Investigation for the mechanism of human and animal cochlea dysfunction of PMP22 gene

• Project/Area Number: 25462630
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Otorhinolaryngology
• Research Institution: Tokyo Medical and Dental University
• Principal Investigator: TAKAHASHI MASATOKI 東京医科歯科大学, 医学部附属病院, 助教 (80401355)
• Co-Investigator(Kenkyū-buntansha): KITAMURA KEN 東京医科歯科大学, 大学院医歯学総合研究科, 特任教授 (90010470)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000); Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000); Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000); Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
• Keywords: 内耳 / マウス / 遺伝子

Outline of Final Research Achievements

Charcot-Marie-Tooth disease causes the sensorineural hearing loss regarded as cochlea dysfunction. We investigated the isolation and molecular mechanism of the PMP gene in the inner ear and cochlear dysfunction in order to figure out the mechanism　of the sensorineural hearing loss for Charcot-Marie-Tooth disease . The portion close to the outer hair cells of the animal models demonstrated intense immunohistochemical reaction by the anti-pmp antibody using confocal laser scanning microscopy and frozen section. However, we could not identify the specification of localization in the organ of Corti and the cause of hearing loss. We need to accumulate the data for analysis. We are also analyzing the data about the localization of pmp22 protein in the human cochlea.

Research Products

• [Journal Article] Delayed restoration of maximum speech discrimination scores in patients with idiopathic sudden sensorineural hearing loss (2016)
  • Author(s): Noguchi Y, Takahashi M, Ito T, Fujikawa T, Kawashima Y, Kitamura K
  • Journal Title: Auris Nasus Larynx. Volume: 43 Issue: 5 Pages: 495
  • DOI: 10.1016/j.anl.2015.12.003
  • NAID: 130005466695
  • Peer Reviewed / Open Access
• [Journal Article] 耳症状を主訴に受診したANCA関連血管炎20例の臨床像 全身型による比較検討 (2016)
  • Author(s): 川島 慶之, 野口 佳裕, 伊藤 卓, 水島 豪太, 高橋 正時, 喜多村 健, 堤 剛
  • Journal Title: 日本耳鼻咽喉科学会会報 Volume: 119 Pages: 110-117
  • NAID: 130005132689
  • Peer Reviewed
• [Journal Article] Bilateral hearing impairment as the initial symptom of sympathetic ophthalmia (2015)
  • Author(s): Yoshiyuki Kawashima, Yoshihiro Noguchi,Hiroshi Takase,Masatoki Takahashi, Shintaro Horie
  • Journal Title: American Journal of Otolaryngology Volume: In Press Issue: 4 Pages: 606-610
  • DOI: 10.1016/j.amjoto.2015.02.007
  • Peer Reviewed / Open Access
• [Journal Article] Ex vivo visualization of the mouse otoconial layer compared with micro-computed tomography. (2015)
  • Author(s): Honda K, Noguchi Y, Kawashima Y, Takahashi M, Nishio A, Kitamura K.
  • Journal Title: Otol Neurotol. Volume: 36 Issue: 2 Pages: 311-317
  • DOI: 10.1097/mao.0000000000000376
  • Peer Reviewed / Open Access
• [Journal Article] 補聴器適合検査の指針(2010)を用いた補聴器適合評価 (2015)
  • Author(s): 澤田 光毅, 野口 佳裕, 高橋 正時, 山本 桂, 吉本 亮一, 喜多村 健
  • Journal Title: Audiology Japan Volume: 58 Pages: 189-197
  • NAID: 130005097128
  • Peer Reviewed