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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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外部リンク |
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関連SDGs |
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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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The prevalence and clinical features of MYO7A-related hearing loss including DFNA11, DFNB2 and USH1B 査読あり 国際共著
Watanabe K., Nishio S.Y., Usami S.I., Kumai T., Katada A., Ogasawara N., Shintani T., Morita S.Y., Takeichi N., Goto S.I., Nanba A., Sasaki A., Kobayashi Y., Honkura Y., Adachi M., Takai S., Oda K., Sato T., Yamada T., Shiina K., Ito T., Shinkawa C., Amano A., Kikuchi D., Ogawa H., Wada T., Hirose Y., Noguchi E., Moriyama N., Ohtsuka K., Shirai K., Sadayasu R., Shimada M., Suzumura H., Tono T., Motegi M., Mitoh I., Tada H., Nagai K., Sakata H., Ishikawa K., Yoshida N., Mizutari K., Suzuki Y., Ikezono T., Matsuda H., Noguchi Y., Takeda H., Kobayashi M., Sakurai Y., Hirabayashi G., Tajima S., Nishiyama N., Shirai K., Kawaguchi S., Iwasaki S., Takahashi M., Furutate S., Oka S.I., Yoshihashi H., Futagawa H., Ohishi N., Hosoya M., Kawashima Y., Ito T., Maruyama A., Kumakawa K., Matsunobu T., Sakuma N., Takahashi K., Kashio A., Monobe H., Miyoshi Y., Yabuki K., Seto Y., Sano H., Araki N., Arai Y., Okami M., Wasano K., Hatakeyama H., Isono Y., Ohira S., Komori M., Izumi S., Fujisaka M., Watanabe A., Okamoto M., Ito Y., Takahashi M., Miyagawa M., Takumi Y., Yoshimura H., Shinagawa J., Moteki H., Tsukamoto K., Ichinose A., Obara N., Kuza B., Takada N.
Scientific Reports 14 ( 1 ) 2024年12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：Scientific Reports
The MYO7A gene is known to be responsible for both syndromic hearing loss (Usher syndrome type1B:USH1B) and non-syndromic hearing loss including autosomal dominant and autosomal recessive inheritance (DFNA11, DFNB2). However, the prevalence and detailed clinical features of MYO7A-associated hearing loss across a large population remain unclear. In this study, we conducted next-generation sequencing analysis for a large cohort of 10,042 Japanese hearing loss patients. As a result, 137 patients were identified with MYO7A-associated hearing loss so that the prevalence among Japanese hearing loss patients was 1.36%. We identified 70 disease-causing candidate variants in this study, with 36 of them being novel variants. All variants identified in autosomal dominant cases were missense or in-frame deletion variants. Among the autosomal recessive cases, all patients had at least one missense variant. On the other hand, in patients with Usher syndrome, almost half of the patients carried biallelic null variants (nonsense, splicing, and frameshift variants). Most of the autosomal dominant cases showed late-onset progressive hearing loss. On the other hand, cases with autosomal recessive inheritance or Usher syndrome showed congenital or early-onset hearing loss. The visual symptoms in the Usher syndrome cases developed between age 5–15, and the condition was diagnosed at about 6–15 years of age.
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Diagnosis of Enlarged Vestibular Aqueduct Using Wideband Tympanometry. 査読あり
Ganaha A, Nojiri N, Nakamura T, Higa T, Kondo S, Tono T
Journal of clinical medicine 13 ( 21 ) 2024年11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：Journal of Clinical Medicine
Background: Wideband tympanometry (WBT) has the potential to distinguish various mechanical middle ear and inner ear pathologies noninvasively. This study investigated the diagnostic value of WBT in the diagnosis of enlarged vestibular aqueduct (EVA). Methods: The absorbance and resonance frequency (RF) of patients with EVA (40 ears, 25 patients) and matched population controls (39 ears, 28 subjects) were compared, alongside receiver operating characteristic (ROC) analysis. Correlations between VA width and RF were also examined. Results: Patients with EVA had higher absorbance at low frequencies (226–917 Hz) and lower absorbance at high frequencies (2520–4896 Hz) compared to controls. The RF of the EVA group was significantly lower versus controls (751 [391–1165] vs. 933 [628–1346] Hz). The ROC analysis revealed area under the curve values of 0.771 and 0.801, respectively, for absorbance and RF. RF had a sensitivity, specificity, positive predictive value, and negative predictive value of 74.4%, 82.5%, 76.7%, and 80.6%, respectively, for diagnosing EVA. In the EVA group, the VA midpoint width (r = −0.334) and VA petrous width (r = −0.402) both significantly correlated with RF. Conclusions: Our findings support the utility of WBT for diagnosing EVA, with RF as the optimal index used.
DOI： 10.3390/jcm13216602
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松井 広大, 白根 美帆, 中村 雄, 髙橋 邦行
ＡＵＤＩＯＬＯＧＹ ＪＡＰＡＮ 67 ( 5 ) 351 - 351 2024年9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