Otologic and audiologic features of ethnic Chinese patients with Turner syndrome in Taiwan

Abstract

Otologic and audiologic characteristics of Turner syndrome (TS) have been well documented in Caucasian-but not in Asian-populations. We report these features and possible causative factors for hearing loss in ethnic Chinese TS patients in Taiwan. The study was a cross-sectional trial that analyzed patients diagnosed with TS. We enrolled patients for otologic evaluations and age-appropriate pure tone audiometry. To explore the potential associations with hearing loss, we studied patient karyotype, history of recurrent otitis media (OM) and various craniofacial anomalies. The 46 patients (mean age 17.3 years, range 5-34 years) enrolled, 22 (47.8%) had a history of recurrent OM. Otoscopic examination identified eardrum abnormalities in 45.6% of patients with myringosclerosis as the most common condition. A total of 21.7% patients showed auricular anomalies. Audiologic analysis revealed five cases (10.7%) with conductive hearing loss (CHL) and eight cases (17.4%) with sensorineural hearing loss (SNHL). Among patients with a history of recurrent OM, CHL prevailed (p=0.0192) over SNHL (p=0.1278). Karyotype and craniofacial anomalies were not associated with CHL or SNHL (p>0.05). We found varying degrees of otologic and audiologic abnormalities among the TS in ethnic Chinese population. About one-half of the patients had recurrent OM, which was more likely to be associated with CHL in early life. Therefore, regular surveillance, early diagnosis, and the initiation of appropriate treatment are crucial in improving the hearing and speech in children with TS as well as in preventing short- and long-term associated complications.