Hearing Disorders in Turner’s Syndrome
Journal Title: Emergency Medicine – Open Journal - Year 2016, Vol 2, Issue 4
Abstract
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 findings 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 dysfunction. Sensorineural hearing loss follows 2 main audiological profiles: 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.
Authors and Affiliations
Massimo Ralli
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