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Newborn hearing

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https://www.readbyqxmd.com/read/29130354/delivery-of-adeno-associated-viral-vectors-in-adult-mammalian-inner-ear-cell-subtypes-without-auditory-dysfunction
#1
Yong Tao, Mingqian Huang, Yilai Shu, Adam Ruprecht, Hongyang Wang, Yong Tang, Luk H Vandenberghe, Qiuju Wang, Guangping Gao, Wei-Jia Kong, Zheng-Yi Chen
Hearing loss, including genetic hearing loss, is one of the most common forms of sensory deficits in human with limited options of treatment. Adeno-associated virus (AAV)-mediated gene transfer has been shown to effectively recover auditory functions in mouse models of genetic deafness when delivered at neonatal stages. However, the mouse cochlea is still developing at those time points whereas in human the newborn inner ears are already fully mature. For effective gene therapy to treat genetic deafness, it is necessary to determine whether or not AAV-mediated therapy can be equally effective in the fully mature mouse inner ear without causing damages to the inner ear...
November 12, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/29116590/detection-of-congenital-cytomegalovirus-in-newborns-using-nucleic-acid-amplification-techniques-and-its-public-health-implications
#2
REVIEW
Guoyu Liu, Rong Hai, Fenyong Liu
Human cytomegalovirus (HCMV), a herpesvirus, is an important human pathogen that causes asymptomatic infections in healthy or immunocompetent individuals but can lead to severe and potentially life-threatening complications in immune-immature individuals such as neonates or immune-compromised patients such as organ-transplant recipients and HIV-positive individuals. Congenital HCMV infection represents a significant public health issue and poses substantial healthcare and economic burden to society. This virus causes the most common viral congenital infection worldwide, and is the leading non-genetic cause of sensorineural hearing loss in children in developed countries...
October 2017: Virologica Sinica
https://www.readbyqxmd.com/read/29106875/regional-differences-of-turkey-in-risk-factors-of-newborn-hearing-loss
#3
Ozlem Konukseven, Sule Kaya, Aydan Genc, Nuray Bayar Muluk, Figen Suren Basar, Gunay Kirkim, Ulku Tuncer, Erkan Karatas, Cigdem Topcu, Hilal Bolat, Ilknur Dincol
OBJECTIVE: The aim of this study was to discover Turkish regional differences in the risk factors of newborn hearing loss. METHOD: A multi-centered retrospective design was used. A total of 443 children, registered to the national newborn hearing screening programme, with bilateral hearing loss, from five different regions of Turkey, were evaluated in terms of the types of hearing loss, the degree of hearing loss, the types of risk factors, parental consanguinity, age at diagnosis and age of auditory intervention, respectively...
November 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29097034/-medical-devices-correcting-the-deafness-hearing-aids-and-auditory-implants
#4
Jérôme Nevoux, Arnaud Coez, Éric Truy
The management of deafness has become a major public health issue as their lack of detection has a deleterious effect in children and increases the risk factors for aggravation of other pathologies in adults. The detection of deafness remains a real challenge: in the newborn, systematic screening at birth is a good strategy, in adults, much remains to be done. The functional rehabilitation of deafness is based on the use of hearing aids by aerial or bone conduction or of auditory implants. There are three types of auditory implants available: bone anchored hearing implants, middle ear implants and cochlear implants...
October 30, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/29096023/school-aged-children-with-mild-bilateral-and-unilateral-hearing-loss-parents-reflections-on-services-experiences-and-outcomes
#5
Viviane Grandpierre, Elizabeth M Fitzpatrick, Eunjung Na, Oreen Mendonca
Following the establishment of newborn hearing screening programs, age of identification and length of time before receiving interventions has been reduced for children, including those with milder degrees of hearing loss who were previously not identified until school age. This population of early-identified children requires new support programs for parents. Although literature is emerging on how parents experience the initial years, there is limited information on support needs during early school years...
October 31, 2017: Journal of Deaf Studies and Deaf Education
https://www.readbyqxmd.com/read/29089220/-genetic-hearing-loss
#6
Lei Tanaka-Ouyang, Sandrine Marlin, Jérôme Nevoux
Deafness is the most common sensory disability in developed countries affecting more than 2 births in 1000. Eighty percent of congenital deafness is genetic. Universal newborn hearing screening has been in place since 2012 in France. All genetic hearing losses are not congenital and all congenital hearing losses are not genetic. Genetic hearing loss may be syndromic (associated with other symptoms) (10 %) or non-syndromic (isolated) (90 %). Hearing loss may initially be the only symptom of syndromic deafness...
October 28, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/29081305/survey-of-otolaryngology-services-in-ukraine-and-neighbouring-central-and-eastern-european-countries
#7
M M Verkerk, R Wagner, R Fishchuk, J J Fagan
OBJECTIVE: The present humanitarian crisis in Ukraine is putting strains on its healthcare system. This study aimed to assess services and training in otolaryngology, audiology and speech therapy in Ukraine and its geographical neighbours. METHOD: Survey study of 327 otolaryngologists from 19 countries. RESULTS: Fifty-six otolaryngologists (17 per cent) from 15 countries responded. Numbers of otolaryngologists varied from 3.6 to 12.3 per 100 000 population (Ukraine = 7...
October 30, 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/29073768/educating-parents-about-infant-language-development-a-randomized-controlled-trial
#8
Dana L Suskind, Christy Y Y Leung, Robert J Webber, Alison C Hundertmark, Kristin R Leffel, Iara E Fuenmayor Rivas, William A Grobman
A total of 427 women (aged 18-45 years) who delivered a singleton neonate without serious medical complications were randomized to watch either an educational intervention (n = 225) or the sudden infant death syndrome (n = 202) video. Linear mixed models showed that the intervention women significantly gained knowledge over time. Knowledge gain was largest among high-socioeconomic status (high-SES) and middle-SES English-speaking, smaller among low-SES Spanish-speaking, and nonsignificant among low-SES English-speaking women...
October 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/29066580/intelligence-and-academic-achievement-with-asymptomatic-congenital-cytomegalovirus-infection
#9
Adriana S Lopez, Tatiana M Lanzieri, Angelika H Claussen, Sherry S Vinson, Marie R Turcich, Isabella R Iovino, Robert G Voigt, A Chantal Caviness, Jerry A Miller, W Daniel Williamson, Craig M Hales, Stephanie R Bialek, Gail Demmler-Harrison
OBJECTIVES: To examine intelligence, language, and academic achievement through 18 years of age among children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected infants. METHODS: We used growth curve modeling to analyze trends in IQ (full-scale, verbal, and nonverbal intelligence), receptive and expressive vocabulary, and academic achievement in math and reading. Separate models were fit for each outcome, modeling the change in overall scores with increasing age for patients with normal hearing (n = 78) or with sensorineural hearing loss (SNHL) diagnosed by 2 years of age (n = 11) and controls (n = 40)...
November 2017: Pediatrics
https://www.readbyqxmd.com/read/29050102/-research-progress-on-the-etiology-of-delayed-onset-hearing-loss-in-children
#10
X Y Wang, L H Huang, Y T Du
Newborn hearing screening is an effective method for early detection of hearing loss, however, it is not able to detect delayed-onset hearing loss. By exploring the etiology of delayed-onset hearing loss in children, it can provide a clinical basis for early detection of delayed-onset hearing loss. Mutations in SLC26A4, mitochondrial, GJB2 and other genes, enlarged vestibular aqueduct, congenital cytomegalovirus infection, extracorporeal membrane oxygenation, and auditory neuropathy et al were more commonly reported risk factors...
October 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/29049630/reflections-on-lake-como-conferences-2000-2016
#11
Ferdinando Grandori, Deborah Hayes
We present an overview of the conceptualization and development of the Newborn Hearing Screening and Hearing Across the Lifespan (Lake Como) conferences from 2000 to 2016.
October 12, 2017: American Journal of Audiology
https://www.readbyqxmd.com/read/29049277/update-interim-guidance-for-the-diagnosis-evaluation-and-management-of-infants-with-possible-congenital-zika-virus-infection-united-states-october-2017
#12
Tolulope Adebanjo, Shana Godfred-Cato, Laura Viens, Marc Fischer, J Erin Staples, Wendi Kuhnert-Tallman, Henry Walke, Titilope Oduyebo, Kara Polen, Georgina Peacock, Dana Meaney-Delman, Margaret A Honein, Sonja A Rasmussen, Cynthia A Moore
CDC has updated its interim guidance for U.S. health care providers caring for infants with possible congenital Zika virus infection (1) in response to recently published updated guidance for health care providers caring for pregnant women with possible Zika virus exposure (2), unknown sensitivity and specificity of currently available diagnostic tests for congenital Zika virus infection, and recognition of additional clinical findings associated with congenital Zika virus infection. All infants born to mothers with possible Zika virus exposure* during pregnancy should receive a standard evaluation at birth and at each subsequent well-child visit including a comprehensive physical examination, age-appropriate vision screening and developmental monitoring and screening using validated tools (3-5), and newborn hearing screen at birth, preferably using auditory brainstem response (ABR) methodology (6)...
October 20, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29046308/progress-toward-development-of-a-vaccine-against-congenital-cytomegalovirus-infection
#13
Mark R Schleiss, Sallie R Permar, Stanley A Plotkin
A vaccine against congenital human cytomegalovirus (CMV) infection is a major public health priority. Congenital CMV causes substantial long-term morbidity in newborns, particularly sensorineural hearing loss (SNHL), and the public health impact of this infection on maternal and child health is under-recognized. Although progress toward development of a vaccine has been limited by an incomplete understanding of the correlates of protective immunity for the fetus, knowledge about some of the key components of the maternal immune response necessary for preventing trans-placental transmission is accumulating...
October 18, 2017: Clinical and Vaccine Immunology: CVI
https://www.readbyqxmd.com/read/29034761/knowledge-and-attitude-of-mothers-regarding-infant-hearing-loss-in-changsha-hunan-province-china
#14
Xiaoli Wang, Dan Wu, Yali Zhao, Danhui Li, Dinghua He
OBJECTIVE: The objective of this study was to explore the knowledge and attitude among mothers of newborns regarding infant hearing loss (HL) in Changsha, Hunan province, China. DESIGN: A questionnaire including 18 items was given to mothers. STUDY SAMPLE: A total of 115 mothers participated in the study. RESULTS: Seven risk factors for hearing loss were identified correctly by above 60% of respondents and the top three were prolonged noise (88...
October 16, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/29032597/more-than-two-years-follow-up-of-infants-with-congenital-cytomegalovirus-infection-in-japan
#15
Shin Koyano, Ichiro Morioka, Akira Oka, Hiroyuki Moriuchi, Kimisato Asano, Yushi Ito, Tetsushi Yoshikawa, Hideto Yamada, Tatsuo Suzutani, Naoki Inoue
BACKGROUND: The aim of the study was to evaluate outcomes of congenital cytomegalovirus (CMV) cases identified by our urine-filter screening assay after follow-up for more than 2 years, and to observe the clinical outcomes after anti-CMV therapies. METHODS: Sixty of the 72 congenital CMV cases were enrolled. Forty-three cases asymptomatic at birth, 7 cases symptomatic at birth but untreated with anti-CMV drugs, and 10 symptomatic cases treated with anti-CMV drugs were clinically observed for more than 2 years...
October 15, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28990963/terrible-twos-or-early-signs-of-psychopathology-developmental-patterns-in-early-identified-preschoolers-with-cochlear-implants-compared-with-hearing-controls
#16
Anouk P Netten, Carolien Rieffe, Lizet Ketelaar, Wim Soede, Kenneth D Gadow, Johan H M Frijns
OBJECTIVE: Cochlear implants (CIs) have dramatically improved the lives of children who are deaf or hard of hearing; however, little is known about its implications for preventing the development of psychiatric symptoms in this at-risk population. This is the first longitudinal study to examine the early manifestation of emotional and behavioral disorders and associated risk and protective factors in early identified preschoolers with CIs compared with hearing peers. DESIGN: Participants were 74 children with CIs and 190 hearing controls between ages 1 and 5 years (mean age, 3...
October 4, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28983456/enzyme-replacement-therapy-attenuates-disease-progression-in-two-japanese-siblings-with-mucopolysaccharidosis-type-vi-10-year-follow-up
#17
Mahoko Furujo, Motomichi Kosuga, Torayuki Okuyama
Early initiation of enzyme replacement therapy (ERT) has demonstrated clinical benefit in patients with mucopolysaccharidosis type VI (MPS VI), a progressive, multisystem autosomal recessive lysosomal disorder caused by N-acetylgalactosamine-4-sulphatase (ASB) deficiency and the consequent accumulation of glycosaminoglycan. A previous case report highlighted that 3 years of ERT with recombinant human ASB (galsulfase) was well tolerated and effective in two Japanese siblings with MPS VI who initiated ERT at 5...
December 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28983455/long-term-cognitive-and-somatic-outcomes-of-enzyme-replacement-therapy-in-untransplanted-hurler-syndrome
#18
Julie B Eisengart, Jeanine Jarnes, Alia Ahmed, Igor Nestrasil, Richard Ziegler, Kathleen Delaney, Elsa Shapiro, Chester Whitley
Mucopolysaccharidosis type I (MPS I) was added to the Recommended Uniform Screening Panel for newborn screening in 2016, highlighting recognition that early treatment of MPS I is critical to stem progressive, irreversible disease manifestations. Enzyme replacement therapy (ERT) is an approved treatment for all MPS I phenotypes, but because the severe form (MPS IH, Hurler syndrome) involves rapid neurocognitive decline, the impermeable blood-brain-barrier is considered an obstacle for ERT. Instead, hematopoietic cell transplantation (HCT) has long been recommended, as it is believed to be the only therapy that arrests neurocognitive decline...
December 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28964301/changes-in-tonal-audiometry-in-children-with-progressive-sensorineural-hearing-loss-and-history-of-neonatal-intensive-care-unit-discharge-a-20-year-long-term-follow-up
#19
Carlos F Martínez-Cruz, Adrián Poblano, Patricia García-Alonso Themann
OBJECTIVE: Newborns from Neonatal intensive care units (NICU) are at high-risk for sensorineural hearing loss (SNHL) a follow-up is needed for early diagnosis and intervention. Our objective here was to describe the features and changes of SNHL at different periods during a follow-up of almost 20 years. METHODS: Risk factors for SNHL during development were analyzed. The audiological examination included: Brainstem auditory evoked potentials (BAEP), and Transient evoked otoacoustic emissions (TEOAE)...
October 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28964278/determinants-of-caregivers-awareness-of-universal-newborn-hearing-screening-in-malaysia
#20
Abdul Halim Abdul Majid, Mohd Normani Zakaria, Nor Azimah Chew Abdullah, Sulaiman Hamzah, Siti Zamratol-Mai Sarah Mukari
OBJECTIVE: This paper aims to investigate the effects of perceived attitude and anxiety on awareness of UNHS among caregivers in Malaysia. METHODS: Using cross sectional research approach, data were collected and some 46 out of 87 questionnaires distributed to caregivers attending UNHS programs at selected public hospitals were usable for analysis (response rate of 52.8%). Partial Least Squares Method (PLS) algorithm and bootstrapping technique were employed to test the hypotheses of the study...
October 2017: International Journal of Pediatric Otorhinolaryngology
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