The impact of amplification on quality of life in women with Turner syndrome.

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.

Cochlear Implant Referral Criteria for the Spanish-Speaking Adult Population.

OBJECTIVE: To evaluate the effectiveness of the "60/60 Guideline" in a diverse patient population. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Data were collected for adult patients (18 years and older) who underwent a cochlear implant evaluation (CIE) between January 2016 and March 2021. MAIN OUTCOME MEASURES: Development of the "60/60 Guideline" has provided better clarity on when to refer English-speaking patients for a CIE. Our study evaluated the effectiveness of this referral tool in the Spanish-speaking population. RESULTS: In our group of patients who underwent a traditional CIE (n = 402), 209 met unaided and aided traditional cochlear implant (CI) candidacy criteria. Of the 193 individuals who did not meet both components of traditional candidacy criteria, a majority met the aided component (86%) but only 4.6% met the unaided component. When applying the 60/60 Guideline to patients who met traditional criteria, there is a sensitivity rating of 84.7% and a specificity index of 50.3%. For English and Spanish speakers who met traditional criteria but did not meet the 60/60 Guideline, a majority (83.3% English, 87.5% Spanish) had a better ear word recognition score (WRS) greater than 60%, suggesting the unaided WRS is the more restrictive component of the "60/60 Guideline." CONCLUSION: Application of the "60/60 Guideline" is an effective method to identify potential CI candidates in the English-speaking population; however, it was less effective in the Spanish-speaking population. Spanish-speaking adults should be referred for a CIE when better ear pure tone average is greater than 60 dB hearing loss, regardless of their unaided WRS. This study highlights the need for inclusion of nonlinguistic test measures in the CI referral criteria and test battery to reduce CI access barriers for patients who speak a language other than English.

Peripheral vestibular loss in noise-exposed firefighters.

Introduction: Occupational workers are increasingly aware of the risk of noise overexposure to the auditory system but lack awareness about potential risks to the vestibular system. The purpose of this study was to investigate changes in vestibular end organ function in a known at-risk noise-exposed population, firefighters compared to age- and sex-matched controls using electrophysiologic measures of cervical vestibular evoked myogenic potentials (cVEMP). Methods: A cross-sectional observational study compared cVEMP response characteristics in 38 noise-exposed firefighters. Firefighters were grouped by years of exposure in the fire service. The cVEMP responses were compared within firefighter groups and between firefighters and age- and sex-matched controls. Dependent variables included the response characteristics of amplitude, latency and threshold. Results: cVEMP response amplitudes were significantly decreased in firefighters compared to their age- and sex-matched controls. Threshold of the cVEMP responses were significantly higher in firefighters compared to controls and firefighters had a higher incidence of absent cVEMP responses compared to controls. Response amplitudes decreased with increasing years in the fire-service at an increased rate compared to their age- and sex-matched controls. Latency of the cVEMP response was not significantly different in firefighters compared to controls. These findings are consistent with both animal and human studies suggesting noise-induced changes in the sacculocollic pathway. Discussion: In the absence of any reported vestibular symptoms or auditory indicators of noise-induced hearing loss, these early effects on the vestibular system point to a potential hidden vestibular loss.