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https://www.readbyqxmd.com/read/29165436/cochlear-implant-in-prelingually-deafened-oralist-adults-speech-perception-outcomes-subjective-benefits-and-quality-of-life-improvement
#1
F Forli, G Turchetti, G Giuntini, S Bellelli, S Fortunato, L Bruschini, M R Barillari, S Berrettini
The aim of this study is to report our results in a group of prelingually deafened adults, who followed an oralist rehabilitation programme, and submitted to cochlear implant at our institution. We evaluated 30 prelingually deafened adult patients, 18 males and 12 females, median age 35 years, of a group of 36 prelingually deafened adult patients consecutively submitted to unilateral cochlear implantation at the ENT Unit of the University of Pisa. After implantation, patients achieved significant benefits in terms of speech perception skills, including the ability to have telephone conversations in some cases, quality of life and their own perception of disability...
October 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/29159249/visual-impairment-reversal-with-oral-acitretin-therapy-in-keratitis-ichthyosis-deafness-kid-syndrome
#2
Christopher M Wolfe, Alexander Davis, Tarek S Shaath, George F Cohen
No abstract text is available yet for this article.
November 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/29159054/delayed-access-to-bilateral-input-alters-cortical-organization-in-children-with-asymmetric-hearing
#3
Melissa Jane Polonenko, Blake Croll Papsin, Karen Ann Gordon
Bilateral hearing in early development protects auditory cortices from reorganizing to prefer the better ear. Yet, such protection could be disrupted by mismatched bilateral input in children with asymmetric hearing who require electric stimulation of the auditory nerve from a cochlear implant in their deaf ear and amplified acoustic sound from a hearing aid in their better ear (bimodal hearing). Cortical responses to bimodal stimulation were measured by electroencephalography in 34 bimodal users and 16 age-matched peers with normal hearing, and compared with the same measures previously reported for 28 age-matched bilateral implant users...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29157937/pure-word-deafness-following-left-temporal-damage-behavioral-and-neuroanatomical-evidence-from-a-new-case
#4
Chiara Maffei, Rita Capasso, Giulia Cazzolli, Cesare Colosimo, Flavio Dell'Acqua, Francesca Piludu, Marco Catani, Gabriele Miceli
Pure Word Deafness (PWD) is a rare disorder, characterized by selective loss of speech input processing. Its most common cause is temporal damage to the primary auditory cortex of both hemispheres, but it has been reported also following unilateral lesions. In unilateral cases, PWD has been attributed to the disconnection of Wernicke's area from both right and left primary auditory cortex. Here we report behavioral and neuroimaging evidence from a new case of left unilateral PWD with both cortical and white matter damage due to a relatively small stroke lesion in the left temporal gyrus...
October 23, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29155975/induced-cortical-responses-require-developmental-sensory-experience
#5
Prasandhya Astagiri Yusuf, Peter Hubka, Jochen Tillein, Andrej Kral
Sensory areas of the cerebral cortex integrate the sensory inputs with the ongoing activity. We studied how complete absence of auditory experience affects this process in a higher mammal model of complete sensory deprivation, the congenitally deaf cat. Cortical responses were elicited by intracochlear electric stimulation using cochlear implants in adult hearing controls and deaf cats. Additionally, in hearing controls, acoustic stimuli were used to assess the effect of stimulus mode (electric versus acoustic) on the cortical responses...
November 16, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29155350/the-pmca-pumps-in-genetically-determined-neuronal-pathologies
#6
REVIEW
Tito Calì, Marisa Brini, Ernesto Carafoli
Ca(2+) signals regulate most aspects of animal cell life. They are of particular importance to the nervous system, in which they regulate specific functions, from neuronal development to synaptic plasticity. The homeostasis of cell Ca(2+) must thus be very precisely regulated: in all cells Ca(2+) pumps transport it from the cytosol to the extracellular medium (the Plasma Membrane Ca(2+) ATPases, hereafter referred to as PMCA pumps) or to the lumen of intracellular organelles (the Sarco/Endoplasmatic Reticulum Ca(2+) ATPase and the Secretory Pathway Ca(2+) ATPase, hereafter referred to as SERCA and SPCA pumps, respectively)...
November 16, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/29155276/cortical-plasticity-as-a-basis-of-phantom-limb-pain-fact-or-fiction
#7
J Andoh, C Milde, J W Tsao, H Flor
Cortical reorganization has been proposed as a major factor involved in phantom pain with prior nociceptive input to the deafferented region and input from the non-deafferented cortex creating neuronal activity that is perceived as phantom pain. There is substantial evidence that these processes play a role in neuropathic pain, although causal evidence is lacking. Recently it has been suggested that a maintenance of the cortical representation of the former hand area is related to phantom pain. Although interesting, evidence for this process is so far scarce...
November 16, 2017: Neuroscience
https://www.readbyqxmd.com/read/29154648/nimble-negotiators-how-theory-of-mind-tom-interconnects-with-persuasion-skills-in-children-with-and-without-tom-delay
#8
Candida C Peterson, Virginia Slaughter, Henry M Wellman
Persuasion is an essential social skill. Yet its development and underpinnings are poorly understood. In 2 studies, a total of 167 children aged 3 to 12 years took theory of mind (ToM) tests and participated in unscripted, seminaturalistic persuasive conversations. Children were typically developing (TD) or had deafness or autism spectrum disorder (ASD). High-level, informationally rich persuasive arguments increased with age in all groups in both studies, as did ToM. In both studies, ToM scores predicted persuasion skill over and above age, language ability, and deafness/ASD status...
November 20, 2017: Developmental Psychology
https://www.readbyqxmd.com/read/29149379/efficacy-of-systemic-and-intratympanic-corticosteroid-combination-therapy-versus-intratympanic-or-systemic-therapy-in-patients-with-idiopathic-sudden-sensorineural-hearing-loss-a-randomized-controlled-trial
#9
Mohammadtaghi Khorsandi Ashtiani, Farzad Firouzi, Shahin Bastaninejad, Sasan Dabiri, Sevil Nasirmohtaram, Niloufar Saeedi, Hossein Ghazavi, Leyla Sahebi
OBJECTIVE: The present study was conducted to compare the rates of recovery from idiopathic sudden deafness after the treatment with oral and intratympanic corticosteroids in both mono and combination therapies. STUDY DESIGN: Triple-blind randomized clinical trial. SETTINGS: Tertiary referral hospital. SUBJECTS AND METHODS: A total of 112 patients who were admitted to the ENT emergency department randomly divided into three groups: an oral corticosteroid plus intratympanic placebo (systemic corticosteroid monotherapy group); an intratympanic corticosteroid plus oral placebo group (IT monotherapy group); and a combination therapy group (IT plus systemic combination group)...
November 17, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/29142287/targeted-next-generation-sequencing-in-italian-patients-with-usher-syndrome-phenotype-genotype-correlations
#10
Chiara M Eandi, Laura Dallorto, Roberta Spinetta, Maria Pia Micieli, Mario Vanzetti, Alessandro Mariottini, Ilaria Passerini, Francesca Torricelli, Camilla Alovisi, Cristiana Marchese
We report results of DNA analysis with next generation sequencing (NGS) of 21 consecutive Italian patients from 17 unrelated families with clinical diagnosis of Usher syndrome (4 USH1 and 17 USH2) searching for mutations in 11 genes: MYO7A, CDH23, PCDH15, USH1C, USH1G, USH2A, ADGVR1, DFNB31, CLRN1, PDZD7, HARS. Likely causative mutations were found in all patients: 25 pathogenic variants, 18 previously reported and 7 novel, were identified in three genes (USH2A, MYO7A, ADGRV1). All USH1 presented biallelic MYO7A mutations, one USH2 exhibited ADGRV1 mutations, whereas 16 USH2 displayed USH2A mutations...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29141319/-homozygous-ectonucleotide-pyrophosphatase-phosphodiesterase-1-variants-in-a-girl-with-hypophosphatemic-rickets-and-literature-review
#11
Z Q Liu, X B Chen, F Y Song, K Gao, M F Qiu, Y Qian, M Du
Objective: To investigate the clinical features and genetic characteristics of patients with ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) gene variants. Method: The clinical data of a patient with ENPP1 homozygous variants from Capital Institute of Pediatrics was collected, the related literature was searched from China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, National Center from Biotechnology Information and PubMed by using search term "ENPP1" , "hypophosphatemic rickets" ...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29140768/public-health-burden-of-hearing-impairment-and-the-promise-of-genomics-and-environmental-research-a-case-study-in-ghana-africa
#12
Samuel Mawuli Adadey, Gordon Awandare, Goffrey Kwabla Amedofu, Ambroise Wonkam
Hearing impairment (HI) is one of the most disabling conditions of major global health burden that contributes adversely to the social and economic development of a country, if not managed properly. A proper assessment of the nationwide burden and etiology of HI is instrumental in the prevention, treatment, and management of the condition. This article sought to perform an expert review of HI in Ghana to determine the present knowledge of its burden and possible causes of the condition. A literature search was conducted in PubMed using the following keywords: "hearing loss" OR "hearing impairment" OR "deafness" AND "Ghana...
November 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/29138749/auris-system-providing-vibrotactile-feedback-for-hearing-impaired-population
#13
Felipe Alves Araujo, Fabricio Lima Brasil, Allison Candido Lima Santos, Luzenildo de Sousa Batista Junior, Savio Pereira Fonseca Dutra, Carlos Eduardo Coelho Freire Batista
Deafness, an issue that affects millions of people around the globe, is manifested in different intensities and related to many causes. This impairment negatively affects different aspects of the social life of the deaf people, and music-centered situations (concerts, religious events, etc.) are obviously not inviting for them. The Auris System was conceived to provide the musical experimentation for people who have some type of hearing loss. This system is able to extract musical information from audio and create a representation for music pieces using different stimuli, a new media format to be interpreted by other senses than the hearing...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29137413/abnormal-neural-activities-of-directional-brain-networks-in-patients-with-long-term-bilateral-hearing-loss
#14
Long-Chun Xu, Gang Zhang, Yue Zou, Min-Feng Zhang, Dong-Sheng Zhang, Hua Ma, Wen-Bo Zhao, Guang-Yu Zhang
The objective of the study is to provide some implications for rehabilitation of hearing impairment by investigating changes of neural activities of directional brain networks in patients with long-term bilateral hearing loss. Firstly, we implemented neuropsychological tests of 21 subjects (11 patients with long-term bilateral hearing loss, and 10 subjects with normal hearing), and these tests revealed significant differences between the deaf group and the controls. Then we constructed the individual specific virtual brain based on functional magnetic resonance data of participants by utilizing effective connectivity and multivariate regression methods...
October 13, 2017: Oncotarget
https://www.readbyqxmd.com/read/29135879/cochlear-implantation-in-children-with-congenital-unilateral-deafness-a-case-series
#15
Rainer Linus Beck, Antje Aschendorff, Frederike Hassepaß, Thomas Wesarg, Stefanie Kröger, Till F Jakob, Susan Arndt
OBJECTIVES: Cochlear implant is regarded as a treatment option for hearing rehabilitation of adults with unilateral sensorineural hearing loss. A clear benefit has been experienced in regard to speech comprehension in noise, localization, and quality of life. The aim of this study was to investigate the benefit of cochlear implantation for children with congenital unilateral hearing loss. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center; cochlear implant program...
December 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29135878/single-sided-deafness-impact-of-cochlear-implantation-on-speech-perception-in-complex-noise-and-on-auditory-localization-accuracy
#16
Julia Döge, Uwe Baumann, Tobias Weissgerber, Tobias Rader
OBJECTIVE: To assess auditory localization accuracy and speech reception threshold (SRT) in complex noise conditions in adult patients with acquired single-sided deafness, after intervention with a cochlear implant (CI) in the deaf ear. STUDY DESIGN: Nonrandomized, open, prospective patient series. SETTING: Tertiary referral university hospital. PATIENTS: Eleven patients with late-onset single-sided deafness (SSD) and normal hearing in the unaffected ear, who received a CI...
December 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29135863/effectiveness-in-rehabilitation-of-current-wireless-cros-technology-in-experienced-bone-anchored-implant-users
#17
Hillary A Snapp, Michael E Hoffer, Xuezhong Liu, Suhrud M Rajguru
OBJECTIVE: To compare the effectiveness of current contralateral routing of signal technology (CROS) to bone-anchored implants in experienced bone-anchored implant users with unilateral severe-profound sensorineural hearing loss. DESIGN: Prospective, within-subject repeated-measures comparison study. SETTING: Tertiary referral center. PATIENTS: Adult, English-speaking patients (n = 12) with severe-profound unilateral sensorineural hearing loss implanted with a bone-anchored implant for the indication of single-sided deafness...
December 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29132260/the-psychological-and-social-consequences-of-single-sided-deafness-in-adulthood
#18
Laura Lucas, Roulla Katiri, Pádraig Thomas Kitterick
OBJECTIVES: This study examined the subjective psychological and social effects of highly asymmetric hearing loss (single-sided deafness [SSD]) in adults. DESIGN: Three group interviews were conducted using the critical incident technique and analysed using an inductive thematic analysis. STUDY SAMPLE: Eight adults with a clinical diagnosis of a moderately severe hearing loss or greater in one ear and normal or near-normal hearing in the other ear...
November 13, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/29131113/speech-production-quality-of-cochlear-implant-users-with-respect-to-duration-and-onset-of-hearing-loss
#19
Suzan Ruff, Tobias Bocklet, Elmar Nöth, Joachim Müller, Eva Hoster, Maria Schuster
PURPOSE: To assess whether postlingual onset and shorter duration of deafness before cochlear implant (CI) provision predict higher speech intelligibility results of CI users. METHODS: For an objective judgement of speech intelligibility, we used an automatic speech recognition system computing the word recognition rate (WR) of 50 adult CI users and 50 age-matched control individuals. All subjects were recorded reading a standardized text. Subjects were divided into three groups: pre- or perilingual deafness (A), both >2 years before implantation, postlingual deafness <2 years before implantation (B), or postlingual deafness >2 years before implantation (C)...
2017: ORL; Journal for Oto-rhino-laryngology and its related Specialties
https://www.readbyqxmd.com/read/29130579/a-homozygous-deleterious-cdk10-mutation-in-a-patient-with-agenesis-of-corpus-callosum-retinopathy-and-deafness
#20
Vincent J Guen, Simon Edvardson, Nitay D Fraenkel, Aviva Fattal-Valevski, Chaim Jalas, Irene Anteby, Avraham Shaag, Talia Dor, David Gillis, Eitan Kerem, Jacqueline A Lees, Pierre Colas, Orly Elpeleg
The primary cilium is a key organelle in numerous physiological and developmental processes. Genetic defects in the formation of this non-motile structure, in its maintenance and function, underlie a wide array of ciliopathies in human, including craniofacial, brain and heart malformations, and retinal and hearing defects. We used exome sequencing to study the molecular basis of disease in an 11-year-old female patient who suffered from growth retardation, global developmental delay with absent speech acquisition, agenesis of corpus callosum and paucity of white matter, sensorineural deafness, retinitis pigmentosa, vertebral anomalies, patent ductus arteriosus, and facial dysmorphism reminiscent of STAR syndrome, a suspected ciliopathy...
November 12, 2017: American Journal of Medical Genetics. Part A
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