Abstract
This review addresses the advances of our molecular understanding of hearing and how
this benefits the hearing impaired. Classical biochemical methods usually fall short
in contributing to the analysis of the molecular mechanisms of hearing e. g. in the
cochlea, the auditory part of the inner ear, due to the scarcity of the cells of interest.
Genetics, molecular cell biology, and physiology, on the other hand, have elucidated
the intricate molecular and cellular mechanisms that bring about the outstanding performance
of the auditory system. Many of those mechanisms are quite unique and specialized
to serve the specific needs of hearing. Hence, their defects often spare other organs
and lead to specific non-syndromic deafness. High throughput sequencing can reveal
causes of sporadic deafness when combined with careful bioinformatics. Molecular approaches
are also helpful for understanding more common forms of hearing impairment such as
noise-induced hearing impairment. While molecular therapies are not yet clinically
available, careful molecular genetic analysis helps to counsel the hearing impaired
subjects.
Key words
Cochlea - molecular mechanism - animal model - genetics - deafness - therapy
