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https://www.readbyqxmd.com/read/28635507/cochlear-implant-effectiveness-in-postlingual-single-sided-deaf-individuals-what-s-the-point
#1
Mareike Finke, Hanna Bönitz, Björn Lyxell, Angelika Illg
OBJECTIVES: By extending the indication criteria for cochlear implants (CI), the population of CI candidates increased in age, as well as range and type of hearing loss. This qualitative study identified factors that contributed to seek CI treatment in single-sided deaf individuals and gained insights how single-sided deafness (SSD) and hearing with a CI affect their lives. DESIGN: An open-ended questionnaire and a standardised inventory (IOI-HA) were used. Qualitative data reflecting the reasons to seek CI treatment and the individual experiences after CI switch-on were collected...
March 5, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28635225/-modification-factors-associated-with-maternally-inherited-non-syndromic-hearing-loss
#2
W J Hong, B J Zheng, J F Qian, H Wu, H Jin, Y T Zhu
Mutations in the mitochondrial DNA have been certified to be one of the most important causes of maternally inherited sensorineural hearing loss. Among these, mitochondrial 12S rRNA1555A>G, 1494C>T and other mutations are associated with both nonsyndromic and drug induced hearing loss caused by aminoglycosides. Individuals carrying 1555A>G or 1494C>T mutation have a variety of clinical manifestations, which implies that the 1555A>G or 1494C>T mutation is a chief factor underlying the development of deafness but insufficient to produce the clinical phenotype...
June 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28632987/the-usher-syndrome-type-iiib-histidyl-trna-synthetase-mutation-confers-temperature-sensitivity
#3
Jamie A Abbott, Ethan Guth, Cindy Kim, Cathy Regan, Victoria M Siu, Charles Anthony Rupar, Borries Demeler, Christopher S Francklyn, Susan M Robey-Bond
Histidyl-tRNA synthetase (HARS) is a highly conserved translation factor that plays an essential role in protein synthesis. HARS has been implicated in the human syndromes Charcot-Marie-Tooth (CMT) Type 2W and Type IIIB Usher (USH3B). The USH3B mutation, which encodes an Y454S substitution in HARS, is inherited in an autosomal recessive fashion and associated with childhood deafness, blindness and episodic hallucinations during acute illness. The biochemical basis of the pathophysiologies linked to USH3B is currently unknown...
June 20, 2017: Biochemistry
https://www.readbyqxmd.com/read/28630650/a-maternally-inherited-8-05%C3%A2-mb-xq21-deletion-associated-with-choroideremia-deafness-and-mental-retardation-syndrome-in-a-male-patient
#4
Siying Liang, Nan Jiang, Shuo Li, Xiaohu Jiang, Dongyi Yu
BACKGROUND: Deletions in Xq21 cause various congenital defects in males including choroideremia, deafness and mental retardation, depending on their size and gene content. Until now only a limited number of patients with Xq21 deletions has been reported. CASE PRESENTATION: Here we describe a 17-year-old male with choroideremia, deafness, and mental retardation syndrome. Using SNP arrays, an 8.05 Mb deletion in Xq21 was identified inherited from the apparently healthy mother...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/28626432/visible-social-interactions-do-not-support-the-development-of-false-belief-understanding-in-the-absence-of-linguistic-input-evidence-from-deaf-adult-homesigners
#5
Deanna L Gagne, Marie Coppola
Congenitally deaf individuals exhibit enhanced visuospatial abilities relative to normally hearing individuals. An early example is the increased sensitivity of deaf signers to stimuli in the visual periphery (Neville and Lawson, 1987a). While these enhancements are robust and extend across a number of visual and spatial skills, they seem not to extend to other domains which could potentially build on these enhancements. For example, congenitally deaf children, in the absence of adequate language exposure and acquisition, do not develop typical social cognition skills as measured by traditional Theory of Mind tasks...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28623039/the-acoustic-characteristics-of-the-voice-in-cochlear-implanted-children-a-longitudinal-study
#6
Yajing Wang, Faya Liang, Jinshan Yang, Xueyuan Zhang, Jiahao Liu, Yiqing Zheng
OBJECTIVE: The purpose of this study was to characterize changes in the voice and vowel articulation of prelingually deaf children after cochlear implantation. METHODS: In this study, the patient group included 30 prelingually deaf children who underwent unilateral cochlear implantation at 4-6 years of age. The control group included normally hearing children of the same age. All deaf children had follow-ups before cochlear implantation and at 1, 3, 6, 12, and 24 months after implantation...
June 13, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28621418/single-cell-analysis-of-the-inner-ear-sensory-organs
#7
Ofer Yizhar-Barnea, Karen B Avraham
The inner ear is composed of a complex mixture of cells, which together allow organisms to hear and maintain balance. The cells in the inner ear, which undergo an extraordinary process of development, have only recently begun to be studied on an individual level. As it has recently become clear that individual cells, previously considered to be of uniform character, may differ dramatically from each other, the need to study cell-to-cell variation, along with distinct transcriptional and regulatory signatures, has taken hold in the scientific community...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/28619607/revisiting-syntactic-development-in-deaf-and-hearing-children-from-a-dependency-approach-comment-on-dependency-distance-a-new-perspective-on-syntactic-patterns-in-natural-languages-by-haitao-liu-et-al
#8
https://www.readbyqxmd.com/read/28618809/binaural-sensitivity-in-children-who-use-bilateral-cochlear-implants
#9
Erica Ehlers, Matthew J Goupell, Yi Zheng, Shelly P Godar, Ruth Y Litovsky
Children who are deaf and receive bilateral cochlear implants (BiCIs) perform better on spatial hearing tasks using bilateral rather than unilateral inputs; however, they underperform relative to normal-hearing (NH) peers. This gap in performance is multi-factorial, including the inability of speech processors to reliably deliver binaural cues. Although much is known regarding binaural sensitivity of adults with BiCIs, less is known about how the development of binaural sensitivity in children with BiCIs compared to NH children...
June 2017: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/28616898/smoking-and-the-eye-what-qu%C3%A3-bec-teenagers-know-and-fear
#10
Julie Brûlé, Benoît Tousignant, Stéphanie Marcotte, Marie-Christelle Moreau
BACKGROUND: Although most people associate smoking with lung cancer and heart disease, few are aware of the impact of smoking on ocular health. Studies have suggested a better knowledge of this association might promote higher quit rates, particularly in teenagers. The purpose of our study was to determine the knowledge of teenagers about the effects of smoking on ocular health and the fear associated with several tobacco-related health conditions. METHODS: A self-administered questionnaire was distributed to 180 high school students aged 14 to 17 years...
June 14, 2017: Clinical & Experimental Optometry: Journal of the Australian Optometrical Association
https://www.readbyqxmd.com/read/28616232/treatment-of-sudden-hearing-loss-using-electro-acupuncture
#11
Ya-Ching Chang, Kwok-Ying Chan
OBJECTIVES: Sudden neurosensory deafness is the second debilitating disease in China. In fact, the current treatment is limited to hearing aids, assistive devices and cochlear implants. However, some patients might not be suitable for surgery or even hearing aids. METHODS: Hereby, we report a pediatric case of sudden sensorineural hearing loss accompanied by otalgia and blocked ear sensation. RESULTS: He firstly failed with conventional treatment but later his hearing symptoms were improved with our electro-acupuncture therapy according to pure tone audiometry findings and clinical responses...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28615054/end-stage-kidney-disease-in-patient-with-epidermolysis-bullosa-what-are-the-treatment-options-case-report
#12
Michał Małecki, Maciej Domański, Kazimierz Ciechanowski
BACKGROUND: Epidermolysis bullosa is a group of diseases caused by mutations in genes for proteins responsible for cells' anchorage at the dermo-epidermal junction. Their common feature are dysfunctional or even absent connections between cells. The typical clinical sign is the formation of blisters, with possible excessive scarring, in response to minimal skin irritation. End stage renal disease may be one of the comorbidities in patients with epidermolysis bullosa. The implementation of renal replacement therapy may be very difficult in this population...
June 14, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28614229/familial-clustering-of-congenital-deafness-patent-ductus-arteriosus-eisenmenger-complex-and-differential-cyanosis-a-case-report
#13
Ting-Wei Lin, Chih-Wei Tseng, Chi-Yao Huang, Kuo-Yang Wang, Kae-Woei Liang
RATIONALE: Few studies had reported syndromes that include patent ductus arteriosus (PDA) with Eisenmenger syndrome and congenital deafness clustered in male siblings without facial, skeletal, or mental abnormalities. PATIENT CONCERNS: Two brothers, who were deaf and had PDA with Eisenmenger complex, were first seen at our Cardiology clinic at the ages of 25 and 41, respectively. They presented with progressive dyspnea on exertion. Upon physical examination, both brothers had clubbing and/or cyanotic toes, normal fingers, and without facial, skeletal, ophthalmological, or mental abnormalities...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28609304/suprameatal-cochlear-implantation-in-a-charge-patient-with-a-novel-chd7-variant-and-kallmann-syndrome-phenotype-a-case-report
#14
Akira Ganaha, Tetsuya Tono, Tadashi Kaname, Kumiko Yanagi, Teruyuki Higa, Shunsuke Kondo, Hiroyuki Maeda, Mikio Suzuki
OBJECTIVE: We present the clinical findings, technique of the suprameatal cochlear implantation, postoperative auditory results, and genetic analysis of the CHD7 gene. PATIENT: A 19-year-old Japanese woman was referred because of progressive hearing loss since early childhood. She had used verbal language for the main mode of communication until the age of 17. Examination revealed coloboma, heart defect, choanal atresia, genital hypoplasia, and deafness, which was diagnosed as CHARGE syndrome...
June 12, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28607883/evaluation-of-requirements-for-staging-the-procedure-of-reconstruction-of-middle-ear-after-canal-wall-down-mastoidectomy
#15
C Ravishankar, R K Datta
Cholesteatoma is a progressive destructive ear disease which can affect any age group. It has been found to be more severe in children and young adults. It erodes the surrounding bone of middle ear, mastoid and ossicles. It causes partial to total deafness, unpleasant smelling discharge, pain, tinnitus, vertigo and facial paralysis. It can even cause meningitis, brain abscess and death. The post-operative outcome of hearing, and the state of the reconstructed middle-ear cavity after concurrent and staged reconstruction of middle ear after canal wall down mastoidectomy was studied in 30 ears with middle-ear cholesteatoma...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28607224/evaluation-of-focused-multipolar-stimulation-for-cochlear-implants-a-preclinical-safety-study
#16
Robert K Shepherd, Andrew K Wise, Ya Lang Enke, Paul M Carter, James B Fallon
OBJECTIVE: Cochlear implants (CIs) have a limited number of independent stimulation channels due to the highly conductive nature of the fluid-filled cochlea. Attempts to develop highly focused stimulation to improve speech perception in CI users includes the use of simultaneous stimulation via multiple current sources. Focused multipolar (FMP) stimulation is an example of this approach and has been shown to reduce interaction between stimulating channels. However, compared with conventional biphasic current pulses generated from a single current source, FMP is a complex stimulus that includes extended periods of stimulation before charge recovery is achieved, raising questions on whether chronic stimulation with this strategy is safe...
June 13, 2017: Journal of Neural Engineering
https://www.readbyqxmd.com/read/28604578/long-term-outcomes-of-cochlear-implantation-in-children-with-congenital-cytomegalovirus-infection
#17
Haruo Yoshida, Haruo Takahashi, Yukihiko Kanda, Kyoko Kitaoka, Minoru Hara
OBJECTIVE: To investigate the role of the developmental delay often observed in children with congenital cytomegalovirus (CMV) infection on the improvement of language understanding after cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. PATIENTS: Sixteen children with severe and/or profound hearing loss due to congenital CMV infection (CMV group) and 107 congenitally deaf children (168 ears) without CMV infection as the cause of deafness (non-CMV group)...
June 9, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28604027/load-theory-behind-the-wheel-perceptual-cognitive-load-effects
#18
Gillian Murphy, Ciara M Greene
Perceptual Load Theory has been proposed as a resolution to the longstanding early versus late selection debate in cognitive psychology. There is much evidence in support of Load Theory but very few applied studies, despite the potential for the model to shed light on everyday attention and distraction. Using a driving simulator, the effect of perceptual and cognitive load on drivers' visual search was assessed. The findings were largely in line with Load Theory, with reduced distractor processing under high perceptual load, but increased distractor processing under high cognitive load...
June 12, 2017: Canadian Journal of Experimental Psychology, Revue Canadienne de Psychologie Expérimentale
https://www.readbyqxmd.com/read/28603488/hypothesis-of-k-recycling-defect-is-not-a-primary-deafness-mechanism-for-cx26-gjb2-deficiency
#19
REVIEW
Hong-Bo Zhao
K(+)-recycling defect is a long-standing hypothesis for deafness mechanism of Connexin26 (Cx26, GJB2) mutations, which cause the most common hereditary deafness and are responsible for >50% of nonsyndromic hearing loss. The hypothesis states that Cx26 deficiency may disrupt inner ear gap junctions and compromise sinking and recycling of expelled K(+) ions after hair cell excitation, causing accumulation of K(+)-ions in the extracellular space around hair cells producing K(+)-toxicity, which eventually induces hair cell degeneration and hearing loss...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28602887/cross-modal-recruitment-of-auditory-and-orofacial-areas-during-sign-language-in-a-deaf-subject
#20
Juan Martino, Carlos Velasquez, Javier Vázquez-Bourgon, Enrique Marco de Lucas, Elsa Gomez
BACKGROUND: Modern signed languages of the deaf are fully expressive, natural human languages that are perceived visually and produced manually.The literature contains little data concerning human brain organization in conditions of deficient sensory information such as deafness. CASE DESCRIPTION: Here, we describe an original case of a deaf-mute patient with a left temporo-insular low-grade glioma that was operated. The patient underwent awake surgery with intraoperative electrical stimulation mapping, allowing for the first time in the literature, to directly study the cortical and subcortical organization of signed language...
June 5, 2017: World Neurosurgery
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