Hearing Disorders in Turner’s Syndrome

Massimo Ralli*, Rosaria Turchetta and Giancarlo Cianfrone

Hearing Disorders in Turner’s Syndrome.

Turner’s Syndrome 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.

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.

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.

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.

Turner’s Syndrome, a common chromosomal condition with an estimated incidence of 1
per 2,000 women, 1
is associated with hearing disorders in about 20 to 40% of affected individuals.

TS, also known as 45,X, is caused by a complete or partial deletion of one of X chromosomes; affected subjects are characterized by short stature, typical dysmorphic features, cardiac
abnormalities, gonadal dysgenesis, congenital lymphoedema, renal malformations, obesity,
diabetes and other minor somatic anomalies described by the Turner’s Syndrome Association.

Intelligence is usually within normal range, minor problems with non-verbal, social and psychomotor skills may be present.

Asymptomatic vestibular dysfunction and tinnitus have also been reported, but only in the form of case reports.

Otolaryngol Open J. 2016; 2(4): 115-119. doi: 10.17140/OTLOJ-2-126