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Analysis of pathology in usher syndrome by method of molecular biology.

Research Project

Project/Area Number 14571613
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Otorhinolaryngology
Research InstitutionHAMAMATSU UNIVERSITY SCHOOL OF MEDICINE

Principal Investigator

IWASAKI Satoshi  Hamamatsu University, School of Medicine Dep. Of Otolaryngology, Assistant Professor, 医学部附属病院, 講師 (00232653)

Co-Investigator(Kenkyū-buntansha) HOTTA Yoshihiro  Hamamatsu University, School of medicine Dep. Of ophthalmology, Professor, 医学部, 教授 (90173608)
Project Period (FY) 2002 – 2003
Project Status Completed (Fiscal Year 2003)
Budget Amount *help
¥2,800,000 (Direct Cost: ¥2,800,000)
Fiscal Year 2003: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 2002: ¥1,900,000 (Direct Cost: ¥1,900,000)
KeywordsRetinitis Pigmentosa / Hearing impairment / Tinnitus / Vertigo / Usher syndrome / Epidemiology / Phenotype / 耳鳴 / 遺伝子表現型 / 視聴覚障害 / 疫学調査 / 分子生物学
Research Abstract

The prevalence of hearing loss, progressive hearing loss, tinnitus and dizziness, and age at onset of hearing loss were evaluated by questionnaire in subjects who were registered members of the Japanese Retinitis Pigmen tosa Society (n=3200) in the year 2002. 834 questionnaires were returned (26.1% response rate), but only 828 subjects (25.9%) who had been diagnosed with Retinitis Pigmentosa (RP) by an ophthalmologist were included in the analysis. The prevalence of cochlear symptoms (hearing loss: 29.5%, tinnitus 30.4%) was 43.0% (n=356). Of 356 subjects with cochlear symptoms, progressive hearing loss and dizziness were 44.9% and 56.5%, respectively. The mean ages at onset of hearing loss and visual loss were 39.2 years old and 31.7 years old, respectively. There was no significant difference in progressive hearing loss among subjects with different ages of hearing loss on set. 63.5% of the subjects with cochlear symptoms had a history of audiometric examination, and 22.1% of the subjects with hearing loss (n=244) used a hearing aid. Overall, the rate of occurrence of cochlear symptoms was calculated to be 7.0 per 100,000 in the general population (where subjects with an age at onset of cochlear symptom of 61 or older were excluded from the calculation). There is a difference in prevalence rate of cochlear symptonis with RP according to place of residence. In Hokkaido (in the most northerly part of Japan) the rate is lowes t (4.7/100,000), whereas it is highest in Kyushu (8.5/100,000). In order to achieve accurate diagnosis of this disease, studies must be performed to correlate clinical phenotypes with genotypes for the majority of patients.

Report

(3 results)
  • 2003 Annual Research Report   Final Research Report Summary
  • 2002 Annual Research Report
  • Research Products

    (12 results)

All Other

All Publications (12 results)

  • [Publications] 岩崎 聡 他: "網膜色素変性症患者における聴覚障害の現状-全国アンケート調査結果-"Otology Japan. 13. 315 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Iwasaki Satoshi et al.: "A model of two-stage newborn hearing screening with automated auditory brainstem response."International Journal of Pediatric Otorhinolaryngology. 67. 1099-1104 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Takeshita T, Iwasaki S, et al.: "Photochemically induced double lateral wall lesions in the guinea pig cochlea."Acta Oto-Laryngol. 123. 355-361 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Iwasaki Satoshi et al.: "Association of clinical features with mutation of TECTA in an autosomal dominant hearing loss family."Arch Otolaryngol. 128. 913-917 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 中野善達 監訳, 岩崎 聡 他共著: "聾・聴覚障害百科事典"明石書店. 1-343 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Iwasaki Satoshi, Nagura M, et al.: "Prevalence of hearing loss with retinitis pigmentosa"Otology Japan. 13. 315 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Iwasaki Satoshi, Hayashi Y, et al.: "A model of two-stage newborn hearing screening with automated auditory brainstem response."International Journal of Pediatric Otorhinolaryngology. 67. 1099-1104 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Takeshita T, Iwasaki S, et al.: "Photochemically induced double lateral wall lesions in the guinea pig cochlea."Acta Oto-Laryngol. 123. 355-361 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Iwasaki Satoshi, Harada D, et al.: "Association of clinical features with mutation of TECTA in an autosomal dominant hearing loss family."Arch Otolaryngol. 128. 913-917 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 岩崎 聡 他: "網膜色素変性症患者における聴覚障害の現状-全国アンケート調査結果-"Otology Japan. 13. 315 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Iwasaki Satoshi et al.: "A model of two-stage newborn hearing screening with automated auditory brainstem response"International Journal of Pediatric Otorhinolaryngology. 67. 1099-1104 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Takeshita T, Iwasaki S, et al.: "Photochemically induced double lateral wall lesions in the guinea pig cochlea"Acta Oto-Laryngol. 123. 913-917 (2003)

    • Related Report
      2003 Annual Research Report

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Published: 2002-04-01   Modified: 2016-04-21  

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