OTOLOGICAL PROBLEMS IN CHILDREN WITH TURNERS-SYNDROME

Ear and hearing disorders are common problems among girls and women wi th Turner's syndrome. During infancy and childhood the girls often suf fer from repeated attacks of acute otitis media and later in life the women frequently complain of a rapid onset of social hearing problems due to sensorineural hearing impairment. A study of 56 girls aged 4-15 years with Turner's syndrome was performed to investigate the prevale nce of eardrum pathology and hearing impairment in young children and teenagers with Turner's syndrome. A possible relation to karyotype was also investigated. A high prevalence (61%) of recurrent acute otitis media was found in the study group and 32% had been treated with venti lation tubes. Fifty-seven percent showed eardrum pathology, such as ef fusion, myringosclerosis, atrophic scars, retraction pockets and perfo rations. Auricular anomalies were noted in 23% of the cases, most comm only in the 45,X group. The audiometric analysis showed conductive hea ring loss lair-bone gap > 10 dB HL) in 43% and the typical sensorineur al dip in the middle frequencies was found in 58% of the girls, of who m the youngest was 6 years old. Four percent were using hearing aids. The data of this study further confirm that the dip is progressive ove r time and may be detectable as early as at the age of 6, giving a cha nce to predict a future hearing loss. The findings emphasize the impor tance of regular otological examinations and audiological evaluations of all girls with Turner's syndrome early in life. (C) 1998 Elsevier S cience B.V. All rights reserved.