Abstract

Turner syndrome (TS) is characterized by short stature and hypergonadotrophic hypogonadism, associated with typical somatic features. Although hearing loss (HL) was not reported in Henry Turner first description of the disease, it has been brought to attention in the last decades in many studies. But the true nature of this problem is still unknown due to inconsistent data resulting from different study methodology. The purpose of this study is to evaluate hearing of TS girls using Audiometry and Otoacustic emissions, both Distortion product (DPOAEs) and Transient (TOAEs) in the outpatient clinic of a tertiary hospital and hearing complaints of these patients. Materials and Methods: prospective cross-sectional study of TS girls aging from six to 19 years old. Results: 13 patients with TS (26 ears) were evaluated. They showed in Audiometry: normal hearing in 14 ears, NSHL in three ears, conductive in seven and mixed HL in two ears (12 alterations). In the DPOAEs: 19 absent responses (73.1% of cases) in at least one frequency, and in TOAEs: 16 absent responses (61.5%). 15 absent responses in both tests (57.7%). Five patients had history of chronic otitis media. Conclusion: The presence of auditory alterations in TS patients assessed by OAE was high, with presence of history of chronic otitis media and their complications. Only one patient had hearing complaints. These findings should bring to attention HL in these patients, even without complaints.

Keywords

Turner syndrome, hearing, hearing loss, otoacustic emissions