Affiliation |
Faculty of Medicine College Hospital Otorhinolaryngology |
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Research Areas 【 display / non-display 】
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Life Science / Otorhinolaryngology
Papers 【 display / non-display 】
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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 2021.5
Authorship:Lead author Language:English Publishing type:Research paper (scientific journal) Publisher: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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特集 TEES 【TEESを学ぶ】 TEESのための画像診断 Invited
中村 雄, 高橋 邦行
JOHNS 40 ( 12 ) 1463 - 1466 2024.12
Authorship:Lead author Language:Japanese Publishing type:Research paper (scientific journal) Publisher:東京医学社
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The prevalence and clinical features of MYO7A-related hearing loss including DFNA11, DFNB2 and USH1B Reviewed International coauthorship
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 ) 2024.12
Language:English Publishing type:Research paper (scientific journal) Publisher: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. Reviewed
Ganaha A, Nojiri N, Nakamura T, Higa T, Kondo S, Tono T
Journal of clinical medicine 13 ( 21 ) 2024.11
Language:English Publishing type:Research paper (scientific journal) Publisher: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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増刊号 ランドマークはこれだ! 局所解剖アトラス〔特別付録Web動画〕 1.耳 乳突削開術のための局所解剖 Invited
中村 雄, 髙橋 邦行
耳鼻咽喉科・頭頸部外科 96 ( 5 ) 38 - 45 2024.4
Authorship:Lead author Language:Japanese Publishing type:Research paper (scientific journal) Publisher:株式会社医学書院
Presentations 【 display / non-display 】
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VSB implantation in a NF1 patient with multiple bony defects in the temporal bones and the skull. International conference
Akira Ganaha, Keiji Matsuda, Kei Kajihara, Takeshi Goto, Takeshi Nakamura, Noriaki Miyanaga, Tetsuya Tono
12th Asia Pacific Symposium on Cochlear Implants and Related Sciences
Event date: 2019.11.27 - 2019.11.30
Language:English Presentation type:Poster presentation
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Benefit of hearing preservation during cochlear implantation in patients with OMAAV. International conference
Nakamura Takeshi, Tetsuya Tono, Shouken Shimoara, Yuusuke Matsuda, Takahiro Nakashima
12th Asia Pacific Symposium on Cochlear Implants and Related Sciences
Event date: 2019.11.27 - 2019.11.30
Language:Japanese Presentation type:Poster presentation
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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
Event date: 2019.10.23 - 2019.10.25
Language:Japanese Presentation type:Poster presentation
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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
Event date: 2019.10.10 - 2019.10.12
Language:Japanese Presentation type:Oral presentation (general)
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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
Event date: 2019.10.10 - 2019.10.12
Language:Japanese Presentation type:Oral presentation (general)
Grant-in-Aid for Scientific Research 【 display / non-display 】
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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
Grant number:22K16914 2022.04 - 2024.03
独立行政法人日本学術振興会 科学研究費補助金 若手研究
Authorship:Principal investigator
Other research activities 【 display / non-display 】
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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/一側聾の包括的治療を目指して