Hearing loss in Turner syndrome.

OBJECTIVE To address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors. STUDY DESIGN Thirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored. RESULTS Twenty cases (35 of 66 ears tested) showed high-frequency (8 kHz) sensory neural hearing loss (HFQ-SNHL). Fifteen cases (26 ears) and 15 cases (24 ears) of the impaired 20 cases were unresponsive to distortion-product otoacoustic emissions and transient-evoked otoacoustic emissions, respectively. HFQ-SNHL showed little relation to the history of middle ear infection and puberty, although middle ear infections were seen in 11 of the 20 cases. The hearing thresholds at high frequencies were correlated with age and body height (P < .001). The age-dependent increase in hearing thresholds in the high frequencies was more apparent in patients with TS with monosomic 45, X than in those with the mosaic type (P < .05). CONCLUSIONS More than 60% of patients with TS had HFQ-SNHL. Because the increase in hearing threshold at high frequencies was shown to depend on karyotype and aging, regular otological examination is important for the determination of proper treatment.