Investigators discuss the advantages and limitations of conventional models of the human auditory system, describe the generation and characteristics of PSC-derived inner ear organoids, and discuss several strategies and recent attempts to model hereditary deafness in vitro.
[Human Genetics]
6445212 JXMQSTSL items 1 apa 0 default asc 1 165620 https://www.stemcellsciencenews.com/wp-content/plugins/zotpress/
%7B%22status%22%3A%22success%22%2C%22updateneeded%22%3Afalse%2C%22instance%22%3A%22zotpress-a288a541e6954645b02f0ab599e30aaa%22%2C%22meta%22%3A%7B%22request_last%22%3A0%2C%22request_next%22%3A0%2C%22used_cache%22%3Atrue%7D%2C%22data%22%3A%5B%7B%22key%22%3A%22JXMQSTSL%22%2C%22library%22%3A%7B%22id%22%3A6445212%7D%2C%22meta%22%3A%7B%22creatorSummary%22%3A%22Romano%20et%20al.%22%2C%22parsedDate%22%3A%222021-08-03%22%2C%22numChildren%22%3A1%7D%2C%22bib%22%3A%22%3Cdiv%20class%3D%5C%22csl-bib-body%5C%22%20style%3D%5C%22line-height%3A%202%3B%20padding-left%3A%201em%3B%20text-indent%3A-1em%3B%5C%22%3E%5Cn%20%20%3Cdiv%20class%3D%5C%22csl-entry%5C%22%3ERomano%2C%20D.%20R.%2C%20Hashino%2C%20E.%2C%20%26amp%3B%20Nelson%2C%20R.%20F.%20%282021%29.%20Deafness-in-a-dish%3A%20modeling%20hereditary%20deafness%20with%20inner%20ear%20organoids.%20%3Ci%3EHuman%20Genetics%3C%5C%2Fi%3E.%20%3Ca%20href%3D%27https%3A%5C%2F%5C%2Fdoi.org%5C%2F10.1007%5C%2Fs00439-021-02325-9%27%3Ehttps%3A%5C%2F%5C%2Fdoi.org%5C%2F10.1007%5C%2Fs00439-021-02325-9%3C%5C%2Fa%3E%20%3Ca%20title%3D%27Cite%20in%20RIS%20Format%27%20class%3D%27zp-CiteRIS%27%20href%3D%27https%3A%5C%2F%5C%2Fwww.stemcellsciencenews.com%5C%2Fwp-content%5C%2Fplugins%5C%2Fzotpress%5C%2Flib%5C%2Frequest%5C%2Frequest.cite.php%3Fapi_user_id%3D6445212%26amp%3Bitem_key%3DJXMQSTSL%27%3ECite%3C%5C%2Fa%3E%20%3C%5C%2Fdiv%3E%5Cn%3C%5C%2Fdiv%3E%22%2C%22data%22%3A%7B%22itemType%22%3A%22journalArticle%22%2C%22title%22%3A%22Deafness-in-a-dish%3A%20modeling%20hereditary%20deafness%20with%20inner%20ear%20organoids%22%2C%22creators%22%3A%5B%7B%22creatorType%22%3A%22author%22%2C%22firstName%22%3A%22Daniel%20R.%22%2C%22lastName%22%3A%22Romano%22%7D%2C%7B%22creatorType%22%3A%22author%22%2C%22firstName%22%3A%22Eri%22%2C%22lastName%22%3A%22Hashino%22%7D%2C%7B%22creatorType%22%3A%22author%22%2C%22firstName%22%3A%22Rick%20F.%22%2C%22lastName%22%3A%22Nelson%22%7D%5D%2C%22abstractNote%22%3A%22Sensorineural%20hearing%20loss%20%28SNHL%29%20is%20a%20major%20cause%20of%20functional%20disability%20in%20both%20the%20developed%20and%20developing%20world.%20While%20hearing%20aids%20and%20cochlear%20implants%20provide%20significant%20benefit%20to%20many%20with%20SNHL%2C%20neither%20targets%20the%20cellular%20and%20molecular%20dysfunction%20that%20ultimately%20underlies%20SNHL.%20The%20successful%20development%20of%20more%20targeted%20approaches%2C%20such%20as%20growth%20factor%2C%20stem%20cell%2C%20and%20gene%20therapies%2C%20will%20require%20a%20yet%20deeper%20understanding%20of%20the%20underlying%20molecular%20mechanisms%20of%20human%20hearing%20and%20deafness.%20Unfortunately%2C%20the%20human%20inner%20ear%20cannot%20be%20biopsied%20without%20causing%20significant%2C%20irreversible%20damage%20to%20the%20hearing%20or%20balance%20organ.%20Thus%2C%20much%20of%20our%20current%20understanding%20of%20the%20cellular%20and%20molecular%20biology%20of%20human%20deafness%2C%20and%20of%20the%20human%20auditory%20system%20more%20broadly%2C%20has%20been%20inferred%20from%20observational%20and%20experimental%20studies%20in%20animal%20models%2C%20each%20of%20which%20has%20its%20own%20advantages%20and%20limitations.%20In%202013%2C%20researchers%20described%20a%20protocol%20for%20the%20generation%20of%20inner%20ear%20organoids%20from%20pluripotent%20stem%20cells%20%28PSCs%29%2C%20which%20could%20serve%20as%20scalable%2C%20high-fidelity%20alternatives%20to%20animal%20models.%20Here%2C%20we%20discuss%20the%20advantages%20and%20limitations%20of%20conventional%20models%20of%20the%20human%20auditory%20system%2C%20describe%20the%20generation%20and%20characteristics%20of%20PSC-derived%20inner%20ear%20organoids%2C%20and%20discuss%20several%20strategies%20and%20recent%20attempts%20to%20model%20hereditary%20deafness%20in%20vitro.%20Finally%2C%20we%20suggest%20and%20discuss%20several%20focus%20areas%20for%20the%20further%2C%20intensive%20characterization%20of%20inner%20ear%20organoids%20and%20discuss%20the%20translational%20applications%20of%20these%20novel%20models%20of%20the%20human%20inner%20ear.%22%2C%22date%22%3A%222021-08-03%22%2C%22language%22%3A%22en%22%2C%22DOI%22%3A%2210.1007%5C%2Fs00439-021-02325-9%22%2C%22ISSN%22%3A%221432-1203%22%2C%22url%22%3A%22https%3A%5C%2F%5C%2Fdoi.org%5C%2F10.1007%5C%2Fs00439-021-02325-9%22%2C%22collections%22%3A%5B%5D%2C%22dateModified%22%3A%222021-08-12T17%3A30%3A29Z%22%7D%7D%5D%7D
Romano, D. R., Hashino, E., & Nelson, R. F. (2021). Deafness-in-a-dish: modeling hereditary deafness with inner ear organoids. Human Genetics. https://doi.org/10.1007/s00439-021-02325-9 Cite