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hear failure

Firas Sbeih, Florian Christov, Michael B Gluth
OBJECTIVE: To describe the course of Meniere's disease with noninvasive treatment during the first few years after initial diagnosis. METHODS: A retrospective review of consecutive patients with newly diagnosed definite Meniere's disease between 2013 and 2016 and a minimum follow-up of 1 year. Patients received a written plan for low sodium, water therapy, and treatment with a diuretic and/or betahistine. Subjects were screened and treated for vestibular migraine as needed...
March 1, 2018: Annals of Otology, Rhinology, and Laryngology
Manuela Germeshausen, Phil Ancliff, Jaime Estrada, Markus Metzler, Eva Ponstingl, Horst Rütschle, Dirk Schwabe, Richard H Scott, Sule Unal, Angela Wawer, Bernward Zeller, Matthias Ballmaier
Heterozygous mutations in MECOM (MDS1 and EVI1 complex locus) have been reported to be causative of a rare association of congenital amegakaryocytic thrombocytopenia and radioulnar synostosis. Here we report on 12 patients with congenital hypomegakaryocytic thrombocytopenia caused by MECOM mutations (including 10 novel mutations). The mutations affected different functional domains of the EVI1 protein. The spectrum of phenotypes was much broader than initially reported for the first 3 patients; we found familial as well as sporadic cases, and the clinical spectrum ranged from isolated radioulnar synostosis with no or mild hematological involvement to severe bone marrow failure without obvious skeletal abnormality...
March 27, 2018: Blood Advances
Honar Cherif, Andreas Greinacher, Norbert Lubenow
We report on a patient with inherited macrothrombocytopenia, MYH9 related disease (MYH9-RD). The patient was wrongly diagnosed and repeatedly treated for immune thrombocytopenia (ITP) for nearly 50 years. Cases of misdiagnosed MYH9-RD and other hereditary thrombocytopenias have been described previously. Typical clinical features such as renal failure and/or progressive loss of hearing should give grounds to suspect hereditary thrombocytopenia. Initial laboratory diagnosis can start with a simple blood smear followed by immunohistochemistry and genotyping...
March 13, 2018: Läkartidningen
Min Wu, Xianming Fu, Ying Ji, Wanhai Ding, Dali Deng, Yehan Wang, Xiaofeng Jiang, Chaoshi Niu
BACKGROUND: Microvascular decompression (MVD) of the trigeminal nerve is the most effective treatment for trigeminal neuralgia (TN). However, when encountering with classical trigeminal neuralgia caused by venous compression, the procedure becomes much more difficult, and failure or recurrence due to incomplete decompression may become frequent. This study aimed to investigate the anatomical variation of the culprit veins, and discuss the surgical strategy for different types respectively...
March 3, 2018: World Neurosurgery
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
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
Maria Stella Arantes do Amaral, Ana Cláudia Mirândola B Reis, Eduardo T Massuda, Miguel Angelo Hyppolito
INTRODUCTION: The surgery during which the cochlear implant internal device is implanted is not entirely free of risks and may produce problems that will require revision surgeries. OBJECTIVE: To verify the indications for cochlear implantation revision surgery for the cochlear implant internal device, its effectiveness and its correlation with certain variables related to language and hearing. METHODS: A retrospective study of patients under 18 years submitted to cochlear implant Surgery from 2004 to 2015 in a public hospital in Brazil...
February 16, 2018: Brazilian Journal of Otorhinolaryngology
Daniele Starnoni, Roy Thomas Daniel, Constantin Tuleasca, Mercy George, Marc Levivier, Mahmoud Messerer
OBJECTIVE During the last decade, the primary objective for large vestibular schwannoma (VS) management has progressively shifted, from tumor excision to nerve preservation by using a combined microsurgical and radiosurgical approach. The aim of this study was to provide a systematic review and meta-analysis of the available literature regarding the combined strategy of subtotal resection (STR) followed by stereotactic radiosurgery (SRS) for large VSs. METHODS The authors performed a systematic review and meta-analysis in compliance with the PRISMA guidelines for article identification and inclusion using the PubMed, Embase, and Cochrane databases...
March 2018: Neurosurgical Focus
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
Olivier Bonnot, Hans-Hermann Klünemann, Christian Velten, Juan Vicente Torres Martin, Mark Walterfang
OBJECTIVES: We conducted the first systematic literature review and analysis of psychiatric manifestations in Niemann-Pick disease type C (NPC) to describe: 1) time of occurrence of psychiatric manifestations relative to other disease manifestations; 2) frequent combinations of psychiatric, neurological and visceral disease manifestations. METHODS: A systematic EMBase literature search was conducted to identify, collate and analyze published data from patients with NPC associated with psychiatric symptoms, published between January 1967 and November 2015...
February 19, 2018: World Journal of Biological Psychiatry
Margaret Aron, Thomas G Landry, Manohar Bance
BACKGROUND: Total ossicular replacement prostheses (TORP) are often used to re-establish ossicular coupling of sound in an ear lacking a stapes supra-structure. The use of TORPs, however, is associated with a 2/3 five year failure rate due to their anatomic instability over time in the middle ear. The use of autologous fat to try and stabilize TORPs may improve long-term results with this challenging ossicular reconstruction technique. METHODS: A cadaveric temporal bone model was developed and laser Doppler vibrometry was used to measure and record round window membrane vibration in response to sound stimulation under the following conditions: normal middle ear, middle ear filled with fat, normal middle ear with TORP prosthesis, TORP prosthesis with fat around its distal end and TORP prosthesis with fat filling the middle ear...
February 17, 2018: Journal of Otolaryngology—Head & Neck Surgery
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
Wegdan Mawlana, Paul Zamiara, Hilary Lane, Margaret Marcon, Eveline Lapidus-Krol, Priscilla Pl Chiu, Aideen M Moore
BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF. METHODS: Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12-36months...
January 31, 2018: Journal of Pediatric Surgery
So Young Kim, Byung Yoon Choi, Eun Young Jung, Hyunsoo Park, Ha-Na Yoo, Kyo Hoon Park
BACKGROUND: We aimed to identify prenatal and postnatal risk factors associated with abnormal newborn hearing screen (NHS) results and subsequently confirmed sensorineural hearing loss (SNHL) in preterm twin neonates. METHODS: Electronic medical records of 159 twin neonates who were born alive after ≤32 weeks were retrospectively reviewed for hearing loss in both ears. Histopathologic examination of the placenta was performed and clinical data, including method of conception and factors specific to twins, were retrieved from a computerized perinatal database...
January 31, 2018: Pediatrics and Neonatology
Courtney Ryan, Hafza Dadabhoy, Tom Baranowski
OBJECTIVE: The most productive methods of recruitment for a videogame for health (G4H) trial are not known. Success or failure of recruitment methods has been reported for a variety of clinical trials, but few specifically for G4H trials. This study's goal was to recruit 444 overweight or obese (body mass index percentile between the 84.5th-99.4th percentiles) children between the ages of 10-12 years. The article reports the results of different methods of participant recruitment. MATERIALS AND METHODS: Participants had to agree to three fasting blood samples (baseline, immediately after, and 2 months later); be willing to wear an accelerometer for 7 days at each assessment; read and speak English fluently (because the games were in English); have no history of any condition that would affect what he/she could eat or how much physical activity he/she could get; and have an eligible home computer purchased in the last 5 years with high-speed internet...
February 2018: Games for Health
Miranda L Camet, Susan S Hayashi, Belinda C Sinks, Jennifer Henry, Katie Gettinger, Ashley Hite, Juliann Kiefer, Caroline Mohrmann, Taryn Sandheinrich, Feng Gao, Robert J Hayashi
BACKGROUND: Sensorineural hearing loss due to ototoxic cancer therapy is well established; effects on the vestibular system are unknown. We examined the feasibility of implementing vestibular screens for pediatric cancer survivors exposed to ototoxic agents. The prevalence of screening failures is reported. METHODS: Cancer survivors who were 6-17 years, at least 1-month posttreatment, and received ototoxic therapy (radiation to the head/neck, cisplatin, carboplatin) were eligible...
January 30, 2018: Pediatric Blood & Cancer
Madeline R Sterling, Frank R Lin, Deanna P Jannat-Khah, Adele M Goman, Sandra E Echeverria, Monika M Safford
No abstract text is available yet for this article.
January 25, 2018: JAMA Otolaryngology—Head & Neck Surgery
Margaret A Kenna, Alexandria L Irace, Julie E Strychowsky, Kosuke Kawai, Devon Barrett, Juliana Manganella, Michael J Cunningham
Importance: Children with Klippel-Feil syndrome (KFS), characterized principally by abnormal fusion of 2 or more cervical vertebrae, may have many additional congenital anomalies. The overall prevalence of otolaryngologic manifestations among patients with KFS has not been previously characterized. Objective: To define the otolaryngologic diagnoses made and procedures performed in 95 patients with KFS, which, to our knowledge, is the largest series of this challenging patient population published to date...
January 25, 2018: JAMA Otolaryngology—Head & Neck Surgery
Sen Chen, Le Xie, Kai Xu, Hai-Yan Cao, Xia Wu, Xiao-Xiang Xu, Yu Sun, Wei-Jia Kong
Mutations in the GJB2 gene (which encodes Connexin26 (Cx26)) are the most common causes of hereditary hearing loss, and previous studies showed postnatal development arrest of the organ of Corti in different Cx26-null mouse models. To further explore the pathological changes and the mechanism behind the cochlear abnormalities in these mice, we established transgenic mouse models by conditional knockdown of cochlear Cx26 at postnatal day (P) 0 and P8. Auditory brainstem responses were recorded and the morphological features in the organ of Corti were analyzed 18 days later after Cx26 knockdown in the different groups of mice...
January 19, 2018: Disease Models & Mechanisms
Augusto Biasini, Fiorella Monti, Maria Chiara Laguardia, Marcello Stella, Lucia Marvulli, Erica Neri
BACKGROUND AND AIM OF THE STUDY: Extrauterine growth restriction and failure to thrive remain a major problem in Extremely Low Birth Weight infants. Nutritional support in preterm babies has the objective to improve the achieve rate of growth similar to those of the fetus in utero at the equivalent gestational age. The aim of the study was to evaluate feeding tolerance, intrahospital growth, neurological outcome and anthropometric data until 24 months of corrected age (mca) from different protein intake assumed by preterm babies <1250 g during their stay in NICU...
January 16, 2018: Acta Bio-medica: Atenei Parmensis
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