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Concept: Hearing impairment

195

The 2016 National Academies of Sciences report “Hearing Health Care for Adults: Priorities for Improving Access and Affordability” included a call to action for government agencies to strengthen efforts to collect, analyze, and disseminate population-based data on hearing loss in adults.

Concepts: Health care, United States, Vital signs, Hearing impairment, Tinnitus, Barack Obama, Massachusetts, 2016 Summer Olympics

170

To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors.

Concepts: Cochlea, Hearing impairment, Cochlear implant, Implants, Otology, Models of deafness

170

Domestic dogs can suffer from hearing losses that can have profound impacts on working ability and quality of life. We have identified a type of adult-onset hearing loss in Border Collies that appears to have a genetic cause, with an earlier age of onset (3-5 years) than typically expected for aging dogs (8-10 years). Studying this complex trait within pure breeds of dog may greatly increase our ability to identify genomic regions associated with risk of hearing impairment in dogs and in humans. We performed a genome-wide association study (GWAS) to detect loci underlying adult-onset deafness in a sample of 20 affected and 28 control Border Collies. We identified a region on canine chromosome 6 that demonstrates extended support for association surrounding SNP Chr6.25819273 (p-value = 1.09×10(-13)). To further localize disease-associated variants, targeted next-generation sequencing (NGS) of one affected and two unaffected dogs was performed. Through additional validation based on targeted genotyping of additional cases (n = 23 total) and controls (n = 101 total) and an independent replication cohort of 16 cases and 265 controls, we identified variants in USP31 that were strongly associated with adult-onset deafness in Border Collies, suggesting the involvement of the NF-κB pathway. We found additional support for involvement of RBBP6, which is critical for cochlear development. These findings highlight the utility of GWAS-guided fine-mapping of genetic loci using targeted NGS to study hereditary disorders of the domestic dog that may be analogous to human disorders.

Concepts: Genetics, Cochlea, Hearing impairment, Genome-wide association study, Audiogram, Dog, Dog breed, Dog health

168

Auditory training involves active listening to auditory stimuli and aims to improve performance in auditory tasks. As such, auditory training is a potential intervention for the management of people with hearing loss.

Concepts: Randomized controlled trial, Auditory system, Hearing impairment, Audiogram, Hearing, Active listening

167

To evaluate the onset of vertigo, hearing loss and tinnitus in Ménière’s disease and the associated endolymphatic hydrops (EH) of the inner ear.

Concepts: Auditory system, Cochlea, Hearing impairment, Ear, Tinnitus, Ménière's disease, Inner ear, Endolymph

165

Hearing loss is the third most common chronic physical condition in the United States, and is more prevalent than diabetes or cancer (1). Occupational hearing loss, primarily caused by high noise exposure, is the most common U.S. work-related illness (2). Approximately 22 million U.S. workers are exposed to hazardous occupational noise (3). CDC compared the prevalence of hearing impairment within nine U.S. industry sectors using 1,413,789 noise-exposed worker audiograms from CDC’s National Institute for Occupational Safety and Health (NIOSH) Occupational Hearing Loss Surveillance Project (4). CDC estimated the prevalence at six hearing impairment levels, measured in the better ear, and the impact on quality of life expressed as annual disability-adjusted life years (DALYs), as defined by the 2013 Global Burden of Disease (GBD) Study (5). The mining sector had the highest prevalence of workers with any hearing impairment, and with moderate or worse impairment, followed by the construction and manufacturing sectors. Hearing loss prevention, and early detection and intervention to avoid additional hearing loss, are critical to preserve worker quality of life.

Concepts: United States, U.S. state, Hearing impairment, World Health Organization, Audiogram, Occupational safety and health, Disability-adjusted life year, Disease burden

145

To evaluate the factors that limit post-cochlear implantation (CI) speech perception in prelingually deaf children.

Concepts: Cochlea, Hearing impairment, Cochlear implant, Otology, Implantation, Deaf culture, Models of deafness, Prelingual deafness

143

A growing interest in cognitive effects associated with speech and hearing processes is spreading throughout the scientific community essentially guided by evidence that central and peripheral hearing loss is associated with cognitive decline. For the present research, 125 participants older than 65 years of age (105 with hearing impairment and 20 with normal hearing) were enrolled, divided into 6 groups according to their degree of hearing loss and assessed to determine the effects of the treatment applied. Patients in our research program routinely undergo an extensive audiological and cognitive evaluation protocol providing results from the Digit Span test, Stroop color-word test, Montreal Cognitive Assessment and Geriatric Depression Scale, before and after rehabilitation. Data analysis was performed for a cross-sectional and longitudinal study of the outcomes for the different treatment groups. Each group demonstrated improvement after auditory rehabilitation or training on short- and long-term memory tasks, level of depression and cognitive status scores. Auditory rehabilitation by cochlear implants or hearing aids is effective also among older adults (median age of 74 years) with different degrees of hearing loss, and enables positive improvements in terms of social isolation, depression and cognitive performance.

Concepts: Cognition, Gerontology, Cochlea, Hearing impairment, Cochlear implant, Otology, Audiogram, Models of deafness

130

Death of cochlear hair cells, which do not regenerate, is a cause of hearing loss in a high percentage of the population. Currently, no approach exists to obtain large numbers of cochlear hair cells. Here, using a small-molecule approach, we show significant expansion (>2,000-fold) of cochlear supporting cells expressing and maintaining Lgr5, an epithelial stem cell marker, in response to stimulation of Wnt signaling by a GSK3β inhibitor and transcriptional activation by a histone deacetylase inhibitor. The Lgr5-expressing cells differentiate into hair cells in high yield. From a single mouse cochlea, we obtained over 11,500 hair cells, compared to less than 200 in the absence of induction. The newly generated hair cells have bundles and molecular machinery for transduction, synapse formation, and specialized hair cell activity. Targeting supporting cells capable of proliferation and cochlear hair cell replacement could lead to the discovery of hearing loss treatments.

Concepts: DNA, Gene, Histone deacetylase, Stem cell, Auditory system, Cochlea, Hearing impairment, Hair cell

89

BACKGROUND Whether hearing loss is independently associated with accelerated cognitive decline in older adults is unknown. METHODS We studied 1984 older adults (mean age, 77.4 years) enrolled in the Health ABC Study, a prospective observational study begun in 1997-1998. Our baseline cohort consisted of participants without prevalent cognitive impairment (Modified Mini-Mental State Examination [3MS] score, ≥80) who underwent audiometric testing in year 5. Participants were followed up for 6 years. Hearing was defined at baseline using a pure-tone average of thresholds at 0.5 to 4 kHz in the better-hearing ear. Cognitive testing was performed in years 5, 8, 10, and 11 and consisted of the 3MS (measuring global function) and the Digit Symbol Substitution test (measuring executive function). Incident cognitive impairment was defined as a 3MS score of less than 80 or a decline in 3MS score of more than 5 points from baseline. Mixed-effects regression and Cox proportional hazards regression models were adjusted for demographic and cardiovascular risk factors. RESULTS In total, 1162 individuals with baseline hearing loss (pure-tone average >25 dB) had annual rates of decline in 3MS and Digit Symbol Substitution test scores that were 41% and 32% greater, respectively, than those among individuals with normal hearing. On the 3MS, the annual score changes were -0.65 (95% CI, -0.73 to -0.56) vs -0.46 (95% CI, -0.55 to -0.36) points per year (P = .004). On the Digit Symbol Substitution test, the annual score changes were -0.83 (95% CI, -0.94 to -0.73) vs -0.63 (95% CI, -0.75 to -0.51) points per year (P = .02). Compared to those with normal hearing, individuals with hearing loss at baseline had a 24% (hazard ratio, 1.24; 95% CI, 1.05-1.48) increased risk for incident cognitive impairment. Rates of cognitive decline and the risk for incident cognitive impairment were linearly associated with the severity of an individual’s baseline hearing loss. CONCLUSIONS Hearing loss is independently associated with accelerated cognitive decline and incident cognitive impairment in community-dwelling older adults. Further studies are needed to investigate what the mechanistic basis of this association is and whether hearing rehabilitative interventions could affect cognitive decline.

Concepts: Psychology, Regression analysis, Proportional hazards models, Observational study, Cognition, Hearing impairment, Audiogram, Numerical digit