瓦氏综合征（WS）伴随螺旋神经节(SGN)退变影响人工耳蜗(CI)术后效果，是公认的世界性医学难题。SGN再生是该领域急需突破的技术瓶颈。我课题组发现干细胞协同CI移植可增效改善荣昌猪（WS大动物疾病模型）听功能。遂提出“场电位可诱导干细胞到SGN富集，SGN微环境及场电位双重作用完美模拟了听神经发育的初始状态，促使干细胞按合理比例分化，补充并重建突触连接，形成具有声音编辑及传导功能的成熟SGN神经网络”这一关键科学假设。本研究拟借助体内细胞互作及单细胞测序技术，比较有/无干细胞及有/无场电位条件下，WS模型猪CI术后听觉改善、SGN数目、类型、分化、功能及转录方式等相关指标，从分子水平到系统功能全方位验证及解析上述假设。该研究将对干细胞协同CI治疗SGN缺陷型感音神经性聋产生深远影响，潜在受益人群远不止WS患者。此外CI及干细胞移植均为成熟技术，可随时对接临床，科学意义及应用前景巨大。

The poor hearing effect after cochlear implantation (CI) in patients with hearing loss due to Waardenburg syndrome (WS) that features a spiral ganglion neuron (SGN) lesion, is a worldwide recognized medical problem. Regarding solving the problem, SGN regeneration is a bottleneck with technical issues. To tackle these urgent issues, our research team tried for many years to find a solution by using Rongchang pig model (WS animal disease model) and found that stem cell synergy with CI can improve the hearing function in these hearing-impaired pigs. We proposed that CI field potential can induce an enrichment of stem cell differentiation into SGN, this might be due to the fact that a SGN microenvironment and field potential perfectly mimic the initial state of auditory nerve development. In such a state, the stem cells differentiated in an adequate proportion, newly produced SGNs re-established synaptic connections and formed sound processing and conducting mature SGN neural network. The key scientific hypothesis, however, needs further experimental evidence. The planned study intends to use the in vivo cell interaction and single cell sequencing technology to compare, between different situations, i.e., with and without stem cells, presence and absence of field potential, the hearing improvement as well as SGN differentiation outcomes (number, type, function and transcriptional pattern) in the WS pig model. The project could, from the molecular level to the system function, fully verify the above assumptions. This study project will have a profound impact on future application of stem cell integrated to CI for treatment of SGN-deficient sensorineural hearing loss, and the beneficiaries could be far more than WS patients. In addition, CI and stem cell transplantation are mature technologies, which have great, immediate clinical usefulness with tremendous scientific significance and practical prospect.