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Hearing loss and cognitive

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https://www.readbyqxmd.com/read/27895549/the-repeatable-battery-for-the-assessment-of-neuropsychological-status-for-hearing-impaired-individuals-rbans-h-before-and-after-cochlear-implantation-a-protocol-for-a-prospective-longitudinal-cohort-study
#1
Annes J Claes, Griet Mertens, Annick Gilles, Anouk Hofkens-Van den Brandt, Erik Fransen, Vincent Van Rompaey, Paul Van de Heyning
Background: Currently, an independent relationship between hearing loss and cognitive decline in older adults is suggested by large prospective studies. In general, cochlear implants improve hearing and the quality of life in severely to profoundly hearing impaired older persons. However, little is known about the effects of cochlear implantation on the cognitive evolution in this population. Aim of the study: The primary goal of this prospective, longitudinal cohort study is to explore the cognitive profile of severely to profoundly postlingually hearing impaired subjects before and after cochlear implantation...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27872819/diagnosis-of-a-mild-peroxisomal-phenotype-with-next-generation-sequencing
#2
Meredith J Ventura, Dianna Wheaton, Mingchu Xu, David Birch, Sara J Bowne, Lori S Sullivan, Stephen P Daiger, Annette E Whitney, Richard O Jones, Ann B Moser, Rui Chen, Michael F Wangler
Peroxisomal biogenesis disorders (PBD) are caused by mutations in PEX genes, and are typically diagnosed with biochemical testing in plasma followed by confirmatory testing. Here we report the unusual diagnostic path of a child homozygous for PEX1 p.G843D. The patient presented with sensorineural hearing loss, pigmentary retinopathy, and normal intellect. After testing for Usher syndrome was negative, he was found to have PBD through a research sequencing panel. When evaluating a patient with hearing loss and pigmentary retinopathy, mild PBD should be on the differential regardless of cognitive function...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27867345/language-impairment-resulting-from-a-de-novo-deletion-of-7q32-1q33
#3
María S Jiménez-Romero, Montserrat Barcos-Martínez, Isabel Espejo-Portero, Antonio Benítez-Burraco
We report on a girl who presents with hearing loss, behavioral disturbances (according to the Inventory for Client and Agency Planning) as well as motor and cognitive delay (according to Battelle Developmental Inventories) which have a significant impact on her speech and language abilities [according to the Peabody Picture Vocabulary Test (ed 3), and the Prueba de Lenguaje Oral de Navarra-Revisada (Navarra Oral Language Test, Revised)]. Five copy number variations (CNVs) were identified in the child: arr[hg18] 7q32...
October 2016: Molecular Syndromology
https://www.readbyqxmd.com/read/27830152/linguistic-factors-influencing-speech-audiometric-assessment
#4
Martine Coene, Stefanie Krijger, Matthias Meeuws, Geert De Ceulaer, Paul J Govaerts
In speech audiometric testing, hearing performance is typically measured by calculating the number of correct repetitions of a speech stimulus. We investigate to what extent the repetition accuracy of Dutch speech stimuli presented against a background noise is influenced by nonauditory processes. We show that variation in verbal repetition accuracy is partially explained by morpholexical and syntactic features of the target language. Verbs, prepositions, conjunctions, determiners, and pronouns yield significantly lower correct repetitions than nouns, adjectives, or adverbs...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27815131/sensory-coding-and-cognitive-processing-of-sound-in-veterans-with-blast-exposure
#5
Scott Bressler, Hannah Goldberg, Barbara Shinn-Cunningham
Recent anecdotal reports from VA audiology clinics as well as a few published studies have identified a sub-population of Service Members seeking treatment for problems communicating in everyday, noisy listening environments despite having normal to near-normal hearing thresholds. Because of their increased risk of exposure to dangerous levels of prolonged noise and transient explosive blast events, communication problems in these soldiers could be due to either hearing loss (traditional or "hidden") in the auditory sensory periphery or from blast-induced injury to cortical networks associated with attention...
November 2, 2016: Hearing Research
https://www.readbyqxmd.com/read/27806352/aging-cognitive-decline-and-hearing-loss-effects-of-auditory-rehabilitation-and-training-with-hearing-aids-and-cochlear-implants-on-cognitive-function-and-depression-among-older-adults
#6
Alessandro Castiglione, Alice Benatti, Carmelita Velardita, Diego Favaro, Elisa Padoan, Daniele Severi, Michela Pagliaro, Roberto Bovo, Antonino Vallesi, Carlo Gabelli, Alessandro Martini
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...
2016: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/27806351/the-epidemiology-of-cognitive-impairment-in-the-aging-population-implications-for-hearing-loss
#7
Andrea Peracino, Sergio Pecorelli
Cognitive impairment and dementia are characterized by a progressive and devastating reduction in most cognitive abilities, functional independence, and social relationships. Dementia represents a substantial financial burden on society, one that is comparable to the financial burden of heart disease and cancer. Due to its insidious onset, cognitive impairment can be clinically silent for several years; therefore, diagnosis occurs late in the disease process, and treatment becomes almost useless. The identification of predictors of dementia may help identify the pathophysiological mechanisms underlying the disease and lead to the development of a more effective medical diagnosis and therapy, and thus an early treatment...
November 3, 2016: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/27788277/using-cognitive-screening-tests-in-audiology
#8
Jing Shen, Melinda C Anderson, Kathryn H Arehart, Pamela E Souza
Purpose: The population of the United States is aging. Those older adults are living longer than ever and have an increased desire for social participation. As a result, audiologists are likely to see an increased demand for service by older clients whose communication difficulty is caused by a combination of hearing loss and cognitive impairment. For these individuals, early detection of mild cognitive impairment is critical for providing timely medical intervention and social support...
October 27, 2016: American Journal of Audiology
https://www.readbyqxmd.com/read/27746215/developmental-hearing-loss-impedes-auditory-task-learning-and-performance-in-gerbils
#9
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
https://www.readbyqxmd.com/read/27725178/top-down-and-bottom-up-neurodynamic-evidence-in-patients-with-tinnitus
#10
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
https://www.readbyqxmd.com/read/27719856/functional-auditory-disorders
#11
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
https://www.readbyqxmd.com/read/27693765/two-novel-mitochondrial-trna-mutations-a7495g-trna-ser-ucn-and-c5577t-trna-trp-are-associated-with-seizures-and-cardiac-dysfunction
#12
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)...
November 2016: Mitochondrion
https://www.readbyqxmd.com/read/27667800/clinical-and-genetic-aspects-of-kbg-syndrome
#13
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
https://www.readbyqxmd.com/read/27658060/associations-of-various-health-ratings-with-geriatric-giants-mortality-and-life-satisfaction-in-older-people
#14
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
https://www.readbyqxmd.com/read/27655111/age-related-hearing-loss-in-older-adults-with-cognitive-impairment
#15
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
https://www.readbyqxmd.com/read/27638585/clinical-electrophysiological-and-biochemical-markers-of-peripheral-and-central-nervous-system-disease-in-canine-globoid-cell-leukodystrophy-krabbe-s%C3%A2-disease
#16
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
https://www.readbyqxmd.com/read/27630013/language-and-speech-outcomes-of-children-with-hearing-loss-and-additional-disabilities-identifying-the-variables-that-influence-performance-at-five-years-of-age
#17
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
https://www.readbyqxmd.com/read/27625615/on-the-un-importance-of-working-memory-in-speech-in-noise-processing-for-listeners-with-normal-hearing-thresholds
#18
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
https://www.readbyqxmd.com/read/27624895/neuropsychological-assessment-of-86-year-old-man-with-broca-s-aphasia-complaining-of-memory-difficulties
#19
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
https://www.readbyqxmd.com/read/27611845/age-related-sensory-impairments-and-risk-of-cognitive-impairment
#20
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)...
October 2016: Journal of the American Geriatrics Society
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