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Teresa V Mitchell
Deafness is known to affect processing of visual motion and information in the visual periphery, as well as the neural substrates for these domains. This study was designed to characterize the effects of early deafness and lifelong sign language use on visual category sensitivity of the N170 event-related potential. Images from nine categories of visual forms including upright faces, inverted faces, and hands were presented to twelve typically hearing adults and twelve adult congenitally deaf signers. Classic N170 category sensitivity was observed in both participant groups, whereby faces elicited larger amplitudes than all other visual categories, and inverted faces elicited larger amplitudes and slower latencies than upright faces...
October 19, 2016: Hearing Research
David L Horn, Jong Ho Won, Jay T Rubinstein, Lynne A Werner
OBJECTIVES: Spectral resolution is a correlate of open-set speech understanding in postlingually deaf adults and prelingually deaf children who use cochlear implants (CIs). To apply measures of spectral resolution to assess device efficacy in younger CI users, it is necessary to understand how spectral resolution develops in normal-hearing children. In this study, spectral ripple discrimination (SRD) was used to measure listeners' sensitivity to a shift in phase of the spectral envelope of a broadband noise...
October 20, 2016: Ear and Hearing
Yun-Wen Yang, Chun-Mei Zhang, Xian-Jie Huang, Xiao-Xin Zhang, Lin-Kai Zhang, Jia-Huang Li, Zi-Chun Hua
Fas-associated protein with death domain (FADD), a pivotal adaptor protein transmitting apoptotic signals, is indispensable for the induction of extrinsic apoptosis. However, overexpression of FADD can form large, filamentous aggregates, termed death effector filaments (DEFs) by self-association and initiate apoptosis independent of receptor cross-linking. A mutant of FADD, which is truncated of the C-terminal tail (m-FADD, 182-205 aa) named N-FADD (m-FADD, 1-181 aa), can dramatically up-regulate the strength of FADD self-association and increase apoptosis...
October 21, 2016: Scientific Reports
L deF Silva, V N de Lima, L P Faverani, M R de Mendonça, R Okamoto, E P Pellizzer
The purpose of this systematic review was to perform a comparative analysis of the use or not of graft material in maxillary sinus lift surgery. Relevant studies published in the last 10 years were identified through a search of the PubMed/MEDLINE, ScienceDirect, and Cochrane Library databases and were assessed against the study inclusion and exclusion criteria. The initial search resulted in 1037 articles. After applying the inclusion and exclusion criteria, 16 articles remained. Four hundred and thirty-six patients were followed up over a postoperative period ranging from 6 months to 11 years...
October 17, 2016: International Journal of Oral and Maxillofacial Surgery
Y L Zhang, Y Cheng, G P Wang, Y X Ma, P Lin
No abstract text is available yet for this article.
October 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
J Y Wang, Y Xia, C C Yang, Z Wang
Objective: To analysis the important genes and functions of cochlear hair cells with oxidative stress injury, by the construction of gene regulatory network which based on different miRNA in cochlear hair cells in vitro with oxidative stress injury, and to explore the molecular mechanisms of deafness based on oxidative stress injury. Method: The oxidative stress damage cochlear hair cell model was induced by 200 μmol/L t-BHP exposure in vitro. Small RNA deep sequencing analyzed the difference expression of miRNA and contructed gene regulatory network by 6 most significant difference miRNA...
October 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
Parthasarathi Dastidar, Koushik Sarkar
A mixed ligand approach was exploited to synthesize a new series of MnII based coordination polymers (CPs), namely, CP1 {[Mn(-dpa)(-4,4-bp)]MeOH}α, CP2 {[Mn3(-dpa)3(2,2-bp)2]}α, CP3 {[Mn3(-dpa)3(1,10-phen)2]2H2O}α, CP4 {[Mn(-dpa)(-4,4-bpe)1.5]H2O}α, CP5 {[Mn2(-dpa)2(-4,4-bpe)2]½DEF}α and CP6 {[Mn(-dpa)(-4,4-bpe)1.5]½DMA}α, [dpa = 3,5-dicarboxy phenyl azide, 2,2-bp = 2,2-bipyridine, 1,10-phen = 1,10-phenanthroline, 4,4-bpe = 1,2-bis(4-pyridyl)ethylene, 4,4-bp = 4,4-bipyridine, DEF = N,N-diethylformamide, DMA = N,N-dimethylacetamide] to develop multifuntional CPs...
October 20, 2016: Chemistry: a European Journal
Sigrid Bosteels, Michel Vandenbroeck, Geert Van Hove
New-born screening programs for congenital disorders and chronic disease are expanding worldwide and children "at risk" are identified by nationwide tracking systems at the earliest possible stage. These practices are never neutral and raise important social and ethical questions. An emergent concern is that a reflexive professionalism should interrogate the ever earlier interference in children's lives. The Flemish community of Belgium was among the first to generalize the screening for hearing loss in young children and is an interesting case to study the public justification of early interventions for families with deaf children...
October 19, 2016: Journal of Bioethical Inquiry
Juan Rodriguez-Paris, Jörg Waldhaus, Jeenal A Gordhandas, Lynn Pique, Iris Schrijver
We characterized a novel GJB2 missense variant, c.133G>A, p.Gly45Arg, and compared it with the only other variant at the same amino acid position of the connexin 26 protein (Cx26) reported to date: c.134G>A, p.Gly45Glu. Whereas both variants are associated with hearing loss and are dominantly inherited, p.Gly45Glu has been implicated in the rare fatal keratitis-ichthyosis-deafness (KID) syndrome, which results in cutaneous infections and septicemia with premature demise in the first year of life. In contrast, p...
2016: PeerJ
Bas P Hartel, Martijn J H Agterberg, Ad F Snik, Henricus P M Kunst, A John V Opstal, Arjan J Bosman, Ronald J E Pennings
OBJECTIVES: Usher syndrome is the leading cause of hereditary deaf-blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localization abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal processing on binaural hearing, including sound localization. DESIGN AND PARTICIPANTS: In this prospective study, patients were fitted with hearing aids with a nonlinear (compression) and linear amplification program...
October 19, 2016: Clinical Otolaryngology
Lianhua Sun, Xiaohua Li, Jun Shi, Xiuhong Pang, Yechen Hu, Xiaowen Wang, Hao Wu, Tao Yang
Waardenburg syndrome (WS) characterized by sensorineural hearing loss and pigmentary abnormalities is genetically heterogeneous and phenotypically variable. This study investigated the molecular etiology and genotype-phenotype correlation of WS in 36 Chinese Han deaf probands and 16 additional family members that were clinically diagnosed with WS type I (WS1, n = 8) and type II (WS2, n = 42). Mutation screening of six WS-associated genes detected PAX3 mutations in 6 (86%) of the 7 WS1 probands. Among the 29 WS2 probands, 13 (45%) and 10 (34%) were identified with SOX10 and MITF mutations, respectively...
October 19, 2016: Scientific Reports
John Patton, Carmen Brewer, Wade Chien, Jennifer J Johnston, Andrew J Griffith, Leslie G Biesecker
Variants in the unconventional myosin gene, MYO1A, have been reported to cause non-syndromic sensorineural hearing loss with a pattern of autosomal dominant inheritance. Others have challenged this association. We used a genotypic ascertainment study design to test the association of MYO1A variants with hearing loss. We evaluated MYO1A variants from a cohort of 951 individuals with exome sequencing who were not ascertained for hearing loss. Five individuals had one of two variants claimed to be associated with sensorineural hearing loss in a prior study and 33 individuals had one of 13 predicted deleterious variants...
October 19, 2016: European Journal of Human Genetics: EJHG
Shilpa Ajit Joshi, Shalomith Uppapalli, Pranav More, Madan Deshpande
Brittle cornea syndrome is a rare generalised connective tissue disorder with ocular features like keratoglobus or keratoconus, severe corneal thinning and a high risk of perforation. Various authors in different case reports and case series have brought out the fact that brittle cornea is a disorder with characteristic systemic manifestations such as deafness, joint hypermobility, hyperelasticity of skin, kyphoscoliosis and dental abnormalities alongwith ophthalmic features. We report a case of globe perforation following trivial trauma, in an individual with brittle cornea without any extraocular manifestations, posing a challenge in the diagnosis and dilemma in surgical repair of cornea, restoration of globe integrity and visual rehabilitation...
October 7, 2016: BMJ Case Reports
Thomas A Masters, John Kendrick-Jones, Folma Buss
Myosins are cytoskeletal motor proteins that use energy derived from ATP hydrolysis to generate force and movement along actin filaments. Humans express 38 myosin genes belonging to 12 classes that participate in a diverse range of crucial activities, including muscle contraction, intracellular trafficking, cell division, motility, actin cytoskeletal organisation and cell signalling. Myosin malfunction has been implicated a variety of disorders including deafness, hypertrophic cardiomyopathy, Usher syndrome, Griscelli syndrome and cancer...
October 19, 2016: Handbook of Experimental Pharmacology
Maja Svrakic, J Thomas Roland, Sean O McMenomey, Mario A Svirsky
OBJECTIVE: To describe our initial operative experience and hearing preservation results with the Advanced Bionics (AB) Mid Scala Electrode (MSE). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Sixty-three MSE implants in pediatric and adult patients were compared with age- and sex-matched 1j electrode implants from the same manufacturer. All patients were severe to profoundly deaf. INTERVENTION: Cochlear implantation with either the AB 1j electrode or the AB MSE...
October 12, 2016: Otology & Neurotology
Aida Costa, Lynn M Powell, Sally Lowell, Andrew P Jarman
The proneural gene, Atoh1, is necessary and in some contexts sufficient for early inner ear hair cell development. Its function is the subject of intensive research, not least because of the possibility that it could be used in therapeutic strategies to reverse hair cell loss in deafness. However, it is clear that Atoh1's function is highly context dependent. During inner ear development, Atoh1 is only able to promote hair cell differentiation at specific developmental stages. Outside the ear, Atoh1 is required for differentiation of a variety of other cell types, for example in the intestine and cerebellum...
October 14, 2016: Seminars in Cell & Developmental Biology
Lotte Skøt, Tina Jeppesen, Angelina Isabella Mellentin, Ask Elklit
PURPOSE: This descriptive study sought to explore barriers faced by Deaf and hard-of-hearing (D/HH) individuals in Denmark when accessing medical and psychosocial services following large-scale disasters and individual traumatic experiences. METHODS: Semi-structured interviews were conducted with nine D/HH individuals who had experienced at least one disaster or other traumatic event. RESULTS: Difficulties were encountered during interactions with first response and healthcare services, which centered on: (1) lack of Deaf awareness among professionals, (2) problems accessing interpreter services, (3) professionals relying on hearing relatives to disseminate information, and (4) professionals who were unwilling to adjust their speech or try different forms of communication...
October 17, 2016: Disability and Rehabilitation
Stephanie Kletke, Vaishnavi Batmanabane, Tianyang Dai, Ajoy Vincent, Shuning Li, Karen A Gordon, Blake C Papsin, Sharon L Cushing, Elise Héon
The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having Usher syndrome. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was done to determine the ocular phenotype...
October 15, 2016: Clinical Genetics
Maryam Beheshtian, Mojgan Babanejad, Hela Azaiez, Niloofar Bazazzadegan, Diana Kolbe, Christina Sloan-Heggen, Sanaz Arzhangi, Kevin Booth, Marzieh Mohseni, Kathy Frees, Mohammad Hossein Azizi, Ahmad Daneshi, Mohammad Farhadi, Kimia Kahrizi, Richard Jh Smith, Hossein Najmabadi
A significant contribution to the causes of hereditary hearing impairment comes from genetic factors. More than 120 genes and 160 loci have been identified to be involved in hearing impairment. Given that consanguine populations are more vulnerable to most inherited diseases, such as hereditary hearing loss (HHL), the genetic picture of HHL among the Iranian population, which consists of at least eight ethnic subgroups with a high rate of intermarriage, is expected to be highly heterogeneous. Using an electronic literature review through various databases such as PubMed, MEDLINE, and Scopus, we review the current picture of HHL in Iran...
October 1, 2016: Archives of Iranian Medicine
Cengiz Gemici, Gokhan Yaprak, Hasan Fevzi Batirel, Mahmut Ilhan, Alpaslan Mayadagli
BACKGROUND: Locoregional recurrence is a major problem in esophageal cancer patients treated with definitive concomitant chemoradiotherapy. Approximately half of the patients fail locoregionally. We analyzed the impact of enlarged radiation field size and higher radiation dose incorporated to chemoradiotherapy on oncologic outcome. METHODS: Seventy-four consecutive patients with histologically proven nonmetastatic squamous or adenocarcinoma of the esophagus were included in this retrospective analysis...
October 13, 2016: World Journal of Surgical Oncology
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