Objective: To analyze the prevalence and importance of the maternally inherited A1555G mutation in the 12S rRNA gene in the Austrian population.
Study design: Investigation for mutations of genetically affected familial and sporadic cases of hearing impairment (HI), including analyses of audiometric data.
Setting: Teaching hospital, tertiary referral center.
Patients: Forty-five familial and 77 sporadic cases of nonsyndromic HI in an Austrian Caucasian ethnic group.
Main outcome measure(s): Pure-tone audiometric data and screening by restriction fragment length polymorphism analysis after exclusion of GJB2 (Connexin 26) caused hearing loss.
Results: In the investigated hearing-impaired population, the mutation A1555G in the mitochondrial 12S rRNA gene was not detected.
Conclusion: The A1555G mutation in the mitochondrial DNA 12S rRNA is not a major cause of HI in the Austrian Caucasian population.