Researchers at Juntendo University report in the journal
Human Molecular Genetics that a stem-cell technique may be used to develop a treatment of a common mutation-related type of deafness. The method involves reproducing cells occurring in the human inner ear where the researchers emphasize
that 'the pathological condition could be reproduced with iPS derived from a typical patient.'
Illustration of the research described in this paper.
Deafness is the most common sensory impairment in newborn children — about 1 child in 1000 is born with hearing loss, or develops it in early childhood. Half of these cases have a genetic cause; very often, this type of deafness is related to a mutation of a gene called 'gap junction beta 2 (GJB2)', which encodes a protein called 'connexin 26 (CX26)'. This protein occurs in cells in the cochlea, the part of the inner ear enabling hearing. One way of treating GJB2-related deafness would be to reproduce properly functioning cochlear cells and introduce them into the inner ear. Now, Kazusaku Kamiya from Juntendo University and colleagues have made an important step forward toward making this treatment possible: using a stem-cell technique, they succeeded in reproducing cochlear supporting cells, a type of cell known to contain CX26 proteins.