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医学部 附属病院 耳鼻いんこう・頭頸部外科 |
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講師 |
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論文 【 表示 ／ 非表示 】
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Nakamura T, Ganaha A, Tono T, Yamada Y, Okuda T, Shimoara S, Matsuda Y
Auris, nasus, larynx 49 ( 6 ) 1072 - 1077 2021年5月
担当区分：筆頭著者 記述言語：英語 掲載種別：研究論文（学術雑誌） 出版者・発行元：Auris Nasus Larynx
There are few reports of the treatment for severe hearing loss due to otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) achieved by cochlear implantation (CI). Here, we have reported the case of a patient with severe bilateral sensorineural hearing loss with low-frequency residual hearing by OMAAV. CI was performed in her right ear based on the results of contrast-enhanced magnetic resonance imaging (CE-MRI) and promontory stimulation test (PST). The residual hearing in her right ear was preserved after CI and utilized for combined electric acoustic stimulation (EAS). The combined EAS was used for 3 years until the residual hearing became stabilized. However, the usable hearing in low frequency worsened gradually, and the fitting strategy of cochlear implant was changed from combined EAS to CI alone 4 years after CI. Even when the speech discrimination score with CI no longer exceeds 50 %, the patient continued using CI because of its advantages in maintaining the quality of life of the patient. The combined EAS was found to be a feasible option even in an OMAAV patient with residual hearing. CE-MRI and PST may thus be helpful in deciding the side of CI surgery in a patient with OMAAV.
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Hearing characteristics of Branchio-oto-renal syndrome in Japan. 査読あり
Goto SI, Sasaki A, Nishio SY, Morita SY, Ogasawara N, Kobayashi Y, Amano A, Shinkawa C, Oda K, Wada T, Ikezono T, Matsuda H, Fujisaka M, Nagai K, Yoshimura H, Kashio A, Nishiyama N, Ito T, Tajima S, Oka SI, Kaga K, Takeda H, Kobayashi M, Sano H, Arai Y, Nakanishi H, Koizumi H, Obara N, Yoshida T, Esaki T, Takeuchi K, Yamazaki H, Horie R, Ohta Y, Morimoto C, Uehara N, Naito Y, Maeda Y, Ishino T, Egusa K, Sugahara K, Teraoka M, Kondo E, Tsuchihashi N, Kihara C, Kanda Y, Nakamura T, Miyanohara I, Kondo S, Usami SI
Acta oto-laryngologica 1 - 10 2026年3月
記述言語：英語 掲載種別：研究論文（学術雑誌） 出版者・発行元：Acta Oto Laryngologica
Background: Branchio-oto-renal (BOR) syndrome is characterized by branchiogenic malformation, hearing loss, and renal anomalies, with EYA1, SIX1, and SIX5 known as the causative genes. As BOR syndrome presents with various clinical phenotypes, its characteristics and genotype-phenotype correlations remain unknown. Aims/objectives: In this study, we aimed to clarify the detailed hearing loss phenotypes and genotype-phenotype correlations of BOR syndrome. Material and methods: In this study, we performed an etiological analysis of 169 BOR syndrome patients from 129 families. We also performed genetic testing for 78 probands. Results: In all, 66.7% of BOR patients carried EYA1 variants, whereas 17.9% carried SIX1 variants. We also clarified the detailed clinical features including the prevalence of major and minor symptoms, asymmetrical hearing loss, type of hearing loss, severity of hearing loss and detailed clinical characteristics of auricular, external ear, and middle ear and inner ear anomalies. In terms of genotype-phenotype correlations, patients with SIX1 variants had no kidney anomalies and fewer middle ear anomalies. Conclusions and significance: We clarified the detailed hearing loss phenotypes of BOR syndrome patients. Our study results will contribute to a better understanding and clinical management of BOR syndrome patients.
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The Clinical Details of MYH9-Related Disease and DFNA17 in a Large Japanese Hearing Loss Cohort. 査読あり
Goto S, Sasaki A, Nishio SY, Shinkawa C, Oda K, Wada T, Ishikawa K, Ikezono T, Oka SI, Nishiyama N, Ito T, Kobayshi M, Kumakawa K, Sakuma N, Nakanishi H, Morimoto C, Uehara N, Okazaki T, Sugahara K, Nakamura T, Usami SI
Genes 17 ( 2 ) 154 2026年1月
記述言語：英語 掲載種別：研究論文（学術雑誌） 出版者・発行元：Genes
Background/Objectives: MYH9 gene variants cause MYH9-related disease (MYH9-RD), which is also known as Epstein syndrome, Fechtner syndrome, May–Hegglin anomaly, and Sebastian syndrome. MYH9-RD is characterized by sensorineural hearing loss, macrothrombocytopenia, thrombocytopenia, hematuria/proteinuria, glomerulonephritis, cataracts purpura, and mucosal bleeding. In addition, the MYH9 gene is also known to be causative of autosomal dominant non-syndromic hearing loss (DFNA17). MYH9-RD is a relatively rare disorder, and the detailed clinical features and mutational spectra remain unclear. Methods: In this study, we performed next-generation sequencing analysis for 15,684 hearing loss patients and identified MYH9-associated hearing loss patients. Detailed clinical information was collected for these patients and summarized. Results: In this study, we identified 24 patients from 18 families with MYH9-associated hearing loss. We clarified the details of hearing deterioration observed in patients based on collected serial audiogram data. Some cases showed rapid hearing deterioration that worsened by about 50 dB within 5 years. Hearing loss is more likely to progress in patients with myosin head domain variants than in patients with myosin tail domain variants, but hearing loss in each set of patients finally deteriorates to bilateral profound hearing loss. Conclusions: In this study, we were able to clarify the detailed characteristics of MYH9-RD- and DFNA17-related hearing loss in a relatively large number of patients, particularly in some cases that showed rapid and asymmetrical hearing deterioration progressing to bilateral profound hearing loss. Our data will be useful for providing more appropriate treatment and follow-up for MYH9-associated hearing loss.
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A Rare Case of Cutaneous Leiomyosarcoma Arising From the External Auditory Canal. 査読あり
Yamashita J, Ide S, Kajihara K, Nakamura T, Takahashi K
Cureus 18 ( 1 ) e102558 2026年1月
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Analysis of Postoperative Vertigo Following Cochlear Implant Surgery
Yuji T., Nakamura T., Kiyomizu K., Takahashi K.
Equilibrium Research 85 ( 1 ) 6 - 12 2026年
記述言語：日本語 掲載種別：研究論文（学術雑誌） 出版者・発行元：Equilibrium Research
We investigated the incidence of postoperative vertigo and explored factors influencing the risk of its development following cochlear implant (CI) surgery. The study included 105 patients aged 18 years or older who underwent their first CI implantation at our institution between April 2014 and March 2024. We defined “postoperative vertigo” as the development of vertigo within one month after the surgery. The patients were classified into two groups-a vertigo group and a no-vertigo group-based on whether they developed/did not develop postoperative vertigo. We examined the incidence of postoperative vertigo and investigated its association with the age, gender, surgical method employed, electrode shape, electrode thickness, prior vertigo history, and preoperative vestibular function. The results revealed no association between the development of postoperative vertigo and the age, gender, surgical method employed, electrode shape, or electrode thickness. However, postoperative vertigo occurred significantly more frequently in patients with prior vertigo history, patients with preoperative vestibular dysfunction at least on one side, especially in those with vestibular dysfunction in the non-surgical ear. In conclusion, it is important to obtain a careful history of prior vertigo episodes and to perform vestibular function tests on both the surgical and non-surgical sides prior to CI surgery to predict the risk of development of postoperative vertigo.
DOI： 10.3757/jser.85.6
講演・口頭発表等 【 表示 ／ 非表示 】
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VSB implantation in a NF1 patient with multiple bony defects in the temporal bones and the skull. 国際会議
Akira Ganaha, Keiji Matsuda, Kei Kajihara, Takeshi Goto, Takeshi Nakamura, Noriaki Miyanaga, Tetsuya Tono
12th Asia Pacific Symposium on Cochlear Implants and Related Sciences
開催年月日： 2019年11月27日 - 2019年11月30日
記述言語：英語 会議種別：ポスター発表
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Benefit of hearing preservation during cochlear implantation in patients with OMAAV. 国際会議
Nakamura Takeshi, Tetsuya Tono, Shouken Shimoara, Yuusuke Matsuda, Takahiro Nakashima
12th Asia Pacific Symposium on Cochlear Implants and Related Sciences
開催年月日： 2019年11月27日 - 2019年11月30日
記述言語：日本語 会議種別：ポスター発表
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めまい主訴の側頭葉てんかん・巨赤芽球性貧血症例．
湯地俊子，清水謙祐，中村 雄，鳥原康治，東野哲也
第78回日本めまい平衡医学会総会・学術講演会
開催年月日： 2019年10月23日 - 2019年10月25日
記述言語：日本語 会議種別：ポスター発表
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アブミ骨手術における顔面神経の解剖学的要因の影響．
中島崇博，梶原 啓，中村 雄，後藤隆史，我那覇章，松田圭二，東野哲也
第29回日本耳科学会総会・学術講演会
開催年月日： 2019年10月10日 - 2019年10月12日
記述言語：日本語 会議種別：口頭発表（一般）
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新生児聴覚スクリーニングにより早期診断に至った先天性伝音難聴に対して手術治療を行った3例の検討．
中村 雄，池ノ上あゆみ，我那覇章，中島崇博，後藤隆史，山田悠祐，猿渡英美，東野哲也
第29回日本耳科学会総会・学術講演会
開催年月日： 2019年10月10日 - 2019年10月12日
記述言語：日本語 会議種別：口頭発表（一般）
科研費（文科省・学振・厚労省）獲得実績 【 表示 ／ 非表示 】
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音源定位をサポートするハプティックデバイスの開発/一側聲の包括的治療を目指して
研究課題/領域番号：22K16914 2022年04月 - 2025年03月
独立行政法人日本学術振興会 科学研究費基金 若手研究
担当区分：研究代表者