http://www.soreeclinic.com/ear_center/hearing/GJB2.pdf
2/3 have a severe HL, 1/3 are profoundly deaf. Less than 3% are moderately HOH. Lots of good information.
Deaf Genetics Project
Two-thirds of people with Connexin 26-related deafness will have audiograms that fall into the profound-to-severe range, but there is quite a bit of variability in the range of hearing loss, including audiograms that fall into the mild and moderate range, and unilateral deafness, even among family members with the same genetic cause of deafness.
http://arep.med.harvard.edu/pdf/Aging/Izumikawa05.pdf
Should stem cells fail to improve my hearing, I will be tested for genetic causes of deafness and wait for genetic therapy to extend to humans. Proof of concept has already been established. I think there's a good chance many who have a genetic basis for their hearing loss can at least achieve partial improvement with stem cells. After all, two people with the same genetic defect can have different audiograms. Person A could get stem cells and have his hearing improved so it's similar to person B who has better hearing to begin with.
