Improving the Performance of Cochlear Implants for DFNB8/10 by accompanying Gene Therapy

通过伴随基因治疗提高 DFNB8/10 人工耳蜗的性能

• 批准号: 518273664
• 负责人: Professorin Dr. Ellen Reisinger
• 金额: --
• 依托单位: Universitätsklinik für Hals-, Nasen- und Ohrenheilkunde
• 依托单位国家: 德国
• 项目类别: Research Grants
• 资助国家: 德国
• 项目状态: 未结题
• 来源: https://gepris.dfg.de/gepris/projekt/518273664?language=en
• 关键词: Improving; Performance; Cochlear; Implants; DFNB8

In cases of profound hearing loss, cochlear implantation (CI) can restore hearing to a level where most people can understand speech in everyday life. Electrodes on the CI electrically stimulate and thus activate spiral ganglion neurons (SGN), the first neurons of the auditory pathway. Therefore, intact SGN and a functioning auditory pathway are absolutely necessary for good CI performance. In the case of mutations in the gene TMPRSS3, which cause the recessively inherited hearing loss DFNB8/10, electrical stimulation evokes lower SGN responses. Cochlear implantation in childhood resulted in good hearing with CI, but the outcome in adults was highly variable. This is in line with the observation that a drastic reduction in SGN cell bodies was observed in the mouse model. My research project aims at deciphering the reason why CIs performance varies and declines in patients with mutations in the TMPRSS3 gene causing DFNB8/10. Subsequently, I aim to develop a gene therapy to improve CI performance in animal models for this form of hearing loss.

在严重听力损失的情况下，人工耳蜗植入（CI）可以将听力恢复到大多数人可以理解日常生活中的语言的水平。CI上的电极电刺激并因此激活螺旋神经节神经元（SGN），听觉通路的第一神经元。因此，完整的SGN和功能正常的听觉通路对于良好的CI性能是绝对必要的。在基因TMPRSS 3突变的情况下，这会导致复发性遗传性听力损失DFNB 8/10，电刺激引起较低的SGN反应。儿童期耳蜗植入术可使CI患者获得良好的听力，但成人的结果差异很大。这与在小鼠模型中观察到SGN细胞体急剧减少的观察结果一致。 我的研究项目旨在破译为什么CI的性能变化和TMPRSS 3基因突变导致DFNB 8/10患者的下降的原因。随后，我的目标是开发一种基因疗法，以改善这种形式的听力损失的动物模型中的CI性能。