ER stress has been linked to the pathogenesis of neurodegenerative diseases, such as Alzheimer's disease and Parkinson's disease, as well as to the pathogenesis of cell death, such as renal tubule lesions in diabetes.21, 22, 23 In zebrafish mutants, the defective CDH23 proteins in hair cells failed to be transferred and contributed to the formation of Usher protein complexes, therefore leading to ER stress.15 Taken together, these results offered us new insight into the pathological mechanisms of and potential therapies for sensorineural deafness. This evidence concerns the gene CDH23 and early-onset autosomal dominant Alzheimer disease.