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Francesca Yoshie Russo, Daniele De Seta, Ghizlene Lahlou, Stéphanie Borel, Yann Nguyen, Didier Bouccara, Olivier Sterkers, Daniele Bernardeschi, Isabelle Mosnier
Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions...
March 1, 2018: Otolaryngology—Head and Neck Surgery
Ricardo F Bento, Fayez Bahmad, Miguel A Hippolyto, Sady S Da Costa
PURPOSE OF REVIEW: Effective hearing rehabilitation with cochlear implantation is challenging in developing countries, and this review focuses on strategies for childhood profound sensorineural hearing loss care in South America. RECENT FINDINGS: Most global hearing loss exists in developing countries; optimal cost-effective management strategies are essential in these environments. This review aims to assess and discuss the challenges of cochlear implantation effectiveness in South America...
March 15, 2018: Current Opinion in Otolaryngology & Head and Neck Surgery
Clifford E Kashtan, Jie Ding, Guido Garosi, Laurence Heidet, Laura Massella, Koichi Nakanishi, Kandai Nozu, Alessandra Renieri, Michelle Rheault, Fang Wang, Oliver Gross
Mutations in the genes COL4A3, COL4A4, and COL4A5 affect the synthesis, assembly, deposition, or function of the collagen IV α345 molecule, the major collagenous constituent of the mature mammalian glomerular basement membrane. These mutations are associated with a spectrum of nephropathy, from microscopic hematuria to progressive renal disease leading to ESRD, and with extrarenal manifestations such as sensorineural deafness and ocular anomalies. The existing nomenclature for these conditions is confusing and can delay institution of appropriate nephroprotective therapy...
March 15, 2018: Kidney International
Sara Salime, Zied Riahi, Soukaina Elrharchi, Lamiae Elkhattabi, Hicham Charoute, Halima Nahili, Hassan Rouba, Mostafa Kabine, Crystel Bonnet, Christine Petit, Abdelhamid Barakat
Deafness and myopia syndrome is characterized by moderate-profound, bilateral, congenital or prelingual deafness and high myopia. Autosomal recessive non-syndromic hearing loss is one of the most prevalent human genetic sensorineural defects. Myopia is by far the most common human eye disorder that is known to have a clear heritable component. The analysis of the two exons of SLITRK6 gene in a Moroccan family allowed us to identify a novel single deleterious mutation c.696delG, p.Trp232Cysfs*10 at homozygous state in the exon 2 of the SLITRK6, a gene reported to cause deafness and myopia in various populations...
March 15, 2018: Gene
Dexter R F Irvine
Perceptual learning, improvement in discriminative ability as a consequence of training, is one of the forms of sensory system plasticity that has driven profound changes in our conceptualization of sensory cortical function. Psychophysical and neurophysiological studies of auditory perceptual learning have indicated that the characteristics of the learning, and by implication the nature of the underlying neural changes, are highly task specific. Some studies in animals have indicated that recruitment of neurons to the population responding to the training stimuli, and hence an increase in the so-called cortical "area of representation" of those stimuli, is the substrate of improved performance, but such changes have not been observed in other studies...
March 12, 2018: Hearing Research
Carolien Zwiers, Mirjam Ea Scheffer-Rath, Enrico Lopriore, Masja de Haas, Helen G Liley
BACKGROUND: Exchange transfusion and phototherapy have traditionally been used to treat jaundice and avoid the associated neurological complications. Because of the risks and burdens of exchange transfusion, intravenous immunoglobulin (IVIg) has been suggested as an alternative therapy for alloimmune hemolytic disease of the newborn (HDN) to reduce the need for exchange transfusion. OBJECTIVES: To assess the effect and complications of IVIg in newborn infants with alloimmune HDN on the need for and number of exchange transfusions...
March 18, 2018: Cochrane Database of Systematic Reviews
P Kushalnagar, A Engleman, G Sadler
OBJECTIVE: To assess whether mode of communication and patient centered communication (PCC) with physicians were associated with the likelihood of deaf smokers inquiring about lung cancer screening. METHODS: An accessible health survey including questions about PCC, modes of communication, smoking status and lung cancer screening was administered in American Sign Language (HINTS-ASL) to a nationwide sample of deaf adults from February to August 2017. Of 703 deaf adults who answered the lung screening question, 188 were 55-80 years old...
March 5, 2018: Patient Education and Counseling
Yan Hao, Dawei Chen, Zhiguo Zhang, Ping Zhou, Yunxia Cao, Zhaolian Wei, Xiaofeng Xu, Beili Chen, Weiwei Zou, Mingrong Lv, Dongmei Ji, Xiaojin He
Hearing loss may place a heavy burden on the patient and patient's family. Given the high incidence of hearing loss among newborns and the huge cost of treatment and care (including cochlear implantation), prenatal diagnosis is strongly recommended. Termination of the fetus may be considered as an extreme outcome to the discovery of a potential deaf fetus, and therefore preimplantation genetic diagnosis has become an important option for avoiding the birth of affected children without facing the risk of abortion following prenatal diagnosis...
April 2018: Oncology Letters
Lozina Shoaib, Sharifullah Khan, Muhammad Azeem Abbas, Ahmad Salman
OBJECTIVE: To mitigate the communication barriers of profound hearing-impaired children by enabling their word articulation ability. METHODS: This pre-experimental pilot study was conducted from September 2016 to March 2017 at the National Special Education Centre for Hearing Impaired Children, Islamabad, Pakistan, and comprised deaf children of both genders aged 5-8 years. A specially designed software application for lip-reading was employed to help the subjects articulate words...
March 2018: JPMA. the Journal of the Pakistan Medical Association
David S Sharlin, Lily Ng, François Verrey, Theo J Visser, Ye Liu, Rafal T Olszewski, Michael Hoa, Heike Heuer, Douglas Forrest
Transmembrane proteins that mediate the cellular uptake or efflux of thyroid hormone potentially provide a key level of control over neurodevelopment. In humans, defects in one such protein, solute carrier SLC16A2 (MCT8) are associated with psychomotor retardation. Other proteins that transport the active form of thyroid hormone triiodothyronine (T3) or its precursor thyroxine (T4) have been identified in vitro but the wider significance of such transporters in vivo is unclear. The development of the auditory system requires thyroid hormone and the cochlea is a primary target tissue...
March 13, 2018: Scientific Reports
Uwe Wolfrum, Kerstin Nagel-Wolfrum
The human Usher syndrome (USH) is a complex, rare disease manifesting in its most common form of inherited deaf-blindness. Due to the heterogeneous manifestation of the clinical symptoms, three clinical types (USH1-3) are distinguished according to the severity of the disease pattern. For a correct diagnosis, in addition to the auditory tests in early newborn screening, ophthalmological examinations and molecular genetic analysis are important. Ten known USH genes encode proteins, which are from heterogeneous protein families, interact in functional protein networks...
March 2018: Klinische Monatsblätter Für Augenheilkunde
Yi Jiang, Song Gao, Lihua Wu, Xiaohua Jin, Tao Deng, Ligang Wang, Shasha Huang, Xue Gao, Juan Chen, Dongyi Han, Huafang Gao, Pu Dai
To analyze the spectrum and founder effect of TMC1 mutations in patients with non-syndromic deafness in the Xiamen area. Sporadic pedigrees were detected by targeted next-generation sequencing, and 110 unrelated patients from Xiamen Special Education School were analyzed through Sanger sequencing for the TMC1 gene. In total, 53 SNPs were designed to analyze the haplotypes of the TMC1 c.2050G>C mutation. The probands of three families were found to be homozygous for TMC1 c.2050G>C, and their parents were all heterozygous for the TMC1 c...
April 2018: American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics
Vincent G Wettstein, Rudolf Probst
BACKGROUND: Postlingual single-sided deafness (SSD) is defined as normal hearing in one ear and severely impaired hearing in the other ear. A right ear advantage and dominance of the left hemisphere are well established findings in individuals with normal hearing and speech processing. Therefore, it seems plausible that a right ear advantage would exist in patients with SSD. METHODS: The audiometric database was searched to identify patients with SSD. Results from the German monosyllabic Freiburg word test and four-syllabic number test in quiet were evaluated...
April 2018: Otology & Neurotology
Joshua Shaw
This essay considers one argument used to defend parents who use preimplantation genetic diagnosis (PGD) to select for deafness and other disabilities. Some bioethicists have argued that a distinction should be drawn between genetically modifying embryos to possess disabilities and using PGD to select embryos that already present markers of them, and that the former is unethical because it inflicts avoidable harms onto the resulting children, whereas the latter is permissible because it allows children with potentially impaired abilities to exist...
April 2018: New Bioethics: a Multidisciplinary Journal of Biotechnology and the Body
Jenny L Singleton, Elena T Remillard, Tracy L Mitzner, Wendy A Rogers
PURPOSE: Technology holds great potential to support Deaf individuals as they age into older adulthood. However, it is unclear to what extent Deaf seniors are using technology in everyday life or whether they experience challenges in using technology. The current study explored technology use among older Deaf adults with regard to attitudes, adoption style, and frequency of use for a wide range of technologies, including assistive technologies (ATs) for persons with hearing loss and general, everyday technologies...
March 9, 2018: Disability and Rehabilitation. Assistive Technology
Pedro J Tomaselli, Mahima Kapoor, Andrea Cortese, James M Polke, Alexander M Rossor, Mary M Reilly
Mutations in mitofusin 2 (MFN2) are the most common cause of axonal Charcot Marie Tooth disease (CMT2) (Polke; 2011). Additional features known to occasionally occur with MFN2-related disorders are optic atrophy, pyramidal signs, scoliosis and deafness (Zuchner, 2006; Feely, 2011; Bombelli, 2014). Cognitive impairment has been reported in a small number of patients with MFN2 mutations (Del Bo, 2008; Genari, 2011; Tufano, 2015).
March 9, 2018: Journal of the Peripheral Nervous System: JPNS
Nicole Rosskothen-Kuhl, Heika Hildebrandt, Ralf Birkenhäger, Robert-Benjamin Illing
Neuron-glia interactions contribute to tissue homeostasis and functional plasticity in the mammalian brain, but it remains unclear how this is achieved. The potential of central auditory brain tissue for stimulation-dependent cellular remodeling was studied in hearing-experienced and neonatally deafened rats. At adulthood, both groups received an intracochlear electrode into the left cochlea and were continuously stimulated for 1 or 7 days after waking up from anesthesia. Normal hearing and deafness were assessed by auditory brainstem responses (ABRs)...
2018: Frontiers in Cellular Neuroscience
Badi Aldosari, Jean-Marc Thomassin
Objective: The purpose of this study is to evaluate our experience with endoscopic repair of ossicular discontinuity at the incudostapedial joint, with or without an intact stapes suprastructure, and present our hearing results. We classify results based on the causative pathology, the type of ossiculoplasty, and type of lesion. We demonstrate the ability to endoscopically place a total ossicular replacement prosthesis (TORP), measuring 4.25 mm, between the stapes footplate and the incus remnant to reestablish ossicular continuity...
September 2017: World Journal of Otorhinolaryngology—Head and Neck Surgery
Adam Schembri, Jordan Fenlon, Kearsy Cormier, Trevor Johnston
This paper examines the possible relationship between proposed social determinants of morphological 'complexity' and how this contributes to linguistic diversity, specifically via the typological nature of the sign languages of deaf communities. We sketch how the notion of morphological complexity, as defined by Trudgill (2011), applies to sign languages. Using these criteria, sign languages appear to be languages with low to moderate levels of morphological complexity. This may partly reflect the influence of key social characteristics of communities on the typological nature of languages...
2018: Frontiers in Psychology
Olivier Yavari-Sartakhti, Frédérique Briche, Daniel Jost, Nicolas Michaud, Michel Bignand, Jean-Pierre Tourtier
Deafness frequently observed in explosion victims, currently following terrorist attack, is a barrier to communication between victims and first responders. This may result in a delay in the initial triage and evacuation. In such situations, Paris Fire Brigade (Paris, France) proposes the use of assistance cards to help conscious, but deafened patients at the site of an attack where there may be numerous victims. Yavari-Sartakhti O , Briche F , Jost D , Michaud N , Bignand M , Tourtier JP . A new triage support tool in case of explosion...
March 8, 2018: Prehospital and Disaster Medicine
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