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.

Hearing disorders in Turner’s syndrome / Ralli, Massimo; Turchetta, Rosaria; Cianfrone, Giancarlo. - In: OTOLARYNGOLOGY. - ISSN 2470-4059. - ELETTRONICO. - 2:4(2016), pp. 115-119. [10.17140/OTLOJ-2-126]

Hearing disorders in Turner’s syndrome

RALLI, MASSIMO
Primo
;
TURCHETTA, Rosaria
Penultimo
;
CIANFRONE, Giancarlo
Ultimo
2016

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 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.
2016
Turner’s syndrome; hearing disorders; hearing loss; tinnitus
01 Pubblicazione su rivista::01g Articolo di rassegna (Review)
Hearing disorders in Turner’s syndrome / Ralli, Massimo; Turchetta, Rosaria; Cianfrone, Giancarlo. - In: OTOLARYNGOLOGY. - ISSN 2470-4059. - ELETTRONICO. - 2:4(2016), pp. 115-119. [10.17140/OTLOJ-2-126]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1000231
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