The team will now test whether the technique works with the human stereocilin gene and test it in a human inner ear cells in a dish, derived from patients with STRC hearing loss. The technique could also be used in other situations where the therapeutic gene is very large, says Jeffrey Holt, PhD, a scientist in the departments of Otolaryngology and Neurology at Boston Children’s and the study’s senior investigator.
Findings were published December 15 in the journal Science Advances. A first-of-its-kind gene therapy technique developed at Boston Children’s Hospital successfully replaced the mutated protein, stereocilin, in the inner ear and reversed severe hearing loss in mice - sometimes to normal levels of hearing. Hearing loss has been linked to mutations in at least 100 different genes, but up to 16 percent of genetic hearing loss can be traced to just one gene, STRC, the second most common genetic cause. view moreĬredit: Carl Nist-Lund, Holt/Géléoc Lab, Boston Children’s Hospital The red arrows point out the restored stereocilin cross-links, which stand the hair cell microvilli up into organized bundles that can contact the overlying tectorial membrane and detect sound vibrations. A neighboring hair cell, at right, took up the dual-AAV gene therapy vector and has its normal organization restored. The image at left shows a disorganized sensory hair cell from the inner ear from a mouse with a mutation in the Strc gene as a result, the cell is missing scaffolding links provided by the stereocilin protein. Published in Frontiers in Cell and Developmental Biology ISSN 2296-634X (Online) Publisher Frontiers Media S.A.Image: This scanning electron micrograph shows sensory outer hair cells, which are required for cochlear amplification and normal hearing. With the development of related research, stem cell therapy will play a greater role in the treatment of diseases related to the inner ear.
At present, stem cell therapy still has limitations, but the role of this technology in the treatment of hearing diseases has been widely recognized. This article describes the methods for inducing the differentiation of endogenous and exogenous stem cells, the implantation operation and regulation of exogenous stem cells after implanted into the inner ear, and it elaborates the relevant signal pathways of stem cells in the inner ear, as well as the clinical application of various new materials. A number of studies have proved that the transplantation of neural stem cells in multiple organs has an important therapeutic effect on activation and regeneration of cells, and restore damaged neurons. In recent years, neural stem cell transplantation has received widespread attention as a new treatment method for supplementing specific cells damaged by disease, such as neurodegenerative diseases. Zuhong He Department of Otorhinolaryngology-Head and Neck Surgery, Zhongnan Hospital of Wuhan University, Wuhan, China Yanyan Ding Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China Yurong Mu Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China Xiaoxiang Xu Department of Otorhinolaryngology-Head and Neck Surgery, Zhongnan Hospital of Wuhan University, Wuhan, China Weijia Kong Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China Renjie Chai State Key Laboratory of Bioelectronics, Jiangsu Province High-Tech Key Laboratory for Bio-Medical Research, School of Life Sciences and Technology, Southeast University, Nanjing, China Renjie Chai Co-Innovation Center of Neuroregeneration, Nantong University, Nantong, China Renjie Chai Institute for Stem Cell and Regeneration, Chinese Academy of Sciences, Beijing, China Renjie Chai Jiangsu Province High-Tech Key Laboratory for Bio-Medical Research, Southeast University, Nanjing, China Renjie Chai Beijing Key Laboratory of Neural Regeneration and Repair, Capital Medical University, Beijing, China Xiong Chen Department of Otorhinolaryngology-Head and Neck Surgery, Zhongnan Hospital of Wuhan University, Wuhan, China DOI Journal volume & issue
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