Achados audiológicos em pacientes com Síndrome de Turner

Abstract

Introduction. Patients with Turner syndrome (TS) may present early hearing loss (HL) with impairment in social life and quality of life. The prevalence of hearing disorders in TS has been described in different countries, however the data in our country are still poorly understood. Conductive hearing loss is related to recurrent infections, but the mechanisms involved with sensorineural hearing loss (SNHL) have not yet been fully elucidated. Aim. To describe the audiological profile of TS patients, determine the prevalence, type and degree of HL, and correlate the findings with age, karyotype, components of the metabolic syndrome, and growth hormone (GH) / estrogen (E) therapy. Methods. A cross-sectional observational study evaluating 43 patients (24.2 ± 10.9) with a diagnosis of TS confirmed by karyotype. Clinical and biochemical data obtained through a questionnaire and review of medical records were correlated with findings of otoscopy, tonal audiometry, vocal and imitanciometry. The relationship between the threshold means in the frequencies of 0.5, 1 and 2 KHz (PTA-3) and 0.5, 1, 2 and 4 KHz (PTA-4) and the other variables were determined by the linear regression model Generalized with Gamma Log function (Sidak significance test, p <0.05), in normal ears or with SNHL. Results. Auditory alterations were detected in 53% of the patients. The prevalence of HL was higher in adult patients (46.3%) . PASN was the most prevalent type (66%) (mean 31 years), especially in adults, not treated with GH in childhood. PTA-3 and PTA-4 were significantly lower (P = 0.001) in those exposed to GH / E therapy than in the non-GH treated group. No relation was observed with the karyotype. Conclusions. SNHL was more common in patients not treated with GH and for the first time it was demonstrated that GH therapy may have a beneficial effect on hearing in patients with TS.