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<p>Individuals with congenital deafness that have received gene therapy represent a unique group who experience hearing recovery and speech development. However, it is unclear how hearing-related cortex changes because of gene therapy. Here we study neural processing in ten patients using functional near-infrared spectroscopy and electroencephalography during a six-month follow-up period. Patients showed an enhancement of activation in the auditory cortex, particularly in parts of the Sylvian parietotemporal area while listening to music. Activation in the right anterior temporal lobe and left Sylvian parietotemporal area was also enhanced when listening to speech. The electroencephalography data showed that the power of the resting-state electroencephalography beta band at time points T2 and T3 was statistically significantly increased after gene therapy, and mismatch negativity amplitudes at T2 and T3 were statistically significantly higher than those at T0. The mental developmental level of the patients also increased after gene therapy. These preliminary findings illuminate the neural and cognitive effects of gene therapy, supporting its potential effectiveness in auditory and mental development.</p>

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Preliminary evidence for enhanced auditory cortex activation and mental development after gene therapy in children with autosomal recessive deafness 9

  • 期刊论文

  • 发表日期：

    2025年5月2日

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  • Jiajia Zhang,
  • Zengzhi Guo,
  • Changjie Pan,
  • Chunchun Hu,
  • Xinyang Weng,
  • Yang-wenyi Liu,
  • Xiaoting Cheng,
  • Jun Lv,
  • Qi Cao,
  • Hui Wang,
  • Yuxin Chen,
  • Daqi Wang,
  • Shaowei Hu,
  • Mengzhao Xun,
  • Longlong Zhang,
  • Zijing Wang,
  • Honghai Tang,
  • Biyun Zhu,
  • Luo Guo,
  • Sha Yu,
  • Xiaoling Hu,
  • Lin Chen,
  • Bing Chen,
  • Zheng-Yi Chen,
  • Shan Sun,
  • Xiu Xu,
  • Huawei Li,
  • Fei Chen,
  • Yilai Shu

摘要

Individuals with congenital deafness that have received gene therapy represent a unique group who experience hearing recovery and speech development. However, it is unclear how hearing-related cortex changes because of gene therapy. Here we study neural processing in ten patients using functional near-infrared spectroscopy and electroencephalography during a six-month follow-up period. Patients showed an enhancement of activation in the auditory cortex, particularly in parts of the Sylvian parietotemporal area while listening to music. Activation in the right anterior temporal lobe and left Sylvian parietotemporal area was also enhanced when listening to speech. The electroencephalography data showed that the power of the resting-state electroencephalography beta band at time points T2 and T3 was statistically significantly increased after gene therapy, and mismatch negativity amplitudes at T2 and T3 were statistically significantly higher than those at T0. The mental developmental level of the patients also increased after gene therapy. These preliminary findings illuminate the neural and cognitive effects of gene therapy, supporting its potential effectiveness in auditory and mental development.