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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
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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 ) 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
井手 慎介, 梶原 啓, 中村 雄, 髙橋 邦行
Cureus 18 ( 1 ) e102558 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：Springer Science and Business Media LLC
Leiomyosarcoma (LMS) is a malignant tumor that originates from smooth muscle. Cutaneous LMS is a distinct, superficial sarcoma that arises from dermal smooth muscle. It generally has a more favorable prognosis than subcutaneous or deep soft tissue LMS. Head and neck LMS accounts for a small proportion of all LMS cases, and primary tumors in the external auditory canal are extremely rare.
We report a case of cutaneous LMS arising from the external auditory canal. The patient was a 32-year-old woman. She presented with a two-month history of a left external auditory canal mass, hearing loss, and ear pain. Contrast-enhanced CT and MRI revealed a 17-mm tumor confined to the cartilaginous portion of the external auditory canal, with no obvious infiltration into the bony portion. Biopsy results showed features of a smooth muscle tumor, and the level of Ki-67 expression led to a diagnosis of LMS. We resected the tumor via a preauricular incision and a longitudinal incision of the external auditory canal, including the external auditory canal cartilage, the surrounding soft tissue, and part of the temporomandibular joint capsule. Histopathology revealed tumor cells with moderately atypical, spindle-shaped nuclei arranged in fasciculated patterns. Necrosis was observed at a rate of 30%-40%, and mitotic cells were observed at a rate of 5 per 10 high-power fields. This led to a diagnosis of Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) Grade 2 LMS. Tumor cells were noted near the resection margin, and the safety margin appeared inadequate. Postoperative epithelialization of the external auditory canal was favorable, and no recurrence was detected on contrast-enhanced CT at five months.
Although there is no established treatment strategy for head and neck soft tissue sarcomas, complete resection with adequate margins is essential. However, achieving adequate surgical margins is challenging due to functional and cosmetic considerations in the head and neck region. As in this case, small, brownish LMS are clinically considered to be cutaneous LMS, which generally have a relatively good prognosis. Close follow-up is required in cases involving resection close to the margin, and adjuvant therapies such as radical resection or radiation should be considered if recurrence occurs. A treatment strategy tailored to the tumor type and resection margin is necessary for primary LMS of the head and neck region. -
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
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