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https://www.readbyqxmd.com/read/28806779/a-mutation-in-nischarin-causes-otitis-media-via-limk1-and-nf-%C3%AE%C2%BAb-pathways
#1
Michael Crompton, Tom Purnell, Hayley E Tyrer, Andrew Parker, Greg Ball, Rachel E Hardisty-Hughes, Richard Gale, Debbie Williams, Charlotte H Dean, Michelle M Simon, Ann-Marie Mallon, Sara Wells, Mahmood F Bhutta, Martin J Burton, Hilda Tateossian, Steve D M Brown
Otitis media (OM), inflammation of the middle ear (ME), is a common cause of conductive hearing impairment. Despite the importance of the disease, the aetiology of chronic and recurrent forms of middle ear inflammatory disease remains poorly understood. Studies of the human population suggest that there is a significant genetic component predisposing to the development of chronic OM, although the underlying genes are largely unknown. Using N-ethyl-N-nitrosourea mutagenesis we identified a recessive mouse mutant, edison, that spontaneously develops a conductive hearing loss due to chronic OM...
August 14, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28806706/auditory-cortex-lesions-impair-contralateral-tone-pattern-detection-under-informational-masking
#2
Lisa Prilop, Alexander Gutschalk
Impaired hearing contralateral to unilateral auditory-cortex lesions is typically only observed under conditions of perceptual competition, such as dichotic presentation or speech in noise. It remains unclear, however, if the source of this effect is direct competition in frequency-specific neurons, or if enhanced processing load in more distant frequencies can also impair auditory detection. To evaluate this question, we studied a group of patients with unilateral auditory-cortex lesions (N = 14, six left-hemispheric (LH), eight right-hemispheric (RH); four females; age range 26-72 years) and a control group (N = 25; 15 females; age range 18-76 years) with a target-detection task in presence of a multi-tone masker, which can produce informational masking...
July 19, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/28806341/impact-of-cochlear-implantation-on-cognitive-functions-of-older-adults-pilot-test-results
#3
Dona M P Jayakody, Peter L Friedland, Esmeralda Nel, Ralph N Martins, Marcus D Atlas, Hamid R Sohrabi
BACKGROUND: A significant relationship between hearing loss and cognitive impairment has been previously reported. Overall, improvement in speech perception in quiet and quality of life has been observed after cochlear implantation. However, the impact of hearing loss treatment using cochlear implantation on cognitive functions is yet to be fully elucidated. OBJECTIVE: To investigate the impact of cochlear implantation on cognitive and psychological functions of older adults...
September 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28806339/cognitive-evaluation-of-cochlear-implanted-adults-using-codex-and-moca-screening-tests
#4
Emmanuèle Ambert-Dahan, Shirley Routier, Lucie Marot, Didier Bouccara, Olivier Sterkers, Evelyne Ferrary, Isabelle Mosnier
OBJECTIVE: The relationship between hearing loss and cognitive function has already been established. The objective of our study was to determine whether the two short cognitive tests, COgnitive Disorders EXamination (CODEX) and Montreal Cognitive Assessment (MoCA), could be used in daily clinical practice to detect cognitive impairment, and its changes after cochlear implantation. PATIENTS: Eighteen patients with severe to profound postlingual progressive hearing loss (mean age ± SEM: 64 ± 3...
September 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28805616/prenatal-diagnosis-and-molecular-cytogenetic-characterization-of-mosaicism-for-a-small-supernumerary-marker-chromosome-derived-from-chromosome-16
#5
Chih-Ping Chen, Tsang-Ming Ko, Schu-Rern Chern, Peih-Shan Wu, Shin-Wen Chen, Shih-Ting Lai, Chien-Wen Yang, Chen-Wen Pan, Wayseen Wang
OBJECTIVE: We present prenatal diagnosis and molecular cytogenetic characterization of a small supernumerary marker chromosome (sSMC) derived from chromosome 16. CASE REPORT: A 28-year-old woman underwent amniocentesis at 17 weeks of gestation because of abnormal maternal serum screening for Down syndrome. Amniocentesis revealed a karyotype of 47,XY,+mar[5]/46,XY[9]. Parental karyotypes were normal. Prenatal ultrasound findings were unremarkable. Array comparative genomic hybridization (aCGH) analysis of cultured amniocytes revealed a de novo 16% gene dosage increase of 16q11...
August 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28805100/older-adults-with-a-combination-of-vision-and-hearing-impairment-experience-higher-rates-of-cognitive-impairment-functional-dependence-and-worse-outcomes-across-a-set-of-quality-indicators
#6
Jacob G S Davidson, Dawn M Guthrie
OBJECTIVES: Hearing and vision impairment were examined across several health-related outcomes and across a set of quality indicators (QIs) in home care clients with both vision and hearing loss (or dual sensory impairment [DSI]). METHOD: Data collected using the Resident Assessment Instrument for Home Care (RAI-HC) were analyzed in a sample of older home care clients. The QIs represent the proportion of clients experiencing negative outcomes (e.g., falls, social isolation)...
August 1, 2017: Journal of Aging and Health
https://www.readbyqxmd.com/read/28802353/hearing-loss-in-children-with-growth-hormone-deficiency
#7
John S Muus, Forest W Weir, Kathryn L Kreicher, Deborah A Bowlby, Christopher M Discolo, Ted A Meyer
OBJECTIVE: Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. METHODS: Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28802351/exome-sequencing-identifies-a-novel-missense-mutation-of-wfs1-as-the-cause-of-non-syndromic-low-frequency-hearing-loss-in-a-chinese-family
#8
Zhijie Niu, Yong Feng, Zhengmao Hu, Jiada Li, Jie Sun, Hongsheng Chen, Chufeng He, Xueping Wang, Lu Jiang, Yalan Liu, Xinzhang Cai, Lili Wang, Yuxiang Cai, Xuezhong Liu, Lingyun Mei
OBJECTIVE: Autosomal dominant non-syndromic low-frequency sensorineural hearing loss (LFSNHL) DFNA6/14/38 is an uncommon type of hearing loss that classically affects low frequencies of 2000 Hz and below, demonstrating an ascending configuration. The current study aimed to investigate the cause of LFSNHL in a five-generation Chinese family. METHODS: The phenotype of the Chinese family was characterized using audiologic testing and pedigree analysis. The combined approach of array screening and whole-exome sequencing was used to identify the disease-causing gene in this family...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28796850/the-economic-impact-of-adult-hearing-loss-a-systematic-review
#9
Matthew G Huddle, Adele M Goman, Faradia C Kernizan, Danielle M Foley, Carrie Price, Kevin D Frick, Frank R Lin
Importance: Hearing impairment (HI) is highly prevalent in older adults and has been associated with adverse health outcomes. However, the overall economic impact of HI is not well described. Objective: The goal of this review was to summarize available data on all relevant costs associated with HI among adults. Evidence Review: A literature search of PubMed, Embase, the Cochrane Library, CINAHL, and Scopus was conducted in August 2015. For this systematic review, data extraction and quality assessment were performed by 2 independent reviewers...
August 10, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28792529/discrepancy-between-self-assessed-hearing-status-and-measured-audiometric-evaluation
#10
So Young Kim, Hyung-Jong Kim, Min-Su Kim, Bumjung Park, Jin-Hwan Kim, Hyo Geun Choi
OBJECTIVE: The purpose of this study was to examine the difference between self-reported hearing status and hearing impairment assessed using conventional audiometry. The associated factors were examined when a concordance between self-reported hearing and audiometric measures was lacking. METHODS: In total, 19,642 individuals ≥20 years of age who participated in the Korea National Health and Nutrition Examination Surveys conducted from 2009 through 2012 were enrolled...
2017: PloS One
https://www.readbyqxmd.com/read/28790873/parent-child-interaction-therapy-current-perspectives
#11
REVIEW
Corey C Lieneman, Laurel A Brabson, April Highlander, Nancy M Wallace, Cheryl B McNeil
Parent-Child Interaction Therapy (PCIT) is an empirically supported intervention originally developed to treat disruptive behavior problems in children between the ages of 2 and 7 years. Since its creation over 40 years ago, PCIT has been studied internationally with various populations and has been found to be an effective intervention for numerous behavioral and emotional issues. This article summarizes progress in the PCIT literature over the past decade (2006-2017) and outlines future directions for this important work...
2017: Psychology Research and Behavior Management
https://www.readbyqxmd.com/read/28780178/the-effects-of-noise-exposure-and-musical-training-on-suprathreshold-auditory-processing-and-speech-perception-in-noise
#12
Ingrid Yeend, Elizabeth Francis Beach, Mridula Sharma, Harvey Dillon
Recent animal research has shown that exposure to single episodes of intense noise causes cochlear synaptopathy without affecting hearing thresholds. It has been suggested that the same may occur in humans. If so, it is hypothesized that this would result in impaired encoding of sound and lead to difficulties hearing at suprathreshold levels, particularly in challenging listening environments. The primary aim of this study was to investigate the effect of noise exposure on auditory processing, including the perception of speech in noise, in adult humans...
July 14, 2017: Hearing Research
https://www.readbyqxmd.com/read/28777514/our-experience-in-the-treatment-of-idiopathic-sensorineural-hearing-loss-isnhl-effect-of-combination-therapy-with-hbo%C3%A2-and-vasodilator-infusion-therapy
#13
Barbora Kratochvílovà, Oliver Profant, Jaromír Astl, Richard Holý
Idiopathic sensorineural hearing loss (ISNHL) is a hearing impairment that occurs suddenly with onset over a period of 24 to 72 hours, affecting at least three adjacent frequencies with hearing loss of 30 decibels or more, caused by damage to the inner ear or auditory nerve without clear reasons. The treatment of ISNHL is still a hot topic. In a retrospective study, we examined 88 patients with ISNHL (2008-2013). The objective of the study was to compare the effects of two different types of therapy: vasodilator infusion treatment combined with hyperbaric oxygen (HBO₂) therapy vs...
November 2016: Undersea & Hyperbaric Medicine: Journal of the Undersea and Hyperbaric Medical Society, Inc
https://www.readbyqxmd.com/read/28776506/neural-decoding-of-attentional-selection-in-multi-speaker-environments-without-access-to-clean-sources
#14
James O'Sullivan, Zhuo Chen, Jose Herrero, Guy M McKhann, Sameer A Sheth, Ashesh D Mehta, Nima Mesgarani
OBJECTIVE: People who suffer from hearing impairments can find it difficult to follow a conversation in a multi-speaker environment. Current hearing aids can suppress background noise; however, there is little that can be done to help a user attend to a single conversation amongst many without knowing which speaker the user is attending to. Cognitively controlled hearing aids that use auditory attention decoding (AAD) methods are the next step in offering help. Translating the successes in AAD research to real-world applications poses a number of challenges, including the lack of access to the clean sound sources in the environment with which to compare with the neural signals...
August 4, 2017: Journal of Neural Engineering
https://www.readbyqxmd.com/read/28767192/expanding-the-phenotype-of-dst-related-disorder-a-case-report-suggesting-a-genotype-phenotype-correlation
#15
Gerarda Cappuccio, Michele Pinelli, Annalaura Torella, Marianna Alagia, Renata Auricchio, Annamaria Staiano, Vincenzo Nigro, Nicola Brunetti-Pierri
The gene DST encodes for the large protein BPAG1 involved in hemidesmosomes. Its alternative splicing gives rise to tissue-enriched isoforms in brain, muscle, and skin. The few patients described so far with bi-allelic mutations in the DST gene have either a skin phenotype of epidermolysis bullosa simplex or a neurological phenotype. Here, we report a 17-year-old female individual presenting with a more complex phenotype consisting of both skin and neuronal involvement, in addition to several previously unreported findings, such as iris heterochromia, cataract, hearing impairment, syringomyelia, behavioral, and gastrointestinal issues, osteoporosis, and growth hormone deficiency...
August 2, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28764460/recognition-of-asynchronous-auditory-visual-speech-by-younger-and-older-listeners-a-preliminary-study
#16
Sandra Gordon-Salant, Grace H Yeni-Komshian, Peter J Fitzgibbons, Hannah M Willison, Maya S Freund
This study examined the effects of age and hearing loss on recognition of speech presented when the auditory and visual speech information was misaligned in time (i.e., asynchronous). Prior research suggests that older listeners are less sensitive than younger listeners in detecting the presence of asynchronous speech for auditory-lead conditions, but recognition of speech in auditory-lead conditions has not yet been examined. Recognition performance was assessed for sentences and words presented in the auditory-visual modalities with varying degrees of auditory lead and lag...
July 2017: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/28763424/congenital-cytomegalovirus-infection-after-a-multiple-birth-pregnancy
#17
Einat Shmueli, Eran Hadar, Joseph Pardo, Joseph Attias, Jacob Amir, Efraim Bilavsky
BACKGROUND: Congenital cytomegalovirus (cCMV) infection is an important cause of hearing loss and neurodevelopment delay. While data on vertical transmission and neonatal outcome after singleton pregnancy with cCMV is well established, only scarce reports have addressed cCMV in multiple birth pregnancies. Furthermore, no studies have yet compared the outcome after birth and long-term follow up of children with cCMV born after a singleton vs. multiple pregnancy. METHODS: Infant outcome after birth of symptomatic vs...
July 31, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28762345/assessment-of-dental-caries-and-periodontal-status-in-institutionalized-hearing-impaired-children-in-khordha-district-of-odisha
#18
Avinash Jnaneswar, Goutham Bala Subramaniya, Jayashree Pathi, Kunal Jha, Vinay Suresan, Gunjan Kumar
INTRODUCTION: Over 5% of the world's population has disabling hearing loss. The oral health of the disabled may be disused for the reason of the disabling condition, a challenging disease or the limited access to oral health care. OBJECTIVES: The objectives of the study were to assess the prevalence of dental caries and periodontal status of institutionalized hearing impaired (HI) children in Khordha district of Odisha. MATERIALS AND METHODS: A descriptive cross-sectional study on the HI children was conducted in Khordha district, Odisha...
July 2017: Journal of the Indian Society of Pedodontics and Preventive Dentistry
https://www.readbyqxmd.com/read/28761208/sudden-bilateral-sensorineural-hearing-loss-following-postpartum-hemorrhage-a-case-report
#19
Sara Mirzaeian, Sedigheh Ayati, Asieh Maleki
The prevalence of bilateral sudden sensorineural hearing loss (SSNHL) is less than 5% and the etiology of most cases is unknown. Due to many structural and functional similarities between the kidney and inner ear, many conditions, diseases, and drugs have both renal and cochlear effects and toxicities. There are several reports of SSNHL in patients with CRF, uraemic patient, hemodialysis treatment, and ARF. Here, we report a rare manifestation of SSNHL following severe postpartum hemorrhage that has simultaneous renal failure and cochlear impairment...
July 2017: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/28760859/sound-evoked-activity-influences-myelination-of-brainstem-axons-in-the-trapezoid-body
#20
James L Sinclair, Matthew J Fischl, Olga Alexandrova, Martin Heß, Benedikt Grothe, Christian Leibold, Conny Kopp-Scheinpflug
Plasticity of myelination represents a mechanism to tune the flow of information by balancing functional requirements with metabolic and spatial constraints. The auditory system is heavily myelinated and operates at the upper limits of action potential generation frequency and speed observed in the mammalian CNS. This study aimed to characterize the development of myelin within the trapezoid body, a central auditory fiber tract, and determine the influence sensory experience has on this process in mice of both sexes...
July 31, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
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