Hearing loss is one of the most common sensory defects that affects mankind
and genetics is known to play a major role in its etiology. The otolaryngo
logist has an important stake in the progress towards a better understandin
g of the genetics of hearing loss since this research leads not only to a b
etter diagnosis, but lays the foundation for the development of new modalit
ies of treatment. A specific genetic defect can now be identified in over o
ne-half of persons with childhood onset hearing loss. This represents a con
siderable improvement in diagnostic accuracy. The importance of early and a
ccurate diagnosis relates to the avoidance of extra testing, improved genet
ic counseling and more accurate prognoses. New genes involved in hearing lo
ss are constantly being discovered. Eventually the otolaryngologist will ha
ve a diagnostic capability of close to 100%.