MOTEGI HIDEAKI 茂木英明

… Alternative Names

Researcher Number	60422698
Affiliation (Current)	2021: 信州大学, 医学部, 特任准教授
Affiliation (based on the past Project Information) *help	2019 – 2020: 信州大学, 医学部附属病院, 医員 2015 – 2018: 信州大学, 学術研究院医学系(医学部附属病院), 講師 2017: 信州大学, 医学部, 助教 2015: 信州大学, 医学部附属病院, 講師 2008 – 2013: 信州大学, 医学部, 助教
Review Section/Research Field	Principal Investigator Otorhinolaryngology / Basic Section 56050:Otorhinolaryngology-related Except Principal Investigator Otorhinolaryngology
Keywords	Principal Investigator 遺伝子 / 難聴 / 次世代シークエンサー / 内耳 / 感音難聴 / 細胞外マトリックス / アレイCGH / CNV / 遺伝子コピー数変化 / aCGH … More Except Principal Investigator 難聴 / 遺伝子 / 内耳 / 感音難聴 / 耳科学 / 次世代シークエンサー / 細胞外マトリクス / 細胞外マトリックス / 蓋膜 / コラーゲン / ABC法 / 細胞外マトリソクス / 先天CMV感染症 / 先天性CMV感染症 / 検査 / 先天性サイトメガロウイルス感染症 / 保存臍帯 / Real-Time PCR解析 / DNA量 / ゲノム / 遺伝性難聴 / 遺伝学 / 医療・福祉 Less

Sensorineural hearing loss caused by genomic alterationPrincipal Investigator
- Principal Investigator
  
  茂木英明
- Project Period (FY)
  2018 – 2020
- Research Category
  
  Grant-in-Aid for Scientific Research (C)
- Review Section
  
  Basic Section 56050:Otorhinolaryngology-related
- Research Institution
  Shinshu University
Copy number variations in hearing lossPrincipal Investigator
- Principal Investigator
  
  MOTEKI Hideaki
- Project Period (FY)
  2015 – 2017
- Research Category
  
  Grant-in-Aid for Scientific Research (C)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University
Development of high throughput sequencing analysis based system for personalized medicine of deafness
- Principal Investigator
  
  Usami Shin-ichi
- Project Period (FY)
  2015 – 2017
- Research Category
  
  Grant-in-Aid for Scientific Research (A)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University
Development of the molecular diagnostic system for congenital CMV infection
- Principal Investigator
  
  IWASAKI Satoshi
- Project Period (FY)
  2011 – 2013
- Research Category
  
  Grant-in-Aid for Scientific Research (C)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University
Comprehensive genetic analysis of hearing loss using next generation sequencer.
- Principal Investigator
  
  USAMI Shinichi
- Project Period (FY)
  2010 – 2012
- Research Category
  
  Grant-in-Aid for Scientific Research (A)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University
Mechanisms of hearing loss caused by gene mutations encoding component proteins of tectorail membrane.Principal Investigator
- Principal Investigator
  
  MOTEKI Hideaki
- Project Period (FY)
  2009 – 2011
- Research Category
  
  Grant-in-Aid for Scientific Research (C)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University
Mechanisms of hearing loss caused by the extra-cellar matrix
- Principal Investigator
  
  ASAMURA Kenji
- Project Period (FY)
  2006 – 2008
- Research Category
  
  Grant-in-Aid for Young Scientists (B)
- Research Field
  
  Otorhinolaryngology
- Research Institution
  Shinshu University

All 2021 2019 2018 2017 2016 2015 2013 2012 2011 2010 2008

All Journal Article Presentation

[Journal Article] Identification of a Novel Copy Number Variation of EYA4 Causing Autosomal Dominant Non-Syndromic Hearing Loss2021
- Author(s)
  Ishino T, Ogawa Y, Sonoyama T, Taruya T, Kono T, Hamamoto T, Ueda T, Takeno S, Moteki H, Nishio SY, Usami SI, Nagano Y, Yoshimura A, Yoshikawa K, Kato M, Ichimoto M, Watanabe R
- Journal Title
  
  Otology and Neurotology
  
  Volume: 14
- DOI
  10.1097/mao.0000000000003169
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-18K09343
[Journal Article] Frequency and clinical features of hearing loss caused by STRC deletions2019
- Author(s)
  Yokota Y, Moteki H, Nishio SY, Yamaguchi T, Wakui K, Kobayashi Y, Ohyama K, Miyazaki H, Matsuoka R, Abe S, Kumakawa K, Takahashi M, Sakaguchi H, Uehara N, Ishino T, Kosho T, Fukushima Y, Usami SI.
- Journal Title
  
  Scientific Reports
  
  Volume: 13 Pages: 4408-4408
- DOI
  10.1038/s41598-019-40586-7
- NAID
  120006601523
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-17K11322, KAKENHI-PROJECT-18K09343
[Journal Article] Comprehensive analysis of syndromic hearing loss patients in Japan.2019
- Author(s)
  Ideura Michie、Nishio Shin-ya、Sugahara Kazuma、他
- Journal Title
  
  Sci Rep
  
  Volume: 9 Pages: 11976-11976
- DOI
  10.1038/s41598-019-47141-4
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-18K09321, KAKENHI-PROJECT-18K09343, KAKENHI-PROJECT-18K09392, KAKENHI-PROJECT-19K09885, KAKENHI-PROJECT-17K11322, KAKENHI-PROJECT-17K11324
[Journal Article] Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss2019
- Author(s)
  Sugiyama、Moteki、Kitajiri、Kitano、Nishio、Yamaguchi、Wakui、Abe、Ozaki、Motegi、Matsui、Teraoka、Kobayashi、Kosho、Usami
- Journal Title
  
  genes
  
  Volume: 10 Pages: 715-732
- DOI
  10.3390/genes10090715
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-18K09343, KAKENHI-PROJECT-18K16829, KAKENHI-PROJECT-17H04345
[Journal Article] A rational approach to identifying newborns with hearing loss caused by congenital cytomegalovirus infection by dried blood spot screening2018
- Author(s)
  Moteki H, Isaka Y, Inaba Y, Motobayashi M, Nishio SY, Ohira S, Yano T, Iwasaki S, Shiozawa T, Koike K, Usami SI.
- Journal Title
  
  Acta Otolaryngol
  
  Volume: 1 Pages: 708-712
- DOI
  10.1080/00016489.2018.1441545
- Peer Reviewed / Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-15K20199, KAKENHI-PROJECT-16K09986
[Journal Article] Diagnostic pitfalls for GJB2-related hearing loss: A novel deletion detected by Array-CGH analysis in a Japanese patient with congenital profound hearing loss2018
- Author(s)
  Abe Satoko、Nishio Shin-ya、Yokota Yoh、Moteki Hideaki、Kumakawa Kozo、Usami Shin-ichi
- Journal Title
  
  Clinical Case Reports
  
  Volume: 6 Pages: 2111-2116
- DOI
  10.1002/ccr3.1800
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-18K09343
[Journal Article] WFS1 mutation screening in a large series of Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis.2018
- Author(s)
  Kobayashi M, Miyagawa M, Nishio SY, Moteki H, Fujikawa T, Ohyama K, Sakaguchi H, Miyanohara I, Sugaya A, Naito Y, Morita SY, Kanda Y, Takahashi M, Ishikawa K, Nagano Y, Tono T, Oshikawa C, Kihara C, Takahashi H, Noguchi Y, Usami SI.
- Journal Title
  
  PLoS One
  
  Volume: 12
- DOI
  10.1371/journal.pone.0193359
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-16K11176, KAKENHI-PROJECT-15K20209, KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-26861365, KAKENHI-PROJECT-17K11322, KAKENHI-PROJECT-18K16890
[Journal Article] Simple and efficient germline copy number variant visualization method for the Ion AmpliSeq custom panel2018
- Author(s)
  Nishio SY, Moteki H, Usami SI.
- Journal Title
  
  Molecular Genetics & Genomic Medicine
  
  Volume: 1 Pages: 678-686
- DOI
  10.1002/mgg3.399
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-17K11322, KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-15K10747, KAKENHI-PROJECT-18K09343
[Journal Article] POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss.2017
- Author(s)
  Kitano T, Miyagawa M, Nishio SY, Moteki H, Oda K, Ohyama K, Miyazaki H, Hidaka H, Nakamura KI, Murata T, Matsuoka R, Ohta Y, Nishiyama N, Kumakawa K, Furutate S, Iwasaki S, Yamada T, Ohta Y, Uehara N, Noguchi Y, Usami SI.
- Journal Title
  
  PLoS One
  
  Volume: 12
- DOI
  10.1371/journal.pone.0177636
- NAID
  120006373770
- Peer Reviewed / Open Access
- Data Source
  KAKENHI-PROJECT-16K11176, KAKENHI-PROJECT-17K11322, KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-26861365, KAKENHI-PROJECT-17K11356
[Journal Article] Social Health Insurance-Based Simultaneous Screening for 154 Mutations in 19 Deafness Genes Efficiently Identified Causative Mutations in Japanese Hearing Loss Patients.2016
- Author(s)
  Mori K, Moteki H, Miyagawa M, Nishio SY, Usami S.
- Journal Title
  
  PLoS ONE
  
  Volume: 11
- DOI
  10.1371/journal.pone.0162230
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-26861365
[Journal Article] Detection and Confirmation of Deafness-Causing Copy Number Variations in the STRC Gene by Massively Parallel Sequencing and Comparative Genomic Hybridization2016
- Author(s)
  Moteki H, Azaiez H, Sloan-Heggen CM, Booth K, Nishio S, Wakui K, Yamaguchi T, Kolbe DL, Iwasa Y, Shearer AE, Fukushima Y, Smith RJ, Usami S.
- Journal Title
  
  Annals of Otology, Rhinology & Laryngology
  
  Volume: 125 Pages: 918-923
- DOI
  10.1177/0003489416661345
- Peer Reviewed / Acknowledgement Compliant / Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-15K10747
[Journal Article] An effective screening strategy for deafness in combination with a next-generation sequencing platform: a consecutive analysis.2016
- Author(s)
  Sakuma N, Moteki H, Takahashi M, Nishio SY, Arai Y, Yamashita Y, Oridate N, Usami S.
- Journal Title
  
  Journal Human Genetics
  
  Volume: 61 Pages: 253-261
- DOI
  10.1038/jhg.2015.143
- NAID
  40020766326
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-26861407
[Journal Article] Comprehensive genetic testing with ethnic-specific filtering by allele frequency in a Japanese hearing-loss population.2015
- Author(s)
  Moteki H, Azaiez H, Booth KT, Shearer AE, Sloan CM, Kolbe DL, Nishio S, Hattori M, Usami S, Smith RJ.
- Journal Title
  
  Clin Genet.
  
  Volume: e-pub
- DOI
  10.1111/cge.12677
- Peer Reviewed / Acknowledgement Compliant / Open Access / Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15K10747, KAKENHI-PROJECT-15H02565
[Journal Article] Novel mutations in LRTOMT associated with moderate progressive hearing loss in autosomal recessive inheritance.2015
- Author(s)
  Ichinose A, Moteki H, Hattori M, Nishio SY, Usami S.
- Journal Title
  
  Ann Otol Rhinol Laryngol.
  
  Volume: 124
- DOI
  10.1177/0003489415575043
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-15H02565
[Journal Article] Non-ocular Stickler syndrome with a novel mutation in COL11A2 diagnosed by massively parallel sequencing in Japanese hearing loss patients.2015
- Author(s)
  Iwasa Y, Moteki H, Hattori M, Sato R, Nishio SY, Takumi Y, Usami S.
- Journal Title
  
  Ann Otol Rhinol Laryngol.
  
  Volume: 124
- DOI
  10.1177/0003489415575044
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-15H02565
[Journal Article] Novel mutation in GRXCR1 at DFNB25 lead to progressive hearing loss and dizziness.2015
- Author(s)
  Mori K, Miyanohara I, Moteki H, Nishio SY, Kurono Y, Usami S.
- Journal Title
  
  Ann Otol Rhinol Laryngol.
  
  Volume: 124
- DOI
  10.1177/0003489415575061
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-15H02565
[Journal Article] Novel PTPRQ mutations identified in three congenital hearing loss patients with various types of hearing loss.2015
- Author(s)
  Sakuma N, Moteki H, Azaiez H, Booth KT, Takahashi M, Arai Y, Shearer AE, Sloan CM, Nishio SY, Kolbe DL, Iwasaki S, Oridate N, Smith RJ, Usami S.
- Journal Title
  
  Annals of Otology, Rhinology, Laryngology
  
  Volume: 124
- DOI
  10.1177/0003489415575041
- Peer Reviewed / Acknowledgement Compliant / Open Access / Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565, KAKENHI-PROJECT-26861407
[Journal Article] The patients associated with TMPRSS3 mutations are good candidates for electric acoustic stimulation.2015
- Author(s)
  Miyagawa M, Nishio SY, Sakurai Y, Hattori M, Tsukada K, Moteki H, Kojima H, Usami S.
- Journal Title
  
  Ann Otol Rhinol Laryngol.
  
  Volume: 124
- DOI
  10.1177/0003489415575056
- Peer Reviewed / Acknowledgement Compliant / Open Access
- Data Source
  KAKENHI-PROJECT-26861365, KAKENHI-PROJECT-15H02565
[Journal Article] Mutations in LOXHD1 gene cause various types and severities of hearing loss.2015
- Author(s)
  Mori K, Moteki H, Kobayashi Y, Azaiez H, Booth KT, Nishio SY, Sato H, Smith RJ, Usami S.
- Journal Title
  
  Ann Otol Rhinol Laryngol.
  
  Volume: 124
- DOI
  10.1177/0003489415574067
- Peer Reviewed / Acknowledgement Compliant / Open Access / Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565
[Journal Article] Evaluation of cortical processing of language by use of positron emission tomography in hearing loss children with congenital cytomegalovirus infection2013
- Author(s)
  ③Moteki H, Suzuki M, Naito Y, Fujiwara K, Oguchi K, Nishio S, Iwasaki S, Usami S
- Journal Title
  
  Int J Pediatr Otorhinolaryngol
  
  Volume: 78(2) Pages: 285-289
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 一側性難聴児における先天性サイトメガロウイルス感染症の関与2013
- Author(s)
  岩崎聡、古舘佐起子、西尾信哉、矢野卓也、茂木英明、工穣、宇佐美真一
- Journal Title
  
  Otol JPN
  
  Volume: 23(5) Pages: 848-853
- NAID
  130005065211
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Patients with CDH23 mutations and the 1555A>G mitochondrial mutation are good candidates for electric acoustic stimulation (EAS).2012
- Author(s)
  Usami S., Miyagawa M., Nishio S., Moteki H., Takumi Y., Suzuki M., Kitano Y., Iwasaki S.
- Journal Title
  
  Acta Otolaryngol.
  
  Volume: 132 Pages: 377-384
- DOI
  10.3109/00016489.2011.649493
- NAID
  120005458478
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-21592153, KAKENHI-PROJECT-21791607, KAKENHI-PROJECT-22249057
[Journal Article] 人工内耳埋め込み術を行ったCDH23遺伝子変異による難聴症例2012
- Author(s)
  宮川麻衣子、茂木英明、工穣、宇佐美真一
- Journal Title
  
  耳喉頭頸
  
  Volume: 84 Pages: 59-63
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-22249057
[Journal Article] 人工内耳装用時期と言語発達の検討―全国多施設調査研究結果―．2012
- Author(s)
  岩崎　聡、西尾信哉、茂木英明、工　穣、笠井紀夫、福島邦博、宇佐美真一
- Journal Title
  
  Audiology Japan
  
  Volume: 55 Pages: 56-60
- NAID
  10030745026
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] TECTA mutations in Japanese with mid-frequency hearing loss affected by zona pellucida domain protein secretion2012
- Author(s)
  Moteki H, Nishio SY, Hashimoto S, Takumi Y, Iwasaki S, Takeichi N, Fukuda S, Usami S.
- Journal Title
  
  J Hum Genet
  
  Volume: 57(9) Pages: 587-92
- DOI
  10.1038/jhg.2012.73
- NAID
  10031056980
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-22249057
[Journal Article] anguage development in Japanese children who receive cochlear implant and/or hearing aid.2012
- Author(s)
  Iwasaki S, Nishio S, Moteki H, Takumi Y, Fukushima K, Kasai N, Usami S
- Journal Title
  
  Int J Pediatr Otorhinolaryngol
  
  Volume: 76 Pages: 433-8
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 乳突皮質形成による残存聴力活用型人工内耳の術後聴力への影響．2012
- Author(s)
  塚田景太、岩崎　聡、茂木英明、工　穣、宮川麻衣子、西尾信哉、宇佐美真一
- Journal Title
  
  耳喉頭頸
  
  Volume: 84 Pages: 91-5
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Language development in Japanese children who receive cochlear implant and/or hearing aid.2012
- Author(s)
  Iwasaki S, Nishio S, Moteki H, Takumi Y, Fukushima K, Kasai N, Usami S
- Journal Title
  
  Int J Pediatr Otorhinolaryngol
  
  Volume: 76 Pages: 433-438
- DOI
  10.1016/j.ijporl.2011.12.027
- NAID
  120005248086
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Patients with CDH23 mutations and the 1555A>G mitochondrial mutation are good candidates for Electric Acoustic Stimulation (EAS).2012
- Author(s)
  Usami S, Miyagawa M, Nishio S, Moteki H, Takumi Y, Suzuki M, Kitano Y, Iwasaki S.
- Journal Title
  
  Acta Otolaryngol
  
  Volume: 132 Pages: 377-84
- NAID
  120005458478
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 人工内耳装用時期と言語発達の検討―全国多施設調査研究結果―2012
- Author(s)
  岩崎　聡、西尾信哉、茂木英明、工　穣、笠井紀夫、福島邦博、宇佐美真一
- Journal Title
  
  Audiology Japan
  
  Volume: 55 Pages: 56-60
- NAID
  10030745026
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations.2011
- Author(s)
  Moteki H, Naito Y, Fujiwara K, Kitoh R, Nishio S Y, Oguchi K, Takumi Y, Usami SI
- Journal Title
  
  Acta Otolaryngol
  
  Volume: 131 Pages: 1232-1236
- DOI
  10.3109/00016489.2011.593719
- NAID
  120005458475
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-21592153, KAKENHI-PROJECT-22249057, KAKENHI-PROJECT-22591894, KAKENHI-PROJECT-22791587
[Journal Article] 残存聴力活用型人工内耳（EAS：electrical acoustic stimulation）：術後聴取能における検討．2011
- Author(s)
  茂木英明、西尾信哉、工　穣、岩崎　聡、宇佐美真一
- Journal Title
  
  Otol Jpn
  
  Volume: 21 Pages: 771-6
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 人工聴覚器の将来　人工内耳～低侵襲、残存聴力温存へ向けた新たな取り組み．2011
- Author(s)
  岩崎　聡、茂木英明、工　穣、宇佐美真一
- Journal Title
  
  日耳鼻会報
  
  Volume: 114 Pages: 801-6
- NAID
  10029834831
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Clinical profile of hearing loss in children with congenital cytomegalovirus (CMV) infection: CMV DNA diagnosis using preserved umbilical cord.2011
- Author(s)
  Furutate S, Iwasaki s, Nishio S, Moteki H, Usami S
- Journal Title
  
  Acta Otolaryngol
  
  Volume: 131 Pages: 976-782
- DOI
  10.3109/00016489.2011.583268
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-22249057, KAKENHI-PROJECT-23592477
[Journal Article] 残存聴力活用型人工内耳(electric acoustic stimulation) ～手術法と聴力保存成績について～2011
- Author(s)
  宇佐美真一、茂木英明、宮川麻衣子、内藤武彦、西尾信哉、工穣、岩崎聡
- Journal Title
  
  Otol Jpn
  
  Volume: 21(5) Pages: 763-770
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-22249057
[Journal Article] 残存聴力活用型人工内耳（EAS：electrical acoustic stimulation）～手術法と聴力保存成績について～．2011
- Author(s)
  宇佐美真一、茂木英明、宮川麻衣子、内藤武彦、西尾信哉、工　穣、岩崎　聡
- Journal Title
  
  Otol Jpn
  
  Volume: 21 Pages: 763-70
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 残存聴力活用型人工内耳（EAS:electric acoustic stimulation）の長期装用者3症例における術後成績．2011
- Author(s)
  茂木英明、西尾信哉、宮川麻衣子、工　穣、岩崎　聡、宇佐美真一
- Journal Title
  
  Audiology Japan
  
  Volume: 54 Pages: 678-85
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] 乾性耳垢と湿性耳垢の脂肪酸分析による比較．2011
- Author(s)
  岩崎　聡、鈴木宏明、茂木英明、工　穣、宇佐美真一
- Journal Title
  
  耳鼻と臨床
  
  Volume: 57 Pages: 35-41
- NAID
  130004862263
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Journal Article] Achievement of hearing preservation in the presence of an electrode covering the residual hearing region.2011
- Author(s)
  Usami S, Moteki H, Suzuki N, Fukuoka H, Miyagawa M, Nishio S, Takumi Y, Iwasaki S, Jolly C
- Journal Title
  
  Acta Otolaryngol
  
  Volume: 131 Pages: 405-12
- NAID
  120005458474
- Peer Reviewed
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] Copy Number Variants in the STRC Gene are a Common Cause of Moderate Hearing Loss in a Japanese Population2018
- Author(s)
  Hideaki Moteki, Yoh Yokota, Shin-ya Nishio, Yomomi Yamaguchi, Keiko Wakui, Shin-ichi Usami
- Organizer
  ARO Annual midwinter meeting
- Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15K10747
[Presentation] Copy number variation in the STRC gene are a common cause of moderate hearing loss in a Japanese population.2018
- Author(s)
  Moteki H, Usami S
- Organizer
  Association for Research in Otolaryngology 2018
- Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] X-linked deafness associated with large genomic deletions in POU3F4 gene2018
- Author(s)
  茂木英明
- Organizer
  第63回日本人類遺伝学会
- Data Source
  KAKENHI-PROJECT-18K09343
[Presentation] 保険収載された難聴の遺伝学的検査の現状2017
- Author(s)
  宇佐美真一、茂木英明、宮川麻衣子、西尾信哉
- Organizer
  第62回　日本人類遺伝学会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] STRC遺伝子Copy Number Variation(CNV)による感音難聴の２症例2017
- Author(s)
  横田陽、茂木英明、西尾信哉、宮崎浩充、日高浩史、大山健二、宇佐美真一
- Organizer
  第62回　日本聴覚医学会総会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] Novel Mutations in EYA4 Lead to Progressive hearing Loss2017
- Author(s)
  Shinagawa J, Moteki H, Nishio S, Usami S
- Organizer
  AAO-HNSF2017 (ENT Annual Meeting)
- Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] COL11A2遺伝子変異によるStickler症候群３型の２症例2017
- Author(s)
  岡晋一郎、岩佐陽一郎、西尾信哉、茂木英明、宇佐美真一
- Organizer
  第62回　日本聴覚医学会総会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 次世代シーケンサーにより見出されたPOU4F3遺伝子変異症例2017
- Author(s)
  北野友裕、宮川麻衣子、西尾信哉、茂木英明、野口佳裕、宇佐美真一
- Organizer
  第118回日本耳鼻咽喉科学会学術講演会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 日本人難聴患者におけるPOU3F4遺伝子変異の検討2017
- Author(s)
  杉山健二郎、茂木英明、宮川麻衣子、西尾信哉、宇佐美真一
- Organizer
  第79回　耳鼻咽喉科臨床学会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 次世代シーケンサーを用いた日本人難聴患者におけるEYA4遺伝子変異の検討2017
- Author(s)
  品川潤、茂木英明、西尾信哉、宇佐美真一
- Organizer
  第79回　耳鼻咽喉科臨床学会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 日本人難聴患者6004例におけるTECTA遺伝子変異ー難聴遺伝子データベースの解析から2017
- Author(s)
  安川梨香、平松憲、茂木英明、西尾信哉、宇佐美真一
- Organizer
  第62回　日本聴覚医学会総会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] STRC遺伝子におけるコピー数変化による難聴2017
- Author(s)
  茂木英明、横田陽、岡晋一郎、西尾信哉、、山口智美、涌井敬子、宇佐美真一
- Organizer
  第62回　日本人類遺伝学会
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 次世代シーケンサーにより見出されたPOU4F3遺伝子変異症例の臨床像2016
- Author(s)
  北野友裕、宮川麻衣子、西尾信哉、茂木英明、野口佳裕、宇佐美真一
- Organizer
  第26回日本耳科学会
- Place of Presentation
  ホテル国際21（長野市）
- Year and Date
  2016-10-05
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 次世代シーケンサーによって見出された日本人難聴患者におけるWFS1遺伝子変異の検討2016
- Author(s)
  小林正史、宮川麻衣子、茂木英明、宇佐美真一
- Organizer
  第117回　日本耳鼻咽喉科学会
- Place of Presentation
  名古屋国際会議場
- Year and Date
  2016-05-18
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 難聴に対する遺伝学的診断の検討2016
- Author(s)
  佐久間直子、茂木英明、高橋優宏、荒井康裕、西尾信哉、折舘伸彦、宇佐美真一
- Organizer
  第26回日本耳科学会
- Place of Presentation
  ホテル国際21（長野市）
- Year and Date
  2016-10-05
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] Copy Number Variants in the STRC Gene are a Common Cause of Genetic Hearing Loss in the Japanese Population.2016
- Author(s)
  Moteki H, Shearer A, Azaiez H, Booth K, Sloan C, Kolbe D, Nishio S, Smith R, Usami S.
- Organizer
  ARO 39th MidWinter Metting
- Place of Presentation
  San Diego, California, USA
- Year and Date
  2016-02-21
- Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15K10747
[Presentation] 先天性難聴の遺伝学的検査ー次世代シーケンサーの臨床応用ー2016
- Author(s)
  茂木英明、西尾信哉、宇佐美真一
- Organizer
  第26回日本耳科学会
- Place of Presentation
  ホテル国際21（長野市）
- Year and Date
  2016-10-05
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 先天性難聴の遺伝子診断における遺伝子コピー数変化（Copy Number Variation）同定の試み2016
- Author(s)
  茂木英明、宮川麻衣子、宇佐美真一
- Organizer
  第117回日本耳鼻咽喉科学会
- Place of Presentation
  名古屋国際会議場
- Year and Date
  2016-05-18
- Data Source
  KAKENHI-PROJECT-15K10747
[Presentation] 先天性難聴の遺伝子診断における遺伝子コピー数変化（Copy Number Variation）同定の試み2016
- Author(s)
  茂木英明、宮川麻衣子、宇佐美真一
- Organizer
  第117回　日本耳鼻咽喉科学会
- Place of Presentation
  名古屋国際会議場
- Year and Date
  2016-05-18
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 次世代シーケンサーを用いた日本人難聴患者におけるPOU4F3遺伝子変異の検討2016
- Author(s)
  北野友裕、宮川麻衣子、茂木英明、宇佐美真一
- Organizer
  第117回　日本耳鼻咽喉科学会
- Place of Presentation
  名古屋国際会議場
- Year and Date
  2016-05-18
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 遺伝子コピー数変化（Copy Number Variation）による先天性感音難聴症例2015
- Author(s)
  茂木英明、宇佐美真一
- Organizer
  第116回日本耳鼻咽喉科学会総会
- Place of Presentation
  東京国際フォーラム
- Year and Date
  2015-05-21
- Data Source
  KAKENHI-PROJECT-15K10747
[Presentation] Benefit of Genetic Testing for Cochlear Implantation Cadidates.2015
- Author(s)
  Usami SI, Miyagawa M, Nishio SY, Moteki H
- Organizer
  2015 Annual CORLAS Meeting
- Place of Presentation
  Palace Hotel, San Francisco USA
- Year and Date
  2015-08-25
- Int'l Joint Research
- Data Source
  KAKENHI-PROJECT-15H02565
[Presentation] 先天性サイトメガロウイルス感染に対するマススクリーニングシステム確立2013
- Author(s)
  矢野卓也、岩崎聡、西尾信哉、工穣、茂木英明、宇佐美真一
- Organizer
  第58回日本聴覚医学会・学術講演会
- Place of Presentation
  松本
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] Cytomegalovirus DNA diagnosis using preserved umbilical cord in hearing impaired children2013
- Author(s)
  Iwasaki S, Furutate S, Nishio S, Yano T, Moteki H, Usami S
- Organizer
  9th Molecular Biology of Hearing and Deafness Conference
- Place of Presentation
  Stanford University
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 信州大学における人工中耳（VibrantSoundbridge）の手術経験．2012
- Author(s)
  岩崎　聡、鈴木宏明、宮川麻衣子、茂木英明、工　穣、宇佐美真一
- Organizer
  人工内耳・中耳手術セミナー2012 IN 宮崎
- Place of Presentation
  宮崎
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 先天性サイトメガロウイルス感染症のマス・スクリーニングおよび治療法に関する研究2012
- Author(s)
  岩崎聡、茂木英明、工穣、宇佐美真一
- Organizer
  第1回耳鼻咽喉科フロンティアカンファレンス
- Place of Presentation
  旭川
- Year and Date
  2012-09-15
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 補聴手段（人工内耳装用の有無）による言語発達の相違について．2011
- Author(s)
  川端右子、西尾信哉、岩崎　聡、茂木英明、工　穣、宇佐美真一
- Organizer
  第56回日本聴覚医学会総会
- Place of Presentation
  福岡
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 人工聴覚手術症例の経験と現状について．2011
- Author(s)
  岩崎　聡、工　穣、茂木英明、宇佐美真一
- Organizer
  第73回耳鼻咽喉科臨床学会
- Place of Presentation
  松本
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 先進医療「先天性難聴の遺伝子診断」の現状2011
- Author(s)
  工穣、岩佐陽一郎、吉村豪兼、矢野卓也、内藤武彦、宮川麻衣子、茂木英明、西尾信哉、宇佐美真一
- Organizer
  第56回日本聴覚医学会総会・学術講演会
- Place of Presentation
  福岡
- Year and Date
  2011-10-27
- Data Source
  KAKENHI-PROJECT-22249057
[Presentation] 両混合性難聴に対して人工中耳埋め込み術Vibrant Soundbridgeを施行した１例．2011
- Author(s)
  鈴木宏明、岩崎　聡、茂木英明、工　穣、宇佐美真一
- Organizer
  第21回日本耳科学会総会
- Place of Presentation
  沖縄
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 人工中耳（Vibrant Soundbridge）の手術経験について．2011
- Author(s)
  岩崎　聡，鈴木宏明、宮川麻衣子、茂木英明、工　穣、宇佐美真一
- Organizer
  第21回日本耳科学会総会
- Place of Presentation
  沖縄
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 残存聴力活用型人工内耳（EAS:lectric acoustic stimulation）の長期装用者３症例における術後成績．2011
- Author(s)
  茂木英明、宮川麻衣子、西尾信哉、工　穣、岩崎　聡、宇佐美真一
- Organizer
  第56回日本聴覚医学会総会
- Place of Presentation
  福岡
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] Experience of RW coupler for round window vibroplasty of Vibrant Soundbridge.2011
- Author(s)
  Iwasaki S, Suzuki H, Moteki H, Yoshimura H, Takumi Y, Usami S
- Organizer
  APSCI 2011
- Place of Presentation
  Daegu, Korea
- Data Source
  KAKENHI-PROJECT-23592477
[Presentation] 信州大学における難聴医療の現況2011
- Author(s)
  宮川麻衣子、茂木英明、工穣、宇佐美真一
- Organizer
  第73回耳鼻咽喉科臨床学会
- Place of Presentation
  松本
- Year and Date
  2011-06-23
- Data Source
  KAKENHI-PROJECT-22249057
[Presentation] 先進医療「先天性難聴の遺伝子診断」の現状2011
- Author(s)
  工穣、岩佐陽一郎、吉村豪兼、矢野卓也、内藤武彦、宮川麻衣子、茂木英明、西尾信哉、宇佐美真一
- Organizer
  第56回日本聴覚医学会総会・学術講演会
- Place of Presentation
  福岡
- Year and Date
  2011-10-27
- Data Source
  KAKENHI-PROJECT-21592153
[Presentation] TECTA遺伝子変異による難聴患者の遺伝子解析と臨床像「優性遺伝形式をとる遺伝性難聴に関する調査研究班」2010
- Author(s)
  茂木英明、西尾信哉、橋本繁成、工穣、宇佐美真一
- Organizer
  研究成果報告会
- Place of Presentation
  東京
- Year and Date
  2010-02-28
- Data Source
  KAKENHI-PROJECT-21592153
[Presentation] TECTA遺伝子変異による難聴患者の遺伝子解析と臨床像2010
- Author(s)
  茂木英明
- Organizer
  優性遺伝形式をとる遺伝性難聴に関する調査研究」研究成果報告会
- Place of Presentation
  虎ノ門病院(東京都)
- Year and Date
  2010-02-28
- Data Source
  KAKENHI-PROJECT-21592153
[Presentation] 日本人難聴患者に見出されたTECTA遺伝子変異についての検討2008
- Author(s)
  茂木英明, 塚田景大, 橋本繁成, 浅村賢二, 工穣, 宇佐美真一
- Organizer
  第18回日本耳科学会総会
- Place of Presentation
  神戸
- Year and Date
  2008-10-17
- Data Source
  KAKENHI-PROJECT-18791199

1. USAMI Shin-ichi (10184996)

# of Collaborated Projects: 3 results

# of Collaborated Products: 44 results
2. NISHIO Shinya (70467166)

# of Collaborated Projects: 3 results

# of Collaborated Products: 54 results
3. ASAMURA Kenji (40377627)

# of Collaborated Projects: 1 results

# of Collaborated Products: 1 results
4. HASHIMOTO Shigenari (90359729)

# of Collaborated Projects: 1 results

# of Collaborated Products: 1 results
5. KUMAKAWA Kozo (40142252)

# of Collaborated Projects: 1 results

# of Collaborated Products: 0 results
6. SATO Hiroaki (40215827)

# of Collaborated Projects: 1 results

# of Collaborated Products: 0 results
7. TONO Tetsuya (80145424)

# of Collaborated Projects: 1 results

# of Collaborated Products: 0 results
8. FURUYA Nobuhiko (80107606)

# of Collaborated Projects: 1 results

# of Collaborated Products: 0 results
9. NAGAI Kyoko (50302469)

# of Collaborated Projects: 1 results

# of Collaborated Products: 0 results
10. TAKUMI Yutaka (70312501)

# of Collaborated Projects: 1 results

# of Collaborated Products: 7 results
11. MIYAKAWA Maiko (60467165)

# of Collaborated Projects: 1 results

# of Collaborated Products: 10 results
12. NAITO Takehiko (50467164)

# of Collaborated Projects: 1 results

# of Collaborated Products: 3 results
13. IWASAKI Satoshi (00232653)

# of Collaborated Projects: 1 results

# of Collaborated Products: 25 results
14. YANO Takuya (10511058)

# of Collaborated Projects: 1 results

# of Collaborated Products: 4 results
15. UJIWARA Keizo

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
16. NAKANISHI Hiroshi

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
17. KITAJIRI Shinichiro

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
18. 野口佳裕

# of Collaborated Projects: 0 results

# of Collaborated Products: 2 results
19. 山田武千代

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
20. 稲葉雄二

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
21. 内藤泰

# of Collaborated Projects: 0 results

# of Collaborated Products: 2 results
22. 菅谷明子

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
23. 菅原一真

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
24. 荒井康裕

# of Collaborated Projects: 0 results

# of Collaborated Products: 2 results
25. 松井祐興

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results
26. 喜多知子

# of Collaborated Projects: 0 results

# of Collaborated Products: 1 results

MOTEGI HIDEAKI 茂木 英明

MOTEKI Hideaki 茂木 英明

Research Projects

Research Products

Co-Researchers

Sensorineural hearing loss caused by genomic alterationPrincipal Investigator

Principal Investigator

Project Period (FY)

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Review Section

Research Institution

Copy number variations in hearing lossPrincipal Investigator

Principal Investigator

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Research Field

Research Institution

Development of high throughput sequencing analysis based system for personalized medicine of deafness

Principal Investigator

Project Period (FY)

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Research Institution

Development of the molecular diagnostic system for congenital CMV infection

Principal Investigator

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Research Field

Research Institution

Comprehensive genetic analysis of hearing loss using next generation sequencer.

Principal Investigator

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Research Institution

Mechanisms of hearing loss caused by gene mutations encoding component proteins of tectorail membrane.Principal Investigator

Principal Investigator

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Research Field

Research Institution

Mechanisms of hearing loss caused by the extra-cellar matrix

Principal Investigator

Project Period (FY)

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Research Institution

[Journal Article] Identification of a Novel Copy Number Variation of EYA4 Causing Autosomal Dominant Non-Syndromic Hearing Loss2021

Author(s)

Journal Title

DOI

Data Source

[Journal Article] Frequency and clinical features of hearing loss caused by STRC deletions2019

Author(s)

Journal Title

DOI

NAID

Data Source

[Journal Article] Comprehensive analysis of syndromic hearing loss patients in Japan.2019

Author(s)

Journal Title

DOI

Data Source

[Journal Article] Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss2019

Author(s)

Journal Title

DOI

Data Source

[Journal Article] A rational approach to identifying newborns with hearing loss caused by congenital cytomegalovirus infection by dried blood spot screening2018

Author(s)

Journal Title

DOI

Data Source

[Journal Article] Diagnostic pitfalls for GJB2-related hearing loss: A novel deletion detected by Array-CGH analysis in a Japanese patient with congenital profound hearing loss2018

Author(s)

Journal Title

DOI

Data Source

[Journal Article] WFS1 mutation screening in a large series of Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis.2018

Author(s)

MOTEGI HIDEAKI 茂木英明

MOTEKI Hideaki 茂木英明