Fig. 2.

Graphical representation of the generation and analysis of PSC-derived inner ear organoid models of hereditary deafness. a Patient-specific iPSCs with mutation in deafness-related gene (X) are generated from somatic cells through induced expression of pluripotency genes. b Precision genome editing is used to create targeted, deafness-related mutations in ESCs. c The coding sequence of a fluorophore (e.g., tdTomato) is inserted downstream of the promoter for a gene-of-interest (e.g., deafness-related gene, cell type-specific gene, or regionally expressed transcription factor) to generate a fluorescent reporter PSC line. d Fluorescent cells within organoids can be selectively harvested, either manually or automatically (e.g., by FACS), for further analyses. ESCs embryonic stem cells, FACS fluorescence-activated cell sorting, iPSC induced pluripotent stem cell