Hearing Disorders in Turner’s Syndrome

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 mal-formations, 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.