Résumé
BACKGROUND AND OBJECTIVES: Turner syndrome (TS) is known to be caused by a total or partial deletion of one X-chromosome. Besides short stature and failure to enter puberty due to ovarian dysgenesis, auricular malformations, middle ear diseases and hearing impairment are also other clinical features of Turner syndrome. The goal of this study is to report otologic and audiologic characteristics in a group of children with Turner syndrome and correlate with these findings to karyotype. SUBJECTS AND METHOD: We retrospectively reviewed the outpatient charts of those who visited at our department for otologic and audiologic screening test between 2008 and 2011. All 23 TS children (46 ears) were enrolled under regular control of their pediatric endocrinologist for treatment with growth hormon and Estrogen. The mean age was 12.6 years (6-24 years). All children were evaluated by otologic history taking, otoscopy, pure tone audiometry and karyotyping. Furthermore, 16 children undertook auditory brain stem response (ABR) test and 10 children temporal bone computed tomography (CT). RESULTS: Abnormal otoscopic findings were found in 48% (22 ears), abnormal otologic history in 70% (16 children), and abnormal audiologic findings in 70% (32 ears). According to karyotyping, the total p-arm deletion group (74%) showed unfavorable audiologic results. ABR test and temporal bone CT did not show any unique findings, except five poor pneumatization of mastoid. CONCLUSION: Hearing impairment can be present at early age in Turner syndrome. Careful follow up during childhood is necessary to detect early ear and hearing problems for active intervention. Karyotype may be used as a predictor for future hearing impairment.