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Summary: MicroRNAs and epigenetic regulation in the mammalian inner
ear: implications for deafness
Lilach M. Friedman · Karen B. Avraham
Received: 17 June 2009 / Accepted: 30 September 2009 / Published online: 30 October 2009
Ó Springer Science+Business Media, LLC 2009
Abstract Sensorineural hearing loss is the most common
sensory disorder in humans and derives, in most cases,
from inner-ear defects or degeneration of the cochlear
sensory neuroepithelial hair cells. Genetic factors make a
significant contribution to hearing impairment. While
mutations in 51 genes have been associated with hereditary
sensorineural nonsyndromic hearing loss (NSHL) in
humans, the responsible mutations in many other chro-
mosomal loci linked with NSHL have not been identified
yet. Recently, mutations in a noncoding microRNA
(miRNA) gene, MIR96, which is expressed specifically in
the inner-ear hair cells, were linked with progressive
hearing loss in humans and mice. Furthermore, additional
miRNAs were found to have essential roles in the devel-
opment and survival of inner-ear hair cells. Epigenetic
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