EAR AND HEARING PROBLEMS IN 44 MIDDLE-AGED WOMEN WITH TURNERS-SYNDROME

The present study has investigated ear and hearing problems in 44 wome n with Turner's syndrome (median age 45.5 years). Social hearing probl ems were common after the age of 40 and 27% were fitted with hearing a ids. Audiograms revealed a hearing loss > 20 dB hearing level (HL) in 91% leading to clinically significant hearing problems in 60%. A disti nct 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 ka ryotype. All women with the karyotype 45,X and 45,X/46,X,i(Xq) demonst rated this dip while in the 45,X/46,XX group it was found in 31%. No d ips were found among 45,X/46,XY and 45,X/46,XX/47,XXX women. With incr easing age a progressive high frequency hearing loss was added to the dip leading to severe hearing problems earlier in the Turner women tha n age-matched controls. This might be due to a genetic defect leading to premature ageing of their hearing organ. These data emphasize the i mportance of providing early information to Turner girls of their pred isposition to hearing impairment. Patient awareness of importance of a udiological evaluations and the benefit of hearing aids should be stre ssed.