Juvenile bilateral sensorineural hearing loss

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  • Takahashi Masahiro
    Department of Otorhinolaryngology, International University of Health and Welfare, Mita Hospital
  • Iwasaki Satoshi
    Department of Otorhinolaryngology, International University of Health and Welfare, Mita Hospital
  • Furutate Sakiko
    Department of Otorhinolaryngology, International University of Health and Welfare, Mita Hospital
  • Oka Shin-ichiro
    Department of Otorhinolaryngology, International University of Health and Welfare, Mita Hospital
  • Nishio Shin-ya
    Department of Hearing Implant Science, Shinshu University School of Medicine
  • Usami Shin-ichi
    Department of Hearing Implant Science, Shinshu University School of Medicine

Bibliographic Information

Other Title
  • 若年発症型両側性感音難聴
  • ジャクネン ハッショウガタ リョウガワ セイカンオン ナンチョウ

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Description

<p>Among patients with idiopathic bilateral sensorineural hearing loss, seven causative genes that develop late-onset hearing loss under the age of 40 were identified and defined as juvenile onset bilateral sensorineural hearing loss; this hearing loss is clearly different from age-related hearing loss. The genes are ACTG1, CDH23, COCH, KCNQ4, TECTA, TEMPRSS3, and WFS1. If the patients have severe to profound hearing loss (70 dB or more), they can be extrapolated as intractable diseases. For patients with juvenile onset bilateral sensorineural hearing loss, genetic testing with next-generation sequencers and genetic counseling are accompanied with autonomous options ranging from hearing aids to hearing implants, which has become very beneficial.</p>

Journal

  • Otology Japan

    Otology Japan 31 (2), 137-141, 2021

    Japan Otological Society

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