Orphanet Journal of Rare Diseases (Mar 2024)
The impact of amplification on quality of life in women with Turner syndrome
Abstract
Abstract Background Individuals with Turner syndrome (TS, ORPHA 881) experience barriers in communication throughout life as they navigate both early conductive, and progressive sensorineural hearing loss amid other healthcare needs. Hearing loss is self-identified as one of the largest unmet healthcare needs. Purpose The purpose of this study was to investigate the impact of treatment for hearing loss on communication confidence and quality of life measures for individuals with TS. Research design We employed a prospective cross-sectional study design that included both online survey data and audiometric data for a subset of participants. Study sample We recruited 179 adults with TS at the Turner Syndrome Society of the United States (TSSUS) Conference, and through a variety of regional TS organizations’ social media platforms. Audiological data was collected onsite at the conference for a subset of 67 participants; 8 of which who were followed after receiving subsequent treatment with hearing aids. Data collection and analysis The online survey design included demographic questions, the Communication Confidence Profile (CCP), and the RAND 36-Item Health Survey 1.0. Audiometric data included tympanometry, puretone air, and puretone bone conduction thresholds. Descriptive statistics, parametric, and non-parametric tests were used to analyze both survey and audiometric data. Results 74% of participants had a self-reported diagnosis of hearing loss, of which 61% were previously recommended amplification. Only 38% of participants reported using hearing aids. For those participants who wore hearing aids, Total CCP Score, ‘Confidence in Ability to Hear Under Various Conditions’, and ‘Energy/Vitality’ metrics were significantly greater than those with untreated hearing loss warranting a hearing aid. Collectively, Total CCP Score and ‘Confidence in Ability to Hear Under Various Conditions’ increased significantly when participants were fit with hearing aids. Conclusion The results support previous data where hearing loss is a self-identified healthcare concern among women with Turner syndrome, yet many fail to receive appropriate hearing evaluation or treatment. Additionally, the use of hearing aids may improve communication confidence and quality of life in women with Turner syndrome. Furthermore, this study confirms the need for long-term audiological care and monitoring in women with Turner syndrome.
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