Hearing disorders in Turner's syndrome: a survey from Iran

Turner syndrome (TS) is one of the most frequently encountered sex-linked chromosomal abnormalities, occurring in one per 2,000 female births. These patients present with short stature and failure to begin puberty. In this syndrome, there are multiple organ abnormalities, including auditory disorders. TS patients were referred to the ENT clinic by a pediatric endocrinologist. A questionnaire was filled out and the patients went through a complete otologic examination. They were then referred to the audiology clinic to undergo audiologic test battery plus high-frequency pure tone audiometry. From a total of 48 ears examined, 11 (22.9 %) had a normal audiometry. Mid-frequency sensorineural hearing loss (SNHL), high-frequency SNHL, combined and mixed hearing loss were diagnosed in 6 (12/5 %), 20 (41/7 %), 6 (12/5 %) and 1 (2/1 %) ear, respectively. Tympanogram results showed normal compliance (A, As, Ad) in the majority of cases. B and C patterns were found in a few cases. Speech discrimination score was normal in all patients whereas speech reception threshold was normal in 92 % of the ears. Audiometry abnormality especially SNHL is common in TS patients, with the high-frequency pattern being the most frequent.