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Hearing loss and cognitive

Gardiner von Trapp, Ishita Aloni, Stephen Young, Malcolm N Semple, Dan H Sanes
The consequences of developmental hearing loss have been reported to include both sensory and cognitive deficits. To investigate these issues in a non-human model, auditory learning and asymptotic psychometric performance were compared between normal hearing (NH) adult gerbils and those reared with conductive hearing loss (CHL). At postnatal day 10, before ear canal opening, gerbil pups underwent bilateral malleus removal to induce a permanent CHL. Both CHL and control animals were trained to approach a water spout upon presentation of a target (Go stimuli), and withhold for foils (Nogo stimuli)...
October 13, 2016: Hearing Research
Sung Kwang Hong, Sejik Park, Min-Hee Ahn, Byoung-Kyong Min
Although a peripheral auditory (bottom-up) deficit is an essential prerequisite for the generation of tinnitus, central cognitive (top-down) impairment has also been shown to be an inherent neuropathological mechanism. Using an auditory oddball paradigm (for top-down analyses) and a passive listening paradigm (for bottom-up analyses) while recording electroencephalograms (EEGs), we investigated whether top-down or bottom-up components were more critical in the neuropathology of tinnitus, independent of peripheral hearing loss...
October 8, 2016: Hearing Research
D M Baguley, T E Cope, D J McFerran
There are a number of auditory symptom syndromes that can develop without an organic basis. Some of these, such as nonorganic hearing loss, affect populations similar to those presenting with functional somatosensory and motor symptoms, while others, such as musical hallucination, affect populations with a significantly different demographic and require different treatment strategies. Many of these conditions owe their origin to measurably abnormal peripheral sensory pathology or brain network activity, but their pathological impact is often due, at least in part, to overamplification of the salience of these phenomena...
2017: Handbook of Clinical Neurology
Djurdja Djordjevic, Lauren Brady, Renkui Bai, Mark A Tarnopolsky
We describe here two novel mitochondrial mutations associated with a complex mitochondrial encephalopathy. An A to G transition at position 7495 (MT-TS1 (MT-tRNSer(UCN))) was identified at 83% heteroplasmy in the muscle of a four year old female with ptosis, hypotonia, seizures, and dilated cardiomyopathy (Case 1). A homoplasmic C to T transition at position 5577 (MT-TW (MT-tRNATrp)) was found in a twenty-four year old woman with exercise intolerance, mild muscle weakness, hearing loss, seizures, and cognitive decline (Case 2)...
September 28, 2016: Mitochondrion
Karen Low, Tazeen Ashraf, Natalie Canham, Jill Clayton-Smith, Charu Deshpande, Alan Donaldson, Richard Fisher, Frances Flinter, Nicola Foulds, Alan Fryer, Kate Gibson, Ian Hayes, Alison Hills, Susan Holder, Melita Irving, Shelagh Joss, Emma Kivuva, Kathryn Lachlan, Alex Magee, Vivienne McConnell, Meriel McEntagart, Kay Metcalfe, Tara Montgomery, Ruth Newbury-Ecob, Fiona Stewart, Peter Turnpenny, Julie Vogt, David Fitzpatrick, Maggie Williams, Sarah Smithson
KBG syndrome is characterized by short stature, distinctive facial features, and developmental/cognitive delay and is caused by mutations in ANKRD11, one of the ankyrin repeat-containing cofactors. We describe 32 KBG patients aged 2-47 years from 27 families ascertained via two pathways: targeted ANKRD11 sequencing (TS) in a group who had a clinical diagnosis of KBG and whole exome sequencing (ES) in a second group in whom the diagnosis was unknown. Speech delay and learning difficulties were almost universal and variable behavioral problems frequent...
November 2016: American Journal of Medical Genetics. Part A
Thomas Puvill, Jolanda Lindenberg, Jacobijn Gussekloo, Anton J M de Craen, Joris P J Slaets, Rudi G J Westendorp
Self-rated health is routinely used in research and practise among general populations. Older people, however, seem to change their health perceptions. To accurately understand these changed perceptions we therefore need to study the correlates of older people's self-ratings. We examined self-rated, nurse-rated and physician-rated health's association with common disabilities in older people (the geriatric giants), mortality hazard and life satisfaction. For this, we used an age-representative population of 501 participant aged 85 from a middle-sized city in the Netherlands: the Leiden 85-plus Study...
2016: PloS One
Olivia Nirmalasari, Sara K Mamo, Carrie L Nieman, Allison Simpson, Jessica Zimmerman, Milap A Nowrangi, Frank R Lin, Esther S Oh
BACKGROUND: Hearing loss can impair effective communication between caregivers and individuals with cognitive impairment. However, hearing loss is not often measured or addressed in care plans for these individuals. The aim of this study is to measure the prevalence of hearing loss and the utilization of hearing aids in a sample of individuals with cognitive impairment in a tertiary care memory clinic. METHODS: A retrospective review of 133 charts of individuals >50 years who underwent hearing assessment at a tertiary care memory clinic over a 12-month period (June 2014-June 2015) was undertaken...
September 22, 2016: International Psychogeriatrics
Allison M Bradbury, Jessica H Bagel, Xuntian Jiang, Gary P Swain, Maria L Prociuk, Caitlin A Fitzgerald, Patricia A O'Donnell, Kyle G Braund, Daniel S Ory, Charles H Vite
Globoid cell leukodystrophy (GLD), or Krabbe's disease, is a debilitating and always fatal pediatric neurodegenerative disease caused by a mutation in the gene encoding the hydrolytic enzyme galactosylceramidase (GALC). In the absence of GALC, progressive loss of myelin and accumulation of a neurotoxic substrate lead to incapacitating loss of motor and cognitive function and death, typically by 2 years of age. Currently, there is no cure. Recent convincing evidence of the therapeutic potential of combining gene and cell therapies in the murine model of GLD has accelerated the requirement for validated markers of disease to evaluate therapeutic efficacy...
November 2016: Journal of Neuroscience Research
Linda Cupples, Teresa Y C Ching, Laura Button, Greg Leigh, Vivienne Marnane, Jessica Whitfield, Miriam Gunnourie, Louise Martin
OBJECTIVE: This study examined language and speech outcomes in young children with hearing loss and additional disabilities. DESIGN: Receptive and expressive language skills and speech output accuracy were evaluated using direct assessment and caregiver report. Results were analysed first for the entire participant cohort, and then to compare results for children with hearing aids (HAs) versus cochlear implants (CIs). STUDY SAMPLE: A population-based cohort of 146 five-year-old children with hearing loss and additional disabilities took part...
September 14, 2016: International Journal of Audiology
Christian Füllgrabe, Stuart Rosen
With the advent of cognitive hearing science, increased attention has been given to individual differences in cognitive functioning and their explanatory power in accounting for inter-listener variability in the processing of speech in noise (SiN). The psychological construct that has received much interest in recent years is working memory. Empirical evidence indeed confirms the association between WM capacity (WMC) and SiN identification in older hearing-impaired listeners. However, some theoretical models propose that variations in WMC are an important predictor for variations in speech processing abilities in adverse perceptual conditions for all listeners, and this notion has become widely accepted within the field...
2016: Frontiers in Psychology
Damith Woods, Schwanagorn Sirirat, Sirirada Pattara-Angkoon, Janja Rattanajan
We report the clinical assessment of J.P., an 86 year-old man with Broca's aphasia complaining of memory problems. Our aim was to objectively investigate his level of cognitive functioning using standardized neuropsychological tests in order to determine the nature of his memory impairment. J.P.'s medical history included left-middle cerebral artery (left-MCA) stroke, high frequency hearing loss, macular degeneration, and a recent hospitalization related to a fall. Results from his neuropsychological testing and from information gathered during the clinical interview with his wife suggested that a deficit in executive functioning might have been the source for some of his perceived memory problems...
September 13, 2016: Applied Neuropsychology. Adult
Mary E Fischer, Karen J Cruickshanks, Carla R Schubert, Alex A Pinto, Cynthia M Carlsson, Barbara E K Klein, Ronald Klein, Ted S Tweed
OBJECTIVES: To evaluate the associations between sensory impairments and 10-year risk of cognitive impairment. DESIGN: The Epidemiology of Hearing Loss Study (EHLS), a longitudinal, population-based study of aging in the Beaver Dam, Wisconsin community. Baseline examinations were conducted in 1993 and follow-up examinations have been conducted every 5 years. SETTING: General community. PARTICIPANTS: EHLS members without cognitive impairment at EHLS-2 (1998-2000)...
September 9, 2016: Journal of the American Geriatrics Society
Kristin D Kernohan, Laila Cigana Schenkel, Lijia Huang, Amanda Smith, Guillaume Pare, Peter Ainsworth, Kym M Boycott, Jodi Warman-Chardon, Bekim Sadikovic
BACKGROUND: DNA methylation is an essential epigenetic mark, controlled by DNA methyltransferase (DNMT) proteins, which regulates chromatin structure and gene expression throughout the genome. In this study, we describe a family with adult-onset autosomal dominant cerebellar ataxia with deafness and narcolepsy (ADCA-DN) caused by mutations in the maintenance methyltransferase DNMT1 and assess the DNA methylation profile of these individuals. RESULTS: We report a family with six individuals affected with ADCA-DN; specifically, patients first developed hearing loss and ataxia, followed by narcolepsy, and cognitive decline...
2016: Clinical Epigenetics
Karen Erickson, Nancy Quick
The present study describes the characteristics of students in Grades 3-12 with significant cognitive disabilities (SCD) and known hearing loss. The study analyzed results of a survey of teachers of students with SCD (n = 38,367) who were slated to participate in an alternate assessment based on alternate achievement standards in 14 states in the United States. Analysis revealed similar profiles in academic achievement and symbolic language use combined with an increased incidence of additional sensory impairments among students with SCD and known hearing loss compared to their peers without known hearing loss...
September 6, 2016: Journal of Deaf Studies and Deaf Education
Samira Anderson, Kimberly Jenkins
Older adults often exhibit speech perception deficits in difficult listening environments. At present, hearing aids or cochlear implants are the main options for therapeutic remediation; however, they only address audibility and do not compensate for central processing changes that may accompany aging and hearing loss or declines in cognitive function. It is unknown whether long-term hearing aid or cochlear implant use can restore changes in central encoding of temporal and spectral components of speech or improve cognitive function...
November 2015: Seminars in Hearing
Melanie Ferguson, Helen Henshaw
Auditory training aims to compensate for degradation in the auditory signal and is offered as an intervention to help alleviate the most common complaint in people with hearing loss, understanding speech in a background noise. Yet there remain many unanswered questions. This article reviews some of the key pieces of evidence that assess the evidence for whether, and how, auditory training benefits adults with hearing loss. The evidence supports that improvements occur on the trained task; however, transfer of that learning to generalized real-world benefit is much less robust...
November 2015: Seminars in Hearing
Maia Ingram, Nicole Marrone, Daisey Thalia Sanchez, Alicia Sander, Cecilia Navarro, Jill Guernsey de Zapien, Sonia Colina, Frances Harris
UNLABELLED: Hearing loss is associated with cognitive decline and impairment in daily living activities. Access to hearing health care has broad implications for healthy aging of the U.S. POPULATION: This qualitative study investigated factors related to the socio-ecological domains of hearing health in a U.S.-Mexico border community experiencing disparities in access to care. A multidisciplinary research team partnered with community health workers (CHWs) from a Federally Qualified Health Center (FQHC) in designing the study...
2016: Frontiers in Public Health
Magda Aline Bauer, Ângela Kemel Zanella, Irênio Gomes Filho, Geraldo de Carli, Adriane Ribeiro Teixeira, Ângelo José Gonçalves Bós
INTRODUCTION: Hearing is essential for the processing of acoustic information and the understanding of speech signals. Hearing loss may be associated with cognitive decline, depression and reduced functionality. OBJECTIVE: To analyze the prevalence of hearing complaints in elderly individuals from Rio Grande do Sul and describe the profile of the study participants with and without hearing complaints. METHODS: 7315 elderly individuals interviewed in their homes, in 59 cities in the state of Rio Grande do Sul, Brazil, participated in the study...
July 31, 2016: Brazilian Journal of Otorhinolaryngology
Alexandre Villeneuve, Caroline Hommet, Charles Aussedat, Emmanuel Lescanne, Kevin Reffet, David Bakhos
The objective of this study is to assess the validity of ASSR as a complementary diagnostic test for peripheral hearing loss by proving a significant correlation between behavioral thresholds and ASSR. The design used in this study is monocentric prospective study from November 2014 to April 2015. The setting used in this study is the ENT-Head and Neck Surgery Department and Geriatrics Department in a French Regional and University Hospital. The participants are patients over 75 years with cognitive impairment (Alzheimer's disease or mild-cognitive impairment) with a Mini-Mental State Examination score under 27/30 and without hearing aids...
August 23, 2016: European Archives of Oto-rhino-laryngology
Janet S Choi, Kyoo S Shim, Kunhwa Kim, Carrie L Nieman, Sara K Mamo, Frank R Lin, Hae-Ra Han
Hearing loss is associated with an accelerated decline in social, cognitive, and physical functioning among older adults. However, little is known about its impact and barriers to hearing health care in any ethnic minorities. The aim of this study was to explore experiences related to hearing loss and barriers to hearing health care among older Korean Americans (KAs). We conducted four focus groups with 19 older KAs with hearing loss and four communication partners. Qualitative content analysis revealed four themes: (a) impact of hearing loss, (b) self-perception of hearing loss, (c) coping strategies for hearing loss, and (d) barriers to hearing health care (price, language, lack of collaborative communications, perceptions about hearing aids, and lack of knowledge)...
August 22, 2016: Journal of Applied Gerontology: the Official Journal of the Southern Gerontological Society
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