Hearing disorders in Turner’s syndrome

Introduction: Turner’s Syndrome (TS) is associated with hearing disorders in about 20 to 50% of affected individuals. The most common hearing disorders include congenital auricular malformations, recurrent otitis media and sensorineural hearing loss, although altered vestibular function and tinnitus have also been reported. Objectives: The aim of this paper is to provide an up-to-date overview of the principal ndings and research perspectives about the association between TS and hearing disorders. Review: Middle ear disorders, found in a range between 21 and 91% of subjects, are a consequence of morphological cranio-facial alterations resulting in middle ear ventilation dysfunc- tion. Sensorineural hearing loss follows 2 main audiological pro les: a bilateral symmetrical mid-frequency dip and a high frequency down-sloping curve. Although the pathophysiologic basis of sensorineural hearing loss in TS patients are still unclear, several hypothesis have been made so far and are reviewed in this paper. Conclusion: Literature confirms that hearing disorders, although not the most relevant clinical problem in these patients, have a high incidence in patients with TS and should therefore undergo early evaluation and monitoring over time.