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高度内耳・内耳道奇形は中枢聴覚神経回路も障害しうるか:機能検査を用いた多角的評価

Research Project

Project/Area Number 25861607
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Otorhinolaryngology
Research InstitutionKyoto University

Principal Investigator

山崎 博司  京都大学, 医学(系)研究科(研究院), 医員 (80536243)

Project Period (FY) 2013-04-01 – 2015-03-31
Project Status Discontinued (Fiscal Year 2014)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Keywords人工内耳 / 内耳奇形 / 聴性脳幹反射 / 聴性脳幹反応
Outline of Annual Research Achievements

内耳・内耳道奇形は先天性高度難聴の約3割を占める。その中で高度な奇形に分類されるIncomplete partition type 1(IP-1), common cavity、蝸牛神経低形成(CND)に注目し、聴覚神経回路の機能を人工内耳手術時にelectrically evoked ABR(EABR)を用いて電気生理学的に評価した。
IP-1では、3例中5例で明瞭なeIII, eVを認め、その振幅と潜時は奇形の無い対照群と有意差を認めなかった。この3例の人工内耳装用効果は極めて良好で、IP-1の過半数で聴覚神経回路の形成は対照群と同等であることが示唆された。
Common cavityでは、奇形内耳の前下方に位置する電極刺激でのみeVが検出された。対象群と比較すると、eVの振幅は小さいものの、潜時に関しては有意差は無かった。しかし、EABRの波形は全例で非典型で、多くの症例で明確なeIIIを認めなかった。これらの結果は、common cavityを伴う症例では、(1)蝸牛神経遠位端は発生学的に蝸牛に相当する奇形内耳の前下方に分布する、(2)内耳より中枢側の神経繊維の機能はある程度保たれているものの、対照群やIP-1とは異なることを示唆している。
CNDを伴う症例では、聴覚神経回路を賦活するために対照群よりも大きな電流を要する。そのため、EABRの波形が非典型でかつ筋原性の反応が混入することが多い。申請者は、(1)刺激電化依存性の振幅増加を確認、(2)症例によっては全身麻酔下で筋弛緩剤を使用することで、神経原性反応と筋原性の反応を区別し、正確にeVを評価した。その結果、CNDではほぼ全例でEABRの波形が非典型であった。しかしEABRでeVを認めるものは人工内耳装用効果が比較的良好であることから、電気生理学的な機能評価が人工内耳予後予測に有用であることを見いだした。

Report

(2 results)
  • 2014 Annual Research Report
  • 2013 Research-status Report
  • Research Products

    (15 results)

All 2015 2014 2013

All Journal Article (9 results) (of which Peer Reviewed: 7 results,  Open Access: 1 results) Presentation (6 results)

  • [Journal Article] Usefulness of MRI and EABR testing for predicting CI outcomes immediately after cochlear implantation in cases with cochlear nerve deficiency2015

    • Author(s)
      Yamazaki H, Jaime L, Briggs R, Naito Y.
    • Journal Title

      Otology and Neurotology

      Volume: in press

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] SLC26A4 p.Thr410Met homozygous mutation in a patient with a cystic cochlea and an enlarged vestibular aqueduct showing characteristic features of incomplete partition type I and II.2014

    • Author(s)
      Yamazaki H, Naito Y, Moroto S, Tamaya R, Yamazaki T, Fujiwara K, Ito J
    • Journal Title

      Int J Pediatr Otorhinolaryngol

      Volume: 78 Issue: 12 Pages: 2322-2326

    • DOI

      10.1016/j.ijporl.2014.10.038

    • NAID

      120005537592

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] EABR-based evaluation of the spatial distribution of auditory neuronal tissue in common cavity deformities2014

    • Author(s)
      Yamazaki H, Naito Y, Fujiwara K, Moroto S, Yamamoto R, Yamazaki T, Sasaki I.
    • Journal Title

      Otology and Neurotology

      Volume: 35 Issue: 8 Pages: 1394-402

    • DOI

      10.1097/mao.0000000000000375

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Comprehensive analysis of cochlear implant failure: usefulness of clinical symptom-based algorithm combined with in situ integrity testing2014

    • Author(s)
      Yamazaki H, O’Leary S, Moran M, Briggs, R.
    • Journal Title

      Otology and Neurotology

      Volume: 35 Issue: 4 Pages: 605-612

    • DOI

      10.1097/mao.0000000000000267

    • Related Report
      2014 Annual Research Report 2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Intra-arterial administration of antibiotics for refractory skull base osteomyelitis2014

    • Author(s)
      Yamazaki H, Kikuchi M, Shinohara S, Naito Y, Fujiwara K, Kanazawa Y, Tona R
    • Journal Title

      Auris Nasus Larynx

      Volume: in press Issue: 4 Pages: 380-383

    • DOI

      10.1016/j.anl.2013.12.003

    • Related Report
      2014 Annual Research Report 2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Clinical features of rapidly progressive bilateral sensorineural hearing loss2014

    • Author(s)
      Kishimoto I, Yamazaki H, Naito Y, Shinohara S, Fujiwara K, Kikuchi M, Kanazawa Y, Tona R, Harada H
    • Journal Title

      Acta Otolaryngol

      Volume: 134(1) Issue: 1 Pages: 58-65

    • DOI

      10.3109/00016489.2013.831993

    • Related Report
      2014 Annual Research Report 2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 悪性外耳道炎の診断と治療2014

    • Author(s)
      山崎博司
    • Journal Title

      耳鼻咽喉科・頭頸部外科

      Volume: 86 Pages: 602-607

    • Related Report
      2014 Annual Research Report
  • [Journal Article] 悪性外耳道炎2014

    • Author(s)
      山崎博司
    • Journal Title

      エントーニ

      Volume: 159 Pages: 38-45

    • Related Report
      2013 Research-status Report
  • [Journal Article] EABR-based evaluation of the spatial distribution of auditory neuronal tissue in common cavity deformities2014

    • Author(s)
      Yamazaki H, Naito Y, Fujiwara K, Moroto S, Yamamoto R, Yamazaki T, Sasaki I
    • Journal Title

      Otology & Neurolology

      Volume: in press

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Presentation] Evaluation of cerebral hemodynamic responses during phonological working memory tasksusing functional near-infrared spectroscopy (fNIRS)2014

    • Author(s)
      Hiroshi Yamazaki, Yuji Kanazawa, Harukazu Hiraumi, Juichi Ito
    • Organizer
      Inner ear biology in Kyoto
    • Place of Presentation
      Kyoto Japan
    • Year and Date
      2014-11-02 – 2014-11-04
    • Related Report
      2014 Annual Research Report
  • [Presentation] Usefulness of MRI and EABR testing for predicting CI outcomes immediately after cochlear implantation in cases with cochlear nerve deficiency2014

    • Author(s)
      Hiroshi Yamazaki, Jaime Leigh, Robert Briggs, Yasushi Naito, Sabro Moroto, Rinko Yamamoto, Tomoko Yamazaki, Juichi Ito
    • Organizer
      EAONO 7th instruction workshop
    • Place of Presentation
      Siena, Italy
    • Year and Date
      2014-09-13 – 2014-09-16
    • Related Report
      2014 Annual Research Report
  • [Presentation] Different CI-mediated auditory brainstem responses observed in different types of the severe inner ear malformations2014

    • Author(s)
      Hiroshi YAMAZAKI, Yasushi NAITO, Saburo MOROTO, Rinko YAMAMOTO, Tomoko YAMAZAKI, Ichiro SASAKI, Juichi ITO
    • Organizer
      13th International Conference on Cochlear Implants and Other Implantable Auditory Technologies
    • Place of Presentation
      Munich, Germany
    • Year and Date
      2014-06-18 – 2014-06-21
    • Related Report
      2014 Annual Research Report
  • [Presentation] SLC26A4 p.T410M homozygous mutation found in a cystic cochlea with an enlarged vestibular aqueduct which is different from IP-II with respect to the lack of a bony modiolus2014

    • Author(s)
      Yamazaki H
    • Organizer
      ARO 2014 MidWinter Meeting
    • Place of Presentation
      San Diego, CA, USA
    • Related Report
      2013 Research-status Report
  • [Presentation] Cochlear Implant Outcomes in Cases with Cochlear Nerve Deficiency (CND)2013

    • Author(s)
      Yamazaki H
    • Organizer
      the 20th World Congress of the International Federation of Oto-Rhino-Laryngological Societies (IFOS)
    • Place of Presentation
      Seoul, Korea
    • Related Report
      2013 Research-status Report
  • [Presentation] Cochlear nerve deficiency症例におけるCT所見と術中EABR検査結果の検討2013

    • Author(s)
      山崎博司
    • Organizer
      第23回日本耳科学会総会・学術講演会
    • Place of Presentation
      宮崎 シーガイアコンベンションセンター
    • Related Report
      2013 Research-status Report

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Published: 2014-07-25   Modified: 2019-07-29  

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