Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.67.440

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.67.409

CiNii Research

Publisher：学研メディカル秀潤社  

DOI： 10.15105/ce.0000001780

CiNii Research

Language：Japanese   Publisher：(一社)日本耳科学会  

細径の蝸牛軸巻き付き型の人工内耳電極は挿入時に蝸牛内で屈曲(tip fold-over)することがある.この問題は電極が曲がる向きと蝸牛が曲がる向きが一致しないミスアライメントという状態のときに起こりやすい.このため,執刀医は電極のカイル平面をなるべく蝸牛のコイル平面に一致させて電極を挿入する必要がある.われわれは,手術中に視認できるキヌタ骨を利用して蝸牛コイル平面を想定する方法を検討した.2017年から2021年の期間に,一人の執刀医が施行した人工内耳植込み術症例のうち内耳奇形症例を除外した65耳(右29耳,左36耳)について検討した.キヌタ骨平面と蝸牛コイル平面が為す角度をキヌタ-蝸牛角と定義し集計した.キヌタ-蝸牛角は平均では34.6°であった.左右および手術時年齢による統計的な有意差は見られなかった.本研究により,手術中に確認できるキヌタ骨平面を利用して蝸牛コイル平面を正確に想定することが可能とわかった.(著者抄録)

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.66.401

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.66.442

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.66.409

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.66.407

CiNii Research

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>We herein report a case series of 18 cochlear implant users who underwent magnet resonance imaging (MRI). The loci of imaging were head/neck (9 cases), thoracic vertebrae (2 cases), shoulder (1 case), liver (1 case), lower vertebrae (2 cases), whole vertebrae (1 case), and pelvis (2 cases). Nine cases had MRI images that contains artifacts caused by the implanted device. Four cases experienced device-related, MRI-derived complications, and one of them underwent magnet replacement surgery. Among the different MRI sequences, diffusion-weighted images and apparent diffusion coefficient (ADC) maps were more negatively influenced by the magnet than T1- or T2-weighted or MR angiography images. Understanding the artifact size and affected structures when MRI is performed on cochlear implant users is useful for determining the indications of MRI and magnet removal surgery, not merely for otolaryngologists but for any healthcare professionals.</p>

DOI： 10.11334/jibi.69.3_153

CiNii Research

Language：Japanese   Publisher：耳鼻と臨床会  

MRIを撮影した人工内耳装用症例を2021年までに18例経験した。MRIの撮影範囲の内訳は頭頸部9例、胸髄2例、肩関節1例、肝臓1例、腰仙髄2例、脊椎1例、骨盤2例であった。このうち頭頸部MRIを撮影した8例は撮影範囲に人工内耳のアーチファクトを認めた。MRI撮影後の人工内耳トラブルが4例に生じ、うち1例で体内器の磁石を入れ替える手術を要した。MRIの強調像のうち、拡散強調像は磁石を入れた状態では評価困難であった。MRI画像の欠損範囲や検査後のトラブルを把握しておくことで、他科の医師とのMRI検査計画を立てやすくなり、また患者の検査への理解を深めることに役立てることが可能と考えられた。(著者抄録)

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>We herein report four operative cases of temporal bone glomus tumors encountered between 2016 and 2021. Two of the cases were glomus jugulare, and the other two were glomus tympanicum. Characteristic symptoms included pulsatile tinnitus in the two patients with glomus tympanicum. Preoperative audiometry revealed conductive hearing loss on the affected side and air-bone gap in three patients. One of the glomus jugulare cases required three surgeries: a biopsy, resection, and reoperation. Vascular embolization was performed in all four cases. There was no evidence of pre- or postoperative neurologic deficit in any cases. No postoperative recurrence or regrowth was observed in any cases, and the patients are under follow-up. Because glomus tumors are rich in blood vessels, preoperative embolization and the use of a high-frequency bipolar coagulator may aid in performing safe surgery.</p>

DOI： 10.11334/jibi.69.2_89

CiNii Research

Publisher：東京医学社  

DOI： 10.24479/ohns.0000000506

CiNii Research

Language：English   Publisher：Virchows Archiv  

High-risk human papillomavirus (HPV) is a risk factor for the development of several head and neck squamous cell carcinomas (SCCs). However, there have been few reports of high-risk HPV infection in temporal bone squamous cell carcinomas (TBSCCs), and thus the prevalence and clinicopathologic significance of high-risk HPV in TBSCCs are still unclear. We retrospectively collected 131 TBSCCs and analyzed them for transcriptionally active high-risk HPV infection using messenger RNA in situ hybridization; we also assessed the utility of p16-immunohistochemistry (IHC) and Rb-IHC to predict HPV infection. Eighteen (13.7%) of the 131 TBSCCs were positive for p16-IHC, and five of them were positive for high-risk HPV infection (the estimated high-risk HPV positivity rate was 3.8% [5/131]). Interestingly, all five HPV-positive patients were male and had TBSCC on the right side. In the p16-IHC+/HPV+ cases (n = 5), the Rb-IHC showed a partial loss pattern (n = 4) or complete loss pattern (n = 1). In contrast, all p16-IHC-negative cases (n = 113) showed an Rb-IHC preserved pattern. The positive predictive value (PPV) of p16-IHC positivity for high-risk HPV infection was low at 27.8%, while the combination of p16-IHC+/Rb-IHC partial loss pattern showed excellent reliability with a PPV of 100%. The prognostic significance of high-risk HPV infection remained unclear. High-risk HPV-related TBSCC is an extremely rare but noteworthy subtype.

DOI： 10.1007/s00428-023-03497-7

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：耳鼻と臨床会  

2016年から2021年にかけて当科で手術を行った側頭骨グロムス腫瘍の4例について報告する。4例の内訳として鼓室型2例、頸静脈球型2例に対し治療を行った。鼓室型の2例では拍動性耳鳴を認めた。術前聴力検査では3例で患側の聴力低下と気骨導差を認めた。頸静脈球型のうち1例では生検、切除、再手術と3回の手術を必要とした。血管塞栓術は鼓室型、頸静脈球型それぞれ1例ずつ施行し、すべての症例において、術前後の神経脱落所見は認めなかった。1例は残存病変があるものの増大なく経過しており、その他3例は再発・再々発なく経過している。グロムス腫瘍は血流豊富であり、栄養血管の塞栓や高周波バイポーラの使用が安全な手術に有用と考えられる。(著者抄録)

Language：Japanese   Publisher：Japan Audiological Society  

<p>　　For the hearing impaired, masks and shields to protect against the spread of the new coronavirus infection COVID-19 cause additional hearing difficulties. We attempted to quantify this additional listening burden using an application to evaluate the listening environment. An application was created that combines speech recognition AI with the CI2004, a Japanese hearing test battery for cochlear implant recipients.</p><p>　　The accurate response rate was 95% when the examination was administered in the quiet. With poorer sound sources or noise, however, the percentage of accurate answers decreased. Although the proportion of correct answers did not significantly drop decrease significantly when the test was administered in a noisy environment with just a mask, it fell significantly when the mask was combined with shielding.</p><p>　　 Our findings suggest that many hearing-impaired individuals find it challenging to understand what is being said by a person hiding talking from behind a shield in noisy settings. Masks do not adversely affect hearing as much as shields, and should be used with consideration for the hearing impaired who use lipreading. It is easy to assess hearing problems in various environments using the same test parameters.</p>

DOI： 10.4295/audiology.66.56

CiNii Research

Language：Japanese   Publisher：(一社)日本聴覚医学会  

難聴者にとっては,新型コロナウイルス感染対策のマスクや遮蔽板がさらなる聴き取りづらさを招く。音声認識AIとCI2004日常会話文を組み合わせたアプリケーションを用いて聴き取りづらさを定量化し,聴取環境を評価する試みを行った。評価対象語60語を正確に聴き取れた数で正解率を算出した。静寂下に検査を行うと正解率は95%であった。劣化音源や雑音下では正解率が低下した。この検査系を用いて院内コンビニエンスストアの聴取環境の評価を行ったところ,著明に正解率が低下した。雑音の有無,マスクと遮蔽板,補助スピーカーの有無で検討を行うと,マスク単体では正解率の有意な低下はなかったが,マスクおよび遮蔽板を設置し雑音下で検査を行うと著明に正解率が低下した。うるさい場所でマスクをした相手が遮蔽板の向こうで話す言葉は聞き取りづらいという難聴者の感覚と矛盾のない結果であった。同じ検査条件を用いれば環境ごとの聴き取りにくさの評価も可能である。(著者抄録)

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>We experienced 15 surgical cases of petrous apex-11 cholesteatomas and 4 cholesterin granulomas-between January 2013 and July 2021. Among the four cases of cholesterin granuloma, one underwent transmastoid surgery, while the others received intranasal endoscopic surgery to create drainage routes. Among the 11 cases of cholesteatoma, 5 received initial surgery, and 6 were reoperations. The extent of the lesion based on Sanna's classification was supralabyrinthine in seven patients, massive in two, and infralabyrinthine in two. In the supralabyrinthine type, the middle fossa approach or combined approach of the middle cranial fossa and translabyrinthine approach was necessary in cases with severe extensions or those receiving reoperation. The minimally invasive procedure through the superior semicircular canal approach was performed in cases with a small extension. In the massive type, the transcochlear and middle fossa combined approach was used, while in the infralabyrinthine type, the infracochlear or infralabyrinthine approach was performed, depending on the localization and degree of the extension. The hearing was preserved in all cases of cholesterin granuloma and three cases of cholesteatomas. Since the petrous apex is located in the deepest part of the temporal bone, it is crucial to have a thorough understanding of the anatomy and select a surgical approach that considers the lesion and hearing.</p>

DOI： 10.11334/jibi.69.1_1

CiNii Research

Language：Japanese   Publisher：耳鼻と臨床会  

2013年1月から2021年7月の期間に手術治療を行った錐体尖病変15例を検討した。コレステリン肉芽腫4例では、1例が経乳突的に、3例は経鼻的にドレナージルートを作成した。錐体尖真珠腫11例では初発例5例、再手術例が6例であり、進展経路は迷路上型が7例、広範迷路型2例、迷路下型2例であった。迷路上型では進展範囲の小さな症例は経上半規管法により低侵襲に摘出し得たが、進展例や再手術例では中頭蓋窩法や、中頭蓋窩法と経迷路法のcombined approachが必要であった。広範迷路型では経蝸牛+中頭蓋窩法のcombined approachを行い、迷路下型では進展範囲に応じて蝸牛下法や迷路下法を用いた。コレステリン肉芽腫全例と錐体尖真珠腫の3例で聴力温存できた。錐体尖は側頭骨最深部に位置し、手術アプローチの選択が重要である。聴力や病変と解剖学的構造の位置関係を考慮して術式を選択する必要がある。(著者抄録)

Language：Japanese   Publisher：Japan Otological Society  

<p>Among recently developed electrode arrays, slim modiolar electrode arrays are prone to folding within the cochlea, a condition known as tip fold-over. One of the mechanisms that cause tip fold-over is the misalignment between the electrode array’s coiling direction and the cochlea’s curving direction. Thus, surgeons should be careful during slim modiolar electrode insertion and align the coiling planes of the electrode array and the cochlea. However, successful alignment of the electrode array’s coiling direction and the cochlea’s curving direction largely depends on the surgeon’s imagination. We recommended preoperative three-dimensional simulation to determine the correct coiling plane of the cochlea. However, not all surgeons are familiar with preoperative simulations using computers. Therefore, we developed a method of determining the coiling plane of the cochlea using anatomical landmarks that can be visualized during cochlear implantation.</p><p>We retrospectively investigated preoperative CT data of 65 ears of patients who underwent cochlear implantation by one surgeon during the period of 2017–2021. Revision cases and inner ear anomaly cases were excluded. The preoperative CT dataset was three-dimensionally reconstructed. A plane was defined containing short and long processes of the incus, as “plane of the incus”. The angle between the plane of the incus and the coiling plane of the cochlea (incudocochlear angle) were measured and collected. The incudocochlear angle was averaged at 34.6 degrees. No statistical differences were noted between the left and right ears. No statistical differences were noted in terms of age at surgery. The 95% confidence interval of the angle was 23.4–45.7 degrees. Thus, once the incus is visualized during surgery, it is possible to correctly determine the coiling plane of the cochlea. This simple method does not require the surgeon’s ability of computer simulation and may help prevent misalignment and subsequent electrode tip fold-over.</p>

DOI： 10.11289/otoljpn.33.170

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.65.353

CiNii Research

Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.65.427

CiNii Research

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

DOI： 10.1097/ono.0000000000000017

PubMed

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>ANCA-associated vasculitis is an autoimmune vasculitis that often develops in systemic diseases, such as renal disorders and pulmonary lesions. However, there have been increasing reports of ANCA-associated vasculitis of otitis media origin and ear-localized type in recent years. A 52-year-old woman with a history of tuberculosis presented with otitis media refractory to antimicrobial agents as well as hearing loss and headache. Although otitis media with ANCA-associated vasculitis (OMAAV) is mentioned as a differential diagnosis for refractory otitis media, the diagnosis was difficult, as ANCA antibodies were negative, biopsy tissue showed no evidence of vasculitis, and there was concern about the possibility of tuberculous otitis media. We ultimately diagnosed the patient with OMAAV by excluding other diseases thorough a collaborative evaluation of other organs, considering the complication of hypertrophic pachymeningitis, which is characteristic of OMAAV. Since we experienced a case of OMAAV that took one year to be diagnosed, we herein report this diagnosis with some discussion of the literature.</p>

DOI： 10.11334/jibi.68.4_266

CiNii Research

Language：Japanese   Publisher：耳鼻と臨床会  

ANCA関連血管炎は腎障害や肺病変などの全身疾患で発症することが多い自己免疫性血管炎であるが、近年、中耳炎初発・耳限局型のANCA関連血管炎の報告が増加している。本症例は結核の既往がある52歳女性で、主症状は抗菌薬無効の中耳炎と難聴、頭痛であった。難治性中耳炎としてANCA関連血管炎性中耳炎(OMAAV)を鑑別に挙げてはいたものの、ANCA抗体陰性で生検組織は血管炎の所見が得られず、また、結核性中耳炎の可能性を懸念し診断に苦慮した。最終的に、OMAAVに特徴的とされる肥厚性硬膜炎の合併や、ANCA関連血管炎やその他自己免疫疾患の診療経験が豊富な膠原病内科医による他臓器評価により除外診断を行いOMAAVの診断を得た。診断までに1年を要したOMAAVの症例を経験したため、主にその診断について若干の文献的考察を加えて報告する。(著者抄録)

Language：Japanese   Publisher：Japanese Society of Otorhinolaryngology-Head and Neck Surgery  

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.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.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%).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： 10.3950/jibiinkotokeibu.125.5_916

PubMed

CiNii Research

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

DOI： 10.11334/jibi.68.3_222

CiNii Research

Language：Japanese   Publisher：Japanese Society of Otorhinolaryngology-Head and Neck Surgery  

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.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%).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.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： 10.3950/jibiinkotokeibu.125.2_213

PubMed

CiNii Research

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

DOI： 10.11334/jibi.68.1_70

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of the American Academy of Audiology  

BACKGROUND: Innovated hearing aids (HAs), termed cartilage conduction hearing aids (CC-HAs), show good performance in patients with closed ears and continuous otorrhea. However, factors other than the ear condition that influence the purchase rate of CC-HAs remain unclear. PURPOSE: To identify the factors that influence the purchase rate of CC-HAs. RESEARCH DESIGN: A correlational study. STUDY SAMPLE: A total of 249 patients were enrolled. DATA COLLECTION AND ANALYSIS: The patients' demographics, clinical characteristics, outcomes, and CC-HA transducer types were compared. The data were analyzed for six groups classified based on the ear condition. RESULTS: In the unilateral closed-ear group, the purchase cases were significantly younger than the nonpurchase cases (p < 0.05). Regarding the outcomes in the bilateral closed-ear group, the purchase cases showed significantly better-aided thresholds at 0.25 and 0.5 kHz than the nonpurchase cases. No significant differences in the functional gains and speech recognition scores were found between purchase and nonpurchase cases in all six groups. Regarding the transducer type, the continued-use rate of the simple transducer type was significantly lower in the bilateral chronic continuous otorrhea, bilateral open, and unilateral open groups. CONCLUSION: In the closed ears, no remarkable negative factors were found. Transducer type had a significant influence on the continued-use rate in the nonclosed ears including the ears with chronic continuous otorrhea, although the purchase rate of CC-HAs in the bilateral chronic continuous otorrhea group was comparable to the closed ears.

DOI： 10.1055/s-0041-1733965

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：JAPAN SOCIETY FOR HEAD AND NECK SURGERY  

本稿では，人工内耳手術を執刀する後輩医師に日頃指導していることを，筆者が重要と考えている順に4点紹介する。手術時間を競わないこと，患者より先に手術室に入ること，罠を張るような紛らわしい指示を出さないこと，そして人工内耳手術においては蝸牛の基底回転が上っていく向きまで予習をした上で手術に臨むことが，普通の人工内耳手術を普通に終わらせ続けるためのリスク管理として有効である。

DOI： 10.5106/jjshns.32.121

CiNii Research

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

BACKGROUND: There is no guideline for hearing compensation after temporal bone resection. This study aimed to retrospectively analyze surgical cases with reconstruction for hearing preservation after temporal bone malignancy resection and propose a new alternative to compensate for hearing loss. METHODS: We retrospectively reviewed the medical records of 30 patients who underwent lateral temporal bone surgery for temporal bone malignancy at our institution and examined their hearing abilities after surgery. RESULT: The hearing outcomes of patients with an external auditory meatus reconstruction varied widely. The mean postoperative air-bone gap at 0.5, 1, 2, and 4 kHz ranged from 22.5 dB to 71.25 dB. On the other hand, the average difference between the aided sound field thresholds with cartilage conduction hearing aid and bone conduction thresholds at 0.5, 1, 2, and 4 kHz ranged from -3.75 to 41.25. More closely located auricular cartilage and temporal bone resulted in smaller differences between the aided sound field and bone conduction thresholds. CONCLUSIONS: There is still room for improvement of surgical techniques for reconstruction of the auditory meatus to preserve hearing after temporal bone resection. The cartilage conduction hearing aid may provide non-invasive postoperative hearing compensation after lateral temporal bone resection.

DOI： 10.3390/audiolres11020024

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

BACKGROUND: Cartilage conduction hearing aids (CCHAs) were newly devised and spread fast in Japan since their launch in 2017. However, little knowledge is available for this new device. PURPOSE: The aim of this study was to establish the knowledge of CCHAs and suggest their indication. RESEARCH DESIGN: Correlational study. STUDY SAMPLE: A total 256 patients were registered. DATA COLLECTION AND ANALYSIS: The fitting of CCHAs was surveyed in nine institutions. The outcomes were assessed by audiometric tests. The patients were classified into seven groups, depending on the ear conditions. The clinical characteristics, assessment results, and purchase rates were compared among the groups. The assessment results of CCHAs were also compared with those of previously used hearing aids. RESULTS: Most patients who used CCHAs were classified into the bilateral closed (aural atresia or severe stenosis) ear (n = 65) or unilateral closed ear (n = 124) groups. The patients in these groups achieved good benefits that resulted in a high purchase rate. The bilateral continuous otorrhea group also supported a high purchase rate, although the benefits of CCHAs were not always excellent. In contrast, the purchase rate was poor in the patients who could use air conduction hearing aids (ACHAs) without absolute problems. As for using a CCHA as a contralateral routing of signals hearing aid, the benefits depended on the patients. CONCLUSIONS: CCHAs are considered as a great option not only to the patients with closed ears but also to those who had difficulties in ACHAs usage.

DOI： 10.1055/s-0041-1728758

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

OBJECTIVES: To obtain new insights into research questions on how executive function and social interaction would be observed to change after the introduction of hearing aids (HAs) in older people with hearing impairment. DESIGN: Multi-institutional prospective single-arm observational study. SETTING AND PARTICIPANTS: Outpatients with complaints of hearing difficulty who visited HA clinics between October 18, 2017, and June 30, 2019, in 7 different university hospitals in Japan. METHODS: The inclusion criteria of the study named Hearing-Aid Introduction for Hearing-Impaired Seniors to Realize a Productive Aging Society-A Study Focusing on Executive Function and Social Activities Study (HA-ProA study) were age >/=60 years and no history of HA use. A series of multi-institution common evaluations including audiometric measurements, the digit symbol substitution test to assess executive functions, convoy model as an index of social relations, and hearing handicap inventory for the elderly (HHIE) were performed before (pre-HA) and after 6 months of the HA introduction (post-HA). RESULTS: Out of 127 enrollments, 94 participants completed a 6-month follow-up, with a mean age of 76.9 years. The digit symbol substitution test score improved significantly from 44.7 at baseline to 46.1 at 6 months (P = .0106). In the convoy model, the social network size indicated by the number of persons in each and whole circles were not significantly different between pre- and post-HA; however, the total count for kin was significantly increased (P = .0344). In the analyses of HHIE, the items regarding the family and relatives showed significant improvement. CONCLUSIONS AND IMPLICATIONS: HA use could benefit older individuals beginning to use HAs in executive function and social interaction, though the results should be interpreted cautiously given methodological limitations such as a single-arm short 6 months observation. Reduction in daily hearing impairment would have a favorable effect on relationships with the family.

DOI： 10.1016/j.jamda.2021.02.035

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)  

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)  

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)  

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

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DOI： 10.1136/bcr-2017-222657

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DOI： 10.1002/lary.25998

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DOI： 10.1007/s11548-016-1441-0

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DOI： 10.1016/j.anl.2015.08.008

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DOI： 10.1007/s00405-014-3467-7

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DOI： 10.1017/S0022215114002308

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DOI： 10.1109/EMBC.2013.6610851

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DOI： 10.1016/j.brainres.2004.03.001

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DOI： 10.1113/jphysiol.2002.018267

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Language：Japanese   Publisher：(株)全日本病院出版会  

先天性難聴の中で遺伝性難聴は50%以上を占める。このため、小児の難聴を診察するうえで、遺伝性難聴の可能性は常に念頭に置くべきである。遺伝性難聴を見逃さないためには、まず言語発達遅滞などから難聴が疑われる児を拾い上げることが重要である。また、家族歴がない場合にも遺伝性難聴である可能性は十分にあること、遺伝性難聴の中には進行性のものもあることに留意し、同意が得られる場合には遺伝学的検査を検討する。家族歴の聴取、詳細な家系図を作成することも遺伝性難聴を見逃さないために必要である。保険収載の遺伝学的検査は頻度の高い遺伝子変異のみをターゲットにしているため、変異の検出がないからといって遺伝性難聴が否定されるわけではない。多施設共同研究で次世代シークエンス法などを用いた検査によって原因遺伝子が判明することがある。(著者抄録)

Language：Japanese   Publisher：耳鼻と臨床会  

Language：Japanese   Publisher：耳鼻と臨床会  

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