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Newborn hearing

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
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
Monika Zych, Grażyna Greczka, Piotr Dąbrowski, Maciej Wróbel, Joanna Szyfter-Harris, Witold Szyfter
The Polish Universal Neonatal Hearing Screening Program (PUNHSP) has been carried out in Poland for 14 years. The main aim of this Program is to organize hearing screening tests and to gather the information about risk factors of hearing loss in almost all newborns in Poland. It consists of 496 centers at 3 referral levels. A total of 5 458 114 children had been registered in the Central Database (CDB) of PUNHSP by the 22nd of August 2017. Bilateral sensorineural hearing loss was the most frequently appearing hearing impairment in children...
February 28, 2018: Otolaryngologia Polska
Hideaki Moteki, Yuichi Isaka, Yuji Inaba, Mitsuo Motobayashi, Shin-Ya Nishio, Satoshi Ohira, Takuya Yano, Satoshi Iwasaki, Tanri Shiozawa, Kenichi Koike, Shin-Ichi Usami
OBJECTIVE: Congenital cytomegalovirus (cCMV) infection is the most common congenital infection, with the majority of infected newborns having no detectable signs. The aim of this study was to examine the accuracy of our newly developed DBS-based assay as an appropriate mass screening method for cCMV infection. METHODS: Between May 2011 and October 2016, newborns delivered at six hospitals in Nagano Prefecture, Japan were enrolled prospectively. We employed dried blood spot (DBS)-based assays with real-time quantitative PCR (qPCR)...
March 7, 2018: Acta Oto-laryngologica
Wei Zhuang, Caiji Wang, Xi Shi, Shiwei Qiu, Shili Zhang, Bing Xu, Min Chen, Wen Jiang, Hongyan Dong, Yuehua Qiao
Congenital cytomegalovirus (CMV) infection is the most common infectious cause of sensorineural hearing loss in children. While the importance of CMV‑induced SNHL has been described, the mechanisms underlying its pathogenesis and the role of inflammatory responses remain elusive. The present study established an experimental model of hearing loss after systemic infection with murine CMV (MCMV) in newborn mice. Auditory brainstem responses were tested to evaluate hearing at 3 weeks, expression of inflammasome‑-associated factors was assessed by immunofluorescence, western blot analysis, reverse transcription‑quantitative polymerase chain reaction and ELISA...
March 6, 2018: International Journal of Molecular Medicine
Thomas Hegyi, Alan Kleinfeld, Andrew Huber, Barry Weinberger, Naureen Memon, Weichung Shih, Mary Carayannopoulos, William Oh
BACKGROUND: Hyperbilirubinemia occurs in over 80% of newborns and severe bilirubin toxicity can lead to neurological dysfunction and death, especially in preterm infants. Currently, the risk of bilirubin toxicity is assessed by measuring the levels of total serum bilirubin (TSB), which are used to direct treatments including immunoglobulin administration, phototherapy, and exchange transfusion. However, free, unbound bilirubin levels (Bf) predict the risk of bilirubin neurotoxicity more accurately than TSB...
March 4, 2018: Journal of Maternal-fetal & Neonatal Medicine
Karen B Fowler, Suresh B Boppana
Each year, thousands of children are born with or develop permanent disabilities such as hearing loss, vision loss, motor and cognitive deficits from congenital CMV infection (cCMV). However, awareness of cCMV and its associated sequelae is very low in pregnant women and healthcare providers. Both targeted and universal approaches to screen newborns for CMV infection are now achievable due to recent scientific advances including the development of a rapid, high-throughput method for detecting CMV in saliva, the efficacy of antiviral treatment in symptomatic infants, and the demonstration of cost effectiveness of CMV screening...
March 1, 2018: Seminars in Perinatology
Ayman W El-Hattab, Mohammed Almannai, V Reid Sutton
Newborn screening programs aim to achieve presymptomatic diagnosis of treatable disorders allowing for early initiation of medical care to prevent or reduce significant morbidity and mortality. Many of the conditions included in the newborn screening panels are inborn errors of metabolism; however, screening for endocrine, hematologic, immunologic, and cardiovascular diseases, and hearing loss is also included in many panels. Newborn screening tests are not diagnostic and therefore diagnostic testing is needed to confirm or exclude the suspected diagnosis...
April 2018: Pediatric Clinics of North America
Yuan Liang, Qi Peng, Kangwei Wang, Pengyuan Zhu, Chunqiu Wu, Chunbao Rao, Jiang Chang, Siping Li, Xiaomei Lu
OBJECTIVES: To identity the genetic causes of hearing loss in a Han Chinese family with enlarged vestibular aqueduct syndrome. METHODS: Multiplex PCR technology combined with Ion Torrent™ next-generation sequencing technology was used to search for pathogenic mutations. A group of 1500 ethnically-matched normal hearing subjects screened for mutations in deafness-related genes using the same method in previously studied were included as a control. RESULTS: The proband and his little sister suffered from typical features of sensorineural hearing loss with enlarged vestibular aqueduct (EVA)...
April 2018: International Journal of Pediatric Otorhinolaryngology
Anke Rissmann, Andrea Koehn, Marja Loderstedt, Cornelia Schwemmle, Gerrit Goetze, Sylva Bartel, Stefan K Plontke, Joerg Langer, Klaus Begall, Peter Matulat, Friedrich-Wilhelm Roehl, Ulrich Vorwerk
OBJECTIVES: Airway management during adenoidectomy is traditionally performed through endotracheal intubation (ETT). Laryngeal mask airway (LMA) may be less stimulating to the airway and allow for shorter overall operating room time. Previous studies report LMA use during adenotonsillectomy with conversion rates to ETT of up to 17%. There has been no prior evaluation of LMA use during adenoidectomy alone. In this study, we attempt to identify the rate and contributing factors of LMA failure during adenoidectomy...
April 2018: International Journal of Pediatric Otorhinolaryngology
Soren Gantt, Ari Bitnun, Christian Renaud, Fatima Kakkar, Wendy Vaudry
Congenital cytomegalovirus infection (cCMV) is the most common congenital infection, occurring in approximately 0.5% of live births. Most infected newborns are asymptomatic, but up to 20% develop sensorineural hearing loss or other permanent neurologic sequelae. The presentation of newborns with symptomatic cCMV is highly variable, and the infection is usually not diagnosed in the absence of a screening program. Newborn cCMV screening programs are estimated to be beneficial and cost-effective, and are increasingly being implemented...
May 2017: Paediatrics & Child Health
Omer Erdeve, Emel Okulu, Ozgur Olukman, Dilek Ulubas, Gokhan Buyukkale, Fatma Narter, Gaffari Tunc, Begum Atasay, Nazli Dilay Gultekin, Saadet Arsan, Esin Koc
BACKGROUND: Neonatal jaundice (NNJ) is common, but few root cause analyses based on national quality registries have been performed. An online registry was established to estimate the incidence of NNJ in Turkey and to facilitate a root cause analysis of NNJ and its complications. METHODS: A multicenter prospective study was conducted on otherwise healthy newborns born at ≥35 weeks of gestation and hospitalized for only NNJ in 50 collaborator neonatal intensive care units across Turkey over a 1-year period...
2018: PloS One
Lise Carolyn Johnson, Margaret Toro, Emily Vishnja, Arielle Berish, Bianca Mills, Zhigang Lu, Ellice Lieberman
BACKGROUND: Although the utility of universal newborn hearing screening is undisputed, testing protocols vary. In particular, the impact of the infant's age at the time of automated auditory brainstem response (AABR) screening has not been well studied. METHODS: We conducted a retrospective review of newborn hearing screening data in 6817 low-risk, term and late-preterm newborns at our large, urban, academic medical center for a 1-year period to analyze the impact of age and other factors on the screening failure rate and referral for diagnostic testing...
February 22, 2018: Hospital Pediatrics
A Smith, A O'Connor, S Hennessy, P G O'Sullivan, L Gibson
The Newborn Hearing Screening Programme (NHSP) was established in Cork University Maternity Hospital (CUMH) in April 2011. Between April 2011 and July 2014, 42 infants were identified with a Permanent Childhood Hearing Impairment (PCHI). Following this diagnosis, infants underwent a paediatric assessment according to recognised guidelines with the intention of identifying the underlying aetiology of the PCHI. The aim of this study was to assess the findings of this aetiological workup via retrospective chart review...
December 18, 2017: Irish Medical Journal
Mark Mercurio
Early in my career as a neonatologist, I was called into the hospital for a newborn who would not stop crying. Screaming, really. When I entered the unit, I was greeted by a loud, shrill, distinctive cry. After hearing the history and examining the baby, I just stood there for a while, watching and listening. It took some time, but eventually, I noticed a subtle regularity, a rhythmicity. I took off my watch, placed it on the bed next to the child, and found that the crying briefly grew louder about every six or seven seconds...
January 2018: Hastings Center Report
Teerawat Wiwatpanit, Natalie N Remis, Aisha Ahmad, Yingjie Zhou, John C Clancy, Mary Ann Cheatham, Jaime García-Añoveros
Acquired hearing loss is the predominant neurodegenerative condition associated with aging in humans. Although mutations on several genes are known to cause congenital deafness in newborns, few genes have been implicated in age-related hearing loss (ARHL), perhaps because its cause is likely polygenic. Here, we generated mice lacking lysosomal calcium channel mucolipins 3 and 1 and discovered that both male and female mice suffered a polygenic form of hearing loss. While mucolipin 1 is ubiquitously expressed in all cells, mucolipin 3 is expressed in a small subset of cochlear cells -hair cells (HCs) and marginal cells of the stria vascularis- and very few other cell types...
February 16, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Eric N Appelbaum, Jessica B Howell, Derek Chapman, Arti Pandya, Kelley M Dodson
OBJECTIVE: To analyze 2007 Joint Committee on Infant Hearing (JCIH) risk factors in children with confirmed unilateral hearing loss (UHL) who initially passed newborn hearing screening. METHODS: Retrospective record review of 16,108 infants who passed newborn hearing screening but had one or more JCIH risk factors prompting subsequent follow-up through the universal newborn hearing screening (UNHS) program in Virginia from 2010 to 2012. The study was reviewed and qualified as exempt by the Virginia Commonwealth University Institutional Review Board (IRB) and the Virginia Department of Health...
March 2018: International Journal of Pediatric Otorhinolaryngology
Katarzyna Wroblewska-Seniuk, Piotr Dabrowski, Grazyna Greczka, Katarzyna Szabatowska, Agata Glowacka, Witold Szyfter, Jan Mazela
OBJECTIVE: The aim of this study was to analyze infants diagnosed with sensorineural or conductive hearing deficit and to identify risk factors associated with these defects. MATERIAL AND METHODS: A retrospective analysis of infants diagnosed with hearing deficit based on the database of the universal newborn hearing screening program and medical records of the patients. RESULTS: 27 935 infants were covered by the universal neonatal hearing screening program...
February 2018: International Journal of Pediatric Otorhinolaryngology
Jonathan Ross Mallen, Jacob B Hunter, Charles Auerbach, Leslie Wexler, Andrea Vambutas
OBJECTIVE: To define the rate and characterize the type of newborn hearing screening failures in multigestational births. METHODS: Retrospective chart review of all multigestational births that occurred in a 10-year period (2002-2012) in which at least one newborn failed newborn hearing screening at two tertiary care hospitals in the Northwell Health System. RESULTS: Out of 125,405 total births, we identified 2961 multigestational births, of which 59 (2...
February 2018: International Journal of Pediatric Otorhinolaryngology
Maggie Yee Yan Lam, Eddie Chi Ming Wong, Chi Wai Law, Helena Hui Ling Lee, Bradley McPherson
OBJECTIVES: To facilitate early diagnosis of infants with hearing loss, a universal newborn hearing screening program (UNHS) has been implemented in Hong Kong's public hospitals for over a decade. However, there have been no known studies investigating parent attitudes to, and satisfaction with, UNHS since its launch in Hong Kong. The present study aimed to investigate knowledge of UNHS as well as infant hearing development, and attitudes and satisfaction with UNHS, in Hong Kong mothers with newborns...
February 2018: International Journal of Pediatric Otorhinolaryngology
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