Zhang Jiajia, Guo Zengzhi, Pan Changjie, Hu Chunchun, Weng Xinyang, Liu Yang-Wenyi, Cheng Xiaoting, Lv Jun, Cao Qi, Wang Hui, Chen Yuxin, Wang Daqi, Hu Shaowei, Xun Mengzhao, Zhang Longlong, Wang Zijing, Tang Honghai, Zhu Biyun, Guo Luo, Yu Sha, Hu Xiaoling, Chen Lin, Chen Bing, Chen Zheng-Yi, Sun Shan, Xu Xiu, Li Huawei, Chen Fei, Shu Yilai
ENT institute and Department of Otorhinolaryngology, Eye & ENT Hospital, Fudan University, Shanghai, China.
NHC Key Laboratory of Hearing Medicine Research and MOE Engineering Research Center of Gene Technology, Fudan University, Shanghai, China.
Nat Hum Behav. 2025 May 2. doi: 10.1038/s41562-025-02184-8.
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.
接受基因治疗的先天性耳聋患者是一个独特的群体，他们经历了听力恢复和语言发展。然而，目前尚不清楚基因治疗如何导致与听力相关的皮层发生变化。在此，我们在为期六个月的随访期间，使用功能近红外光谱和脑电图对10名患者的神经处理情况进行了研究。患者在听音乐时，听觉皮层的激活增强，尤其是在颞叶顶枕区的部分区域。在听语音时，右侧颞前叶和左侧颞叶顶枕区的激活也增强。脑电图数据显示，基因治疗后，静息态脑电图β频段在时间点T2和T3的功率在统计学上显著增加，且T2和T3时的失配负波幅值在统计学上显著高于T0时。基因治疗后患者的智力发育水平也有所提高。这些初步研究结果揭示了基因治疗的神经和认知效应，支持了其在听觉和智力发育方面的潜在有效性。
