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 conse
quence of morphological cranio-facial alterations resulting in middle ear ventilation dysfunc
tion. 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 un
dergo early evaluation and monitoring over time