and ConvCT. All CT images were obtained with 0.5 mm section thickness and a 512 x 512 matrix, and field of view of 80 mm. Transverse scans were acquired in a plane parallel to the orbitomeatal plane in the helical mode with 120 kV. Images of the 30 temporal bones of unaffected side were reviewed by two independent neuroradiologists who rated the visibility of key anatomic structures for both U-HRCT and ConvCT. The ratings between U-HRCT and ConvCT were compared using Wilcoxon matched-pairs signed rank test. The interobserver agreement on the rating of stapedius tendon was evaluated using weighted kappa statistics. RESULTS: Excellent interobserver agreement was shown for U-HRCT (kappa = 0.920), whereas good agreement was obtained for ConvCT (kappa = 0.733). According to both observers, stapedius tendon was more clearly visualized using U-HRCT than ConvCT (p < 0.0001). All other anatomic structures were well visualized using both CT scanners. CONCLUSION: The anatomy of temporal bone is more conspicuous on U-HRCT than on ConvCT because of its ultra-high-resolution detector. U-HRCT may provide beneficial information for determining surgical indication or procedures.

DOI： 10.1007/s00405-018-5101-6

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: To evaluate the effects of chronic kidney disease (CKD) and hemodialysis (HD) on the inner ear using the G width (the width between the bimodal peaks of the conductance (G) tympanogram at 2,000 Hz), which reflects the inner ear pressure and/or the existence of endolymphatic hydrops. MATERIALS AND METHODS: We selected five patients (10 ears) from the patients with CKD who were hospitalized for creation of arteriovenous fistula prior to initiation of HD (non-HD group), and we selected seven patients (14 ears) from the patients with CKD who were undergoing HD (the HD group). As a control group, we selected 80 healthy individuals (160 ears); these were mainly the medical staff of the hospital. We measured the G width of the control group and that of patients with CKD using multifrequency tympanometry. RESULTS: The mean G widths of the HD (measured just before an HD session), non-HD, and control groups were 210.7, 128.4, and 97.0 daPa, respectively. The G width of the HD group was significantly greater than that of the control and non-HD groups (p<0.01 and p<0.01, respectively; Tukey-Kramer test after one-way analysis of variance). The non-HD group also had a greater G width than the control, but it was not significant (p=0.20; Tukey-Kramer). No significant changes were observed in the G widths of the HD group, just before and after a single HD session (p=0.423; paired t-test). CONCLUSION: The greater G width observed in hemodialyzed CKD patients suggests either an increased inner ear pressure or the existence of endolymphatic hydrops in these patients, which is probably related to their otologic symptoms.

DOI： 10.5152/iao.2018.4736

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Voice-aligned compression (VAC) is a method used in Oticon's hearing aids to provide more comfortable hearing without sacrificing speech discrimination. The complex, non-linear compression curve for the VAC strategy is designed based on the frequency profile of certain spoken Western languages. We hypothesized that hearing aids could be further customized for Japanese-speaking users by modifying the compression curve using the frequency profile of spoken Japanese. METHODS: A double-blind randomized controlled crossover study was performed to determine whether or not Oticon's modified amplification strategy (VAC-J) provides subjectively preferable hearing aids for Japanese-speaking hearing aid users compared to the same company's original amplification strategy (VAC). The participants were randomized to two groups. The VAC-first group received a pair of hearing aids programmed using the VAC strategy and wore them for three weeks, and then received a pair of hearing aids programmed using VAC-J strategy and wore them for three weeks. The VAC-J-first group underwent the same study, but they received hearing aids in the reverse sequence. A Speech, Spatial and Qualities (SSQ) questionnaire was administered before beginning to use the hearing aids, at the end of using the first pair of hearing aids, and at the end of using the second pair of hearing aids. RESULTS: Twenty-five participants that met the inclusion/exclusion criteria from January 1 to October 31, 2016, were randomized to two groups. Twenty-two participants completed the study. There were no statistically significant differences in the increment of SSQ scores between the participants when using the VAC- or the VAC-J-programmed hearing aids. However, participants preferred the VAC-J strategy to the VAC strategy at the end of the study, and this difference was statistically significant. CONCLUSION: Japanese-speaking hearing aid users preferred using hearing aids that were fitted with the VAC-J strategy. Our results show that the VAC strategy can be adjusted to the frequency profile of different languages and that participants expressed their subjective preference more clearly than was reflected in the SSQ scores. A similar language-specific strategy may improve user's satisfaction while using hearing devices, and this concept may be extended to implantable hearing devices. CLINICAL RESEARCH REGISTRATION NUMBER: R000023191.

DOI： 10.1016/j.anl.2017.11.007

Language：English   Publishing type：Research paper (scientific journal)  

BJECTIVE: This study aimed to verify cutoff values for G width (the width of bimodal peaks for the waveform obtained when measuring conductance at 2000Hz) in Japanese individuals diagnosed with Meniere's disease (MD) using multifrequency tympanometry (MFT) and to determine the relationship between the G width and ability to hear low-pitched sounds using measurements over time. METHODS: The study included 51 patients with clinically diagnosed MD, who had not undergone endolymphatic sac surgery, but had no other known ear disease (57 ears in patients aged 22-80 years were affected, and 45 ears in patients aged 18-83 years were unaffected; mean age: 53.3+/-16.9 years). We also enlisted 80 healthy controls with no prior history of ear disease (160 ears, aged 22-76 years, mean age: 40.8+/-15.7 years). MFT was used to measure the bimodal peak width of the waveform obtained when measuring conductance at resonance frequency of 2000Hz. For patients who had G width measured several times over multiple outpatient visits, we used initial test data to analyze cutoff values. In nine cases with four or more measurements over time, we evaluated a possible correlation between G width and the sum of the hearing threshold for three low-pitched frequencies (125Hz, 250Hz, and 500Hz). We used Student's t-test to determine significance. RESULTS: The both ears in the MD patients had a G width wider than the distribution in the control group. There was a significant difference between G width in the control group and in affected ears with MD (p=0.00026) and there was also a significant difference between G width in the control group and in unaffected ears of MD patients (p=0.0056). The cutoff value set with a specificity of 95% was 200daPa, with a sensitivity of 35.1% and specificity of 95.6%. The cutoff value set with a sensitivity of 50% was 140daPa, with sensitivity of 50.9% and specificity of 78.8%. There was no significant difference between resonance frequency of ears in the control group and ears with MD (p=0.41). In nine cases with four or more measurements over time, a case showed a statistically significant positive correlation between the G width and hearing ability threshold for low-pitched sounds (125Hz, 250Hz, and 500Hz) (p=0.03), while an another case showed a tendency toward a positive correlation, which was not statistically significant (p=0.08). Further, there were cases that did not show significant differences in the present study, but might have shown a negative correlation if the number of measurements had been increased. CONCLUSION: Measurement of G width using MFT may have accuracy as the traditional endolymphatic hydrops test. MFT is non-invasive, causes little discomfort for patients, requires little time to perform, and can be performed by paramedics. MFT was shown to be useful in screening for MD and it is effective in diagnosing MD to measure the change over time of G width using MFT.

DOI： 10.1016/j.anl.2017.05.008

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1136/bcr-2017-222657

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1002/lary.25998

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s11548-016-1441-0

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.anl.2015.08.008

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s00405-014-3467-7

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1017/S0022215114002308

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (international conference proceedings)  

DOI： 10.1109/EMBC.2013.6610851

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：Japanese   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.brainres.2004.03.001

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1113/jphysiol.2002.018267

難聴児療育に関する、市民を対象にした市民公開講座、障害児教育に携わる教育者、障害児支援に関わる自治体福祉課職員を対象にした教育講演を行い、難聴児療育の知見を啓蒙する。

Audience：Infants,　Schoolchildren,　Junior students,　High school students

Type：Other