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https://www.readbyqxmd.com/read/29332068/the-role-of-preoperative-steroids-for-hearing-preservation-cochlear-implantation-results-of-a-randomized-controlled-trial
#1
Jafri Kuthubutheen, Samidha Joglekar, Leah Smith, Lendra Friesen, Kari Smilsky, Tara Millman, Amy Ng, David Shipp, Harvey Coates, Christoph Arnoldner, Julian Nedzelski, Joseph Chen, Vincent Lin
OBJECTIVES: To determine whether preoperative steroids can improve hearing outcomes in cochlear implantation (CI). METHODS: This is a randomized controlled trial involving 30 postlingual deaf CI patients. Subjects had preoperative thresholds of better than or equal to 80 dB at 125 and 250 Hz, and better than or equal to 90 dB at 500 and 1,000 Hz. The subjects were randomized to a control group, an oral steroid group (receiving 1 mg/kg/day of prednisolone for 6 days prior to surgery), or a transtympanic steroid group (receiving a single dose of 0...
January 13, 2018: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/29331002/genetics-of-human-hereditary-hearing-impairment
#2
Rahat Meena, Muhammad Ayub
Hereditary hearing impairment is heterogeneous type of disorder which can be caused due to environmental as well as genetical factors. Two distinct types of hereditary hearing loss are syndromic or non-syndromic. Non-syndromic hearing loss is further categorized as autosomal recessive, autosomal dominant, X-linked and mitochondrial deafness. Autosomal recessive occurs more frequently as compared to autosomal dominant. Mutations in various genes are responsible for hereditary hearing impairment. To date, about 99 autosomal recessives and 67 autosomal dominant genes for deafness have been discovered...
October 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29330314/developmental-disability-at-school-age-and-difficulty-obtaining-follow-up-data
#3
Lex W Doyle, Peter J Anderson, Alice Burnett, Catherine Callanan, Marion McDonald, Marie Hayes, Gillian Opie, Elizabeth Carse, Jeanie L Y Cheong
BACKGROUND: The relationship of developmental disability rates with difficulty obtaining follow-up data is unclear. With this study, we aimed to determine if children who attended research follow-up assessments with more difficulty had more disability at school age, compared with those who attended with less difficulty, and to establish the relationship between follow-up and disability rates. METHODS: Two groups, comprising 219 consecutive survivors born at <28 weeks' gestation or at <1000 g birth weight in the state of Victoria, Australia, in 2005, and 218 term-born, normal birth weight controls were assessed at 8 years of age for neurodevelopmental disability (any of IQ <-1 SD, cerebral palsy, blindness, or deafness)...
January 12, 2018: Pediatrics
https://www.readbyqxmd.com/read/29326171/abdominal-vagal-afferents-modulate-the-brain-transcriptome-and-behaviors-relevant-to-schizophrenia
#4
Melanie Klarer, Jean-Philippe Krieger, Juliet Richetto, Ulrike Weber-Stadlbauer, Lydia Günther, Christine Winter, Myrtha Arnold, Wolfgang Langhans, Urs Meyer
Reduced activity of vagal efferents has long been implicated in schizophrenia and appears to be responsible for diminished parasympathetic activity and associated peripheral symptoms such as low heart rate variability and cardiovascular complications in affected individuals. In contrast, only little attention has been paid to the possibility that impaired afferent vagal signaling may be relevant for the disorder's pathophysiology as well. The present study explored this hypothesis using a model of subdiaphragmatic vagal deafferentation (SDA) in male rats...
January 11, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29325119/mir-96-is-required-for-normal-development-of-the-auditory-hindbrain
#5
Tina Schlüter, Christina Berger, Elena Rosengauer, Pascal Fieth, Constanze Krohs, Kathy Ushakov, Karen P Steel, Karen B Avraham, Alexander Hartmann, Felix Felmy, Hans Gerd Nothwang
The peripheral deafness gene Mir96 is expressed in both the cochlea and central auditory circuits. To investigate whether it plays a role in the auditory system beyond the cochlea, we characterized homozygous Dmdo/Dmdo mice with a point mutation in miR-96. Anatomical analysis demonstrated a significant decrease in volume of auditory nuclei in Dmdo/Dmdo mice. This decrease resulted from decreased cell size. Non-auditory structures in the brainstem of Dmdo/Dmdo mice or auditory nuclei of the congenital deaf Cldn14-/- mice revealed no such differences...
January 9, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29324475/bilateral-cochlear-implants-maximizing-expected-outcomes
#6
Kate E Willis, Nathan J Blum, Stephanie A Waryasz, Marilyn Augustyn
Sonia is a 4 1/12-year-old girl with Waardenburg syndrome and bilateral sensorineural hearing loss who had bilateral cochlear implants at 2 7/12 years of age. She is referred to Developmental-Behavioral Pediatrics by her speech/language pathologist because of concerns that her language skills are not progressing as expected after the cochlear implant. At the time of the implant, she communicated using approximately 20 signs and 1 spoken word (mama). At the time of the evaluation (18 months after the implant) she had approximately 70 spoken words (English and Spanish) and innumerable signs that she used to communicate...
January 10, 2018: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/29323325/an-ode-to-gene-edits-that-prevent-deafness
#7
Fyodor Urnov
No abstract text is available yet for this article.
January 11, 2018: Nature
https://www.readbyqxmd.com/read/29321651/auditory-neural-activity-in-congenitally-deaf-mice-induced-by-infrared-neural-stimulation
#8
Xiaodong Tan, Israt Jahan, Yingyue Xu, Stuart Stock, Changyow Claire Kwan, Carmen Soriano, Xianghui Xiao, Jaime García-Añoveros, Bernd Fritzsch, Claus-Peter Richter
To determine whether responses during infrared neural stimulation (INS) result from the direct interaction with spiral ganglion neurons (SGNs), we tested three genetically modified deaf mouse models: Atoh1-cre; Atoh1 f/f (Atoh1 conditional knockout, CKO), Atoh1-cre; Atoh1 f/kiNeurog1 (Neurog1 knockin, KI), and the Vglut3 knockout (Vglut3 -/-) mice. All animals were exposed to tone bursts and clicks up to 107 dB (re 20 µPa) and to INS, delivered with a 200 µm optical fiber. The wavelength (λ) was 1860 nm, the radiant energy (Q) 0-800 µJ/pulse, and the pulse width (PW) 100-500 µs...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29321625/cerebellar-grey-matter-modifications-in-lower-limb-amputees-not-using-prosthesis
#9
Antonella Di Vita, Maddalena Boccia, Liana Palermo, Federico Nemmi, Marco Traballesi, Stefano Brunelli, Roberto De Giorgi, Gaspare Galati, Cecilia Guariglia
Plastic brain changes following peripheral deafferentation, in particular those following limb amputations, are well-documented, with significant reduction of grey matter (GM) in the sensory-motor cerebral areas representing the amputated limb. However, few studies have investigated the role played by the use of a prosthesis in these structural brain modifications. Here we hypothesized that using a functional prosthesis that allows individuals to perform actions may reduce grey matter reduction. We investigated the brain structural reorganization following lower limb amputation by using a Voxel Based Morphometry (VBM) analysis of structural magnetic resonance imaging (MRI) in 8 right-handed individuals with lower limb amputation (LLA) fitted with prostheses (LLAwp), compared to 6 LLA who had never used a prosthesis (LLAnp)...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29321360/ellis-van-creveld-syndrome-and-profound-deafness-resulted-by-sequence-variants-in-the-evc-evc2-and-tmc1-genes
#10
Muhammad Umair, Heide Seidel, Ishtiaq Ahmed, Asmat Ullah, Tobias B Haack, Bader Alhaddad, Abid Jan, Afzal Rafique, Tim M Strom, Farooq Ahmad, Thomas Meitinger, Wasim Ahmad
Ellis-van Creveld syndrome is an autosomal recessive skeletal dysplasia primarily characterized by the features such as disproportionate dwarfism, short ribs, short limbs, dysplastic nails, cardiovascular malformations, post-axial polydactyly (PAP) (bilateral) of hands and feet. EVC/EVC2 located in head-to-head arrangement on chromosome 4p16 are the causative genes for EvC syndrome. In the study, we present two families, A and B, with Pakistani and Republic of Kosovo origin, respectively. They showed features of EvC syndrome and were clinically and genetically characterized...
December 2017: Journal of Genetics
https://www.readbyqxmd.com/read/29320647/long-term-effects-of-inhaled-budesonide-for-bronchopulmonary-dysplasia
#11
Dirk Bassler, Eric S Shinwell, Mikko Hallman, Pierre-Henri Jarreau, Richard Plavka, Virgilio Carnielli, Christoph Meisner, Corinna Engel, Alexander Koch, Karen Kreutzer, Johannes N van den Anker, Matthias Schwab, Henry L Halliday, Christian F Poets
BACKGROUND: The long-term effects on neurodevelopment of the use of inhaled glucocorticoids in extremely preterm infants for the prevention or treatment of bronchopulmonary dysplasia are uncertain. METHODS: We randomly assigned 863 infants (gestational age, 23 weeks 0 days to 27 weeks 6 days) to receive early (within 24 hours after birth) inhaled budesonide or placebo. The prespecified secondary long-term outcome was neurodevelopmental disability among survivors, defined as a composite of cerebral palsy, cognitive delay (a Mental Development Index score of <85 [1 SD below the mean of 100] on the Bayley Scales of Infant Development, Second Edition, with higher scores on the scale indicating better performance), deafness, or blindness at a corrected age of 18 to 22 months...
January 11, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29319875/association-between-sensory-impairment-and-dementia-in-older-adults-evidence-from-china
#12
Yanan Luo, Ping He, Chao Guo, Gong Chen, Ning Li, Xiaoying Zheng
OBJECTIVES: To determine the association between sensory impairment and dementia in Chinese older adults. DESIGN: Cross-sectional. SETTING: Older adults in 31 provinces of China. PARTICIPANTS: Individuals aged 65 and older (N = 250,752). MEASUREMENTS: Psychiatrists ascertained dementia based on the International Classification of Diseases, 10th Revision. Sensory impairment was measured as only hearing impairment, only vision impairment, and combined sensory impairment (combined hearing and vision impairment)...
January 10, 2018: Journal of the American Geriatrics Society
https://www.readbyqxmd.com/read/29316760/-analysis-on-occupational-noise-induced-hearing-loss-of-different-type-workers-in-underground-mining
#13
Q C Liu, C H Duo, Z Wang, K Yan, J Zhang, W Xiong, M Zhu
Objective: To investigate hearing loss status of blasters, drillers mechanics and so on in underground mining, and put forward suggestion diagnosis of occupational explosive deafness and occupational deafness. Methods: Underground excavation workers in a metal mine were recruited in this study, those with a history of ear disease and non-occupational deafness were all excluded. Finally, the features of pure tone audiometry of 459 noise-exposed workers were analyzed. Results: High-frequency hearing loss occurred on 351workers and the positive detection rate was 74...
November 20, 2017: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/29316750/-study-the-impacts-of-diagnosis-on-occupational-noise-induced-deafness-after-bring-into-the-different-high-frequency-hearing-threshold-weighted-value
#14
L J Xue, A C Yang, H Chen, W X Huang, J J Guo, X Y Liang, Z Q Chen, Q L Zheng
Objective: Study of the results and the degree on occupational noise-induced deafness in-to the different high frequency hearing threshold weighted value, in order to provide theoretical basis for the re-vision of diagnostic criteria on occupational noise-induced deafness. Methods: A retrospective study was con-ducted to investigate the cases on the diagnosis of occupational noise-induced deafness in Guangdong province hospital for occupational disease prevention and treatment from January 2016 to January 2017...
November 20, 2017: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/29315194/relations-between-self-reported-executive-functioning-and-speech-perception-skills-in-adult-cochlear-implant-users
#15
Aaron C Moberly, Tirth R Patel, Irina Castellanos
HYPOTHESIS: As a result of their hearing loss, adults with cochlear implants (CIs) would self-report poorer executive functioning (EF) skills than normal-hearing (NH) peers, and these EF skills would be associated with performance on speech recognition tasks. BACKGROUND: EF refers to a group of high order neurocognitive skills responsible for behavioral and emotional regulation during goal-directed activity, and EF has been found to be poorer in children with CIs than their NH age-matched peers...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29315183/middle-fossa-approach-for-cochlear-implantation
#16
Wojciech Gawęcki, Michał Karlik, Łukasz Borucki, Maciej Wróbel, Olgierd Maciej Stieler, Witold Szyfter
OBJECTIVE: To present qualification criteria, surgery, and results of cochlear implantation via the middle fossa approach. STUDY DESIGN: A retrospective analysis of medical charts of patients. SETTING: Cochlear implant program in academic medical center. PATIENTS: Six adults after middle ear surgery due to chronic otitis media with bilateral deafness or profound hearing loss. INTERVENTION: Analysis aimed to identify patients qualified for cochlear implantation via middle fossa approach and to present surgery and result...
February 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29313147/voice-discrimination-by-adults-with-cochlear-implants-the-benefits-of-early-implantation-for-vocal-tract-length-perception
#17
Yael Zaltz, Raymond L Goldsworthy, Liat Kishon-Rabin, Laurie S Eisenberg
Cochlear implant (CI) users find it extremely difficult to discriminate between talkers, which may partially explain why they struggle to understand speech in a multi-talker environment. Recent studies, based on findings with postlingually deafened CI users, suggest that these difficulties may stem from their limited use of vocal-tract length (VTL) cues due to the degraded spectral resolution transmitted by the CI device. The aim of the present study was to assess the ability of adult CI users who had no prior acoustic experience, i...
January 8, 2018: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/29311684/a-severely-short-statured-girl-with-47-xx-%C3%A2-%C3%A2-14-46-xx-upd-14-mat-mosaicism
#18
Kikumi Ushijima, Syuichi Yatsuga, Takako Matsumoto, Akie Nakamura, Maki Fukami, Masayo Kagami
The predominant symptoms of trisomy 14 mosaicism are prenatal and postnatal growth failure, ear abnormalities, congenital heart disease, developmental delay, and genitourinary abnormalities. Maternal uniparental disomy of chromosome 14 (upd(14)mat) presents discernible clinical features such as prenatal and postnatal growth failure, hypotonia, precocious puberty, and obesity. Given the small number of previously reported patients with a combination of trisomy 14 mosaicism and upd(14)mat, the detailed clinical features of these patients remain to be elucidated...
January 9, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29304186/fixed-and-adaptive-beamforming-improves-speech-perception-in-noise-in-cochlear-implant-recipients-equipped-with-the-med-el-sonnet-audio-processor
#19
Clemens Honeder, Rudolfs Liepins, Christoph Arnoldner, Hana Šinkovec, Alexandra Kaider, Erich Vyskocil, Dominik Riss
OBJECTIVE: To determine the impact of the fixed and adaptive beamforming technology of the new MED-EL SONNET cochlear implant audio processor on speech perception in noise. METHODS: The study cohort comprises 18 postlingually deafened adult cochlear implant recipients with at least six months of experience. Speech reception thresholds were measured with the Oldenburg Sentence Test in continuous, speech-shaped noise. Target sentences were presented in front of the listener, with noise sources placed at -135° and 135°, respectively...
2018: PloS One
https://www.readbyqxmd.com/read/29301908/deletion-of-a-long-range-dlx5-enhancer-disrupts-inner-ear-development-in-mice
#20
Kenneth R Johnson, Leona H Gagnon, Cong Tian, Chantal M Longo-Guess, Benjamin E Low, Michael V Wiles, Amy E Kiernan
Distal enhancers are thought to play important roles in the spatiotemporal regulation of gene expression during embryonic development, but few predicted enhancer elements have been shown to affect transcription of their endogenous genes or to alter phenotypes when disrupted. Here, we demonstrate that a 123.6 kb deletion within the mouse Slc25a13 gene is associated with reduced transcription of Dlx5, a gene located 660 kb away. Mice homozygous for the Slc25a13 deletion mutation (named hyperspin, hspn) have malformed inner ears and are deaf with balance defects, whereas previously reported Slc25a13 knockout mice showed no phenotypic abnormalities...
January 3, 2018: Genetics
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