Ear and hearing problems in 44 middle-aged women with Turner's syndrome

The present study has investigated ear and hearing problems in 44 women with Turner's syndrome (median age 45.5 years). Social hearing problems were common after the age of 40 and 27% were fitted with hearing aids. Audiograms revealed a hearing loss > 20 dB hearing level (HL) in 91% leading to clinically significant hearing problems in 60%. A distinct dip in the 1.5 kHz frequency range, with a mean value of 46 dB was found in 30 women. The occurrence of the dip was correlated to the karyotype. All women with the karyotype 45,X and 45,X/46,X,i(Xq) demonstrated this dip while in the 45,X/46,XX group it was found in 31%. No dips were found among 45,X/46,XY and 45,X/46,XX/47,XXX women. With increasing age a progressive high frequency hearing loss was added to the dip leading to severe hearing problems earlier in the Turner women than age-matched controls. This might be due to a genetic defect leading to premature ageing of their hearing organ. These data emphasize the importance of providing early information to Turner girls of their predisposition to hearing impairment. Patient awareness of importance of audiological evaluations and the benefit of hearing aids should be stressed.