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

Research Project

Project/Area Number 21592153
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Otorhinolaryngology
Research InstitutionShinshu University

Principal Investigator

MOTEKI Hideaki  信州大学, 医学部, 助教 (60422698)

Project Period (FY) 2009 – 2011
Project Status Completed (Fiscal Year 2011)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2011: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2010: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2009: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywords内耳 / 遺伝子 / 感音難聴 / 細胞外マトリックス
Research Abstract

One of the causes of sensory neural hearing loss is affected with genetic mutations of encoding proteins of inner ear cochlear components. In this study, we examined the prevalence of genetic mutations and confirmed genotype phenotype correlation in congenital hearing loss patients. In our results, the mutation of TECTA gene encoding tectorial membrane protein may be a high incidence in autosomal dominant hearing loss. Mutation screening can diagnose the genetic cause of hearing loss with tectorial membrane defect.

Report

(4 results)
  • 2011 Annual Research Report   Final Research Report ( PDF )
  • 2010 Annual Research Report
  • 2009 Annual Research Report
  • Research Products

    (5 results)

All 2012 2011 2010

All Journal Article (2 results) (of which Peer Reviewed: 2 results) Presentation (3 results)

  • [Journal Article] Patients with CDH23 mutation 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 Issue: 4 Pages: 377-384

    • DOI

      10.3109/00016489.2011.649493

    • NAID

      120007110630

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations2011

    • Author(s)
      Moteki H, Naito Y, Fujiwara K, Kitoh R, Nishio S Y, Oguchi K, Takumi Y, Usami SI
    • Journal Title

      Acta Oto-Laryngol

      Volume: 131 Issue: 11 Pages: 1232-1236

    • DOI

      10.3109/00016489.2011.593719

    • NAID

      120007110633

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [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
    • Related Report
      2011 Annual Research Report
  • [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
    • Related Report
      2011 Final Research Report
  • [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
    • Related Report
      2009 Annual Research Report

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Published: 2009-04-01   Modified: 2016-04-21  

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