Hearing loss in adult women with Turner’s syndrome and other congenital hypogonadisms

Abstract

More than a half of TS females presented HL. SNHL is the most frequent pattern among middle-aged women with TS. Old age, karyotype and recurrent otitis are predisposition factors to produce HL, while oestrogens play a minor role.

Many women with Turner syndrome lack protective antibodies to common respiratory pathogens, Haemophilus influenzae type B and Streptococcus Pneumoniae

“…Exploration of the age at time of vaccination (arbitrary age cut‐off of 30), presence of autoimmune disorder(s) and iXq karyotype did not find any correlation with poor vaccination response. Of note, 46% (53/114) of our cohort have appreciable hearing impairment, consistent with findings of a multicentre study reporting hearing impairment of at least moderate severity in 41%, which has contextual relevance because a history of recurrent otitis is strongly associated with hearing loss in patients with TS …”

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…Only approximately one‐third of Turner syndrome patients have normal hearing (Bonnard, Hederstierna, Bark, & Hultcrantz, ; Verver et al, ). The hearing loss progresses with age and is present in nearly all women over 50 years of age (Beckman et al, ; King et al, ; Ros et al, ). The underlying reasons for hearing loss are multifactorial.…”

<p>Metabolic Syndrome as a Risk Factor for Sensorineural Hearing Loss in Adult Patients with Turner Syndrome</p>

“…An elevated incidence of hearing loss (HL) has been reported in adult patients with TS. [12][13][14] This deficiency can be conductive, sensorineural, or mixed. 13,15 Sensorineural hearing loss (SNHL) is the most prevalent as it affects at least half of the women with TS 35 years or older.…”

“…[12][13][14] This deficiency can be conductive, sensorineural, or mixed. 13,15 Sensorineural hearing loss (SNHL) is the most prevalent as it affects at least half of the women with TS 35 years or older. 16 SNHL in TS is gradually progressive, resulting at age 40 in hearing comparable to that of women aged 60 in the general population.…”

“…As well as, associated factors to HL, such as the influence of age, chromosomal constitution, effect of the parental origin of the retained X-chromosome, estrogen and rhGH therapies, craniofacial malformations and ear infections have been previously evaluated in TS subjects. [12][13][14][15]23,24 Nevertheless, the effect of such associated factors on HL has been inconsistent and controversial and there is no general agreement of which the main factors affecting HL in TS are. In addition, several studies to date have tended to focus on the influence of one rather than multiple associated factors.…”