Abstract: AIM:To determine a mitochondrial DNA mutation in a maternal inherited deafness family, and guide aminoglycosides antibiotics individual application in other family members.METHODS:PCR-re-striction fragment length polymorphism analysis and DNA sequencing were conducted in a maternal inherited deafness patient with a definite aminoglycosides antibiotics application history.RESULTS:The patient was confirmed with a 1555 A →G mutation in mitochondrial DNA.CONCLUSION:It suggested mitochondrial DNA point mutation is one of the main reasons of deafness, the patient's sib should be cautious to use aminoglycosides antibiotics, and her progeny should be prohibited to use aminoglycosides antibiotics.

Key words: maternal inheritance, chondriosome DNA, aminoglycosides, gene mutation