The ototoxic effects of aminoglycoside antibiotics are well known. How
ever, a molecular and cellular mechanism for the death of cochlear hai
r cells has remained difficult to prove. Human genetic studies have sh
own that a rare trait for hypersensitivity to aminoglycosides is confe
rred by mitochondrial genetic variation. Recently, a gene involved has
been identified as the mitochondrial small ribosomal RNA gene, consis
tent with the known mechanism of aminoglycoside action against bacteri
a. We used the existing data as a basis for our hypothesis of a molecu
lar and cellular model for aminoglycoside ototoxicity that is describe
d in this paper.