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https://www.readbyqxmd.com/read/27898605/initial-results-of-a-safety-and-feasibility-study-of-auditory-brainstem-implantation-in-congenitally-deaf-children
#1
Eric P Wilkinson, Laurie S Eisenberg, Mark D Krieger, Marc S Schwartz, Margaret Winter, Jamie L Glater, Amy S Martinez, Laurel M Fisher, Robert V Shannon
OBJECTIVE: To determine the safety and feasibility of the auditory brainstem implant (ABI) in congenitally deaf children with cochlear aplasia and/or cochlear nerve deficiency. STUDY DESIGN: Phase I feasibility clinical trial of surgery in 10 children, ages 2 to 5 years, over a 3-year period. SETTING: Tertiary children's hospital and university-based pediatric speech/language/hearing center. INTERVENTION(S): ABI implantation and postsurgical programming...
November 24, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27896132/divergent-clinical-outcomes-of-alpha-glucosidase-enzyme-replacement-therapy-in-two-siblings-with-infantile-onset-pompe-disease-treated-in-the-symptomatic-or-pre-symptomatic-state
#2
Takashi Matsuoka, Yoshiyuki Miwa, Makiko Tajika, Madoka Sawada, Koichiro Fujimaki, Takashi Soga, Hideshi Tomita, Shigeru Uemura, Ichizo Nishino, Tokiko Fukuda, Hideo Sugie, Motomichi Kosuga, Torayuki Okuyama, Yoh Umeda
Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid α-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectancy of patients with IOPD...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27879419/tympanoplasty-outcomes-for-blast-induced-perforations-from-iraq-and-afghanistan-2007-2012
#3
Sungjin A Song, Shankar K Sridhara, Philip D Littlefield
OBJECTIVE: To describe characteristics of blast-induced tympanic membrane perforations that do not spontaneously heal, evaluate the outcomes of tympanoplasty techniques, and understand the factors associated with surgical success. SETTING: Two tertiary military health care institutions. STUDY DESIGN: Case series with chart review. SUBJECTS AND METHODS: This study reviewed the practice of 1 military neurotologist and included all tympanoplasties for combat blast-induced perforations from 2007 to 2012, which comprised a total of 55 patients...
November 22, 2016: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/27863036/newborn-hearing-screening-in-prematurity-fate-of-screening-failures-and-auditory-maturation
#4
Hyung Chae Yang, Chung Man Sung, Dong Joo Shin, Yong Beom Cho, Chul Ho Jang, Hyong-Ho Cho
OBJECTIVES: The purpose of this study was to identify delayed auditory maturation and the fate of premature infants who failed the newborn hearing screening (NHS) in neonatal intensive care unit. MATERIALS AND METHODS: A total of 1,375 neonates underwent NHS using the transient evoked otoacoustic emission (TEOAE) in a tertiary hospital between 2007 and 2010 according to the Joint Committee on Infant Hearing Guidelines. In addition, a structured telephone survey was given to caregivers of infants who were lost to follow-up NHS...
November 12, 2016: Clinical Otolaryngology
https://www.readbyqxmd.com/read/27849250/health-vulnerability-index-and-newborn-hearing-screening-urban-inequality
#5
Gabriela Cintra Januário, Claudia Regina Lindgren Alves, Stela Maris Aguiar Lemos, Maria Cristina de Mattos Almeida, Ramon Costa Cruz, Amélia Augusta de Lima Friche
Purpose: To analyze the intra-urban differentials related to the outcome of the Newborn Hearing Screening (NHS) of children living in Belo Horizonte tested in a reference service using the Health Vulnerability Index (HVI). Methods: cross-sectional study with children living in Belo Horizonte evaluated by a Newborn Hearing Screening Reference Service (NHSRS) between 2010 and 2011. The HVI of the census tract of each child was obtained by the georeferencing of their respective addresses...
September 2016: CoDAS
https://www.readbyqxmd.com/read/27816395/-alport-syndrome-hereditary-nephropathy-associated-with-mutations-in-genes-coding-for-type-iv-collagen-chains
#6
Laurence Heidet, Marie-Claire Gubler
Alport syndrome is an inherited disorder characterized by the association of a progressive haematuric nephropathy with ultrastructural abnormalities of the glomerular basement membranes, a progressive sensorineural hearing loss and sometimes ocular involvement. Its incidence is less than 1 per 5000 individuals and the disease is the cause of about 2% of end stage renal disease in Europe and the United States. Alport syndrome is clinically and genetically heterogeneous. It is related to mutations in the genes encoding one of three chains, α3, α4 α5 of type IV collagen, the main component of basement membranes, expressed in the glomerular basement membrane...
November 2, 2016: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/27815131/sensory-coding-and-cognitive-processing-of-sound-in-veterans-with-blast-exposure
#7
Scott Bressler, Hannah Goldberg, Barbara Shinn-Cunningham
Recent anecdotal reports from VA audiology clinics as well as a few published studies have identified a sub-population of Service Members seeking treatment for problems communicating in everyday, noisy listening environments despite having normal to near-normal hearing thresholds. Because of their increased risk of exposure to dangerous levels of prolonged noise and transient explosive blast events, communication problems in these soldiers could be due to either hearing loss (traditional or "hidden") in the auditory sensory periphery or from blast-induced injury to cortical networks associated with attention...
November 2, 2016: Hearing Research
https://www.readbyqxmd.com/read/27812847/needs-priorities-and-recommendations-for-engaging-underrepresented-populations-in-clinical-research-a-community-perspective
#8
Jennifer Cunningham Erves, Tilicia L Mayo-Gamble, Alecia Malin-Fair, Alaina Boyer, Yvonne Joosten, Yolanda C Vaughn, Lisa Sherden, Patrick Luther, Stephania Miller, Consuelo H Wilkins
Engaging underrepresented groups in outcomes research is a public health priority for reducing health and health care disparities; yet, engaging these groups is challenging. Failure to involve these underrepresented populations in research further exacerbates these disparities. This article presents the health and research priorities of diverse groups of underrepresented populations in biomedical research, their concerns for participating in research, and strategies to engage them in their healthcare and research studies...
November 3, 2016: Journal of Community Health
https://www.readbyqxmd.com/read/27812817/translabyrinthine-vestibular-neurectomy-and-simultaneous-cochlear-implant-for-m%C3%A3-ni%C3%A3-re-s-disease
#9
Pietro Canzi, Marco Manfrin, Marco Perotti, Federico Aprile, Silvia Quaglieri, Elisabetta Rebecchi, Giulia Locatelli, Marco Benazzo
BACKGROUND: Surgical management of Ménière's disease (MD) is recommended in case of medical and intratympanic treatment failures. Translabyrinthine vestibular nerve section has been considered the gold standard for denervation procedures in order to control vertigo attacks, although at the cost of sacrificing residual hearing. To the best of our knowledge, no work has been published with regard to a group of patients submitted to translabyrinthine vestibular neurectomy and simultaneous cochlear implant for MD...
November 4, 2016: Acta Neurochirurgica
https://www.readbyqxmd.com/read/27811529/adult-outcomes-after-newborn-respiratory-failure-treated-with-extracorporeal-membrane-oxygenation
#10
William A Engle, Karen W West, Gail A Hocutt, Eugenia K Pallotto, Barbara Haney, Rachel J Keith, Dan L Stewart, Ellen Knodel, Denise Suttner, Rachel Chapman, Alison Thomas, Beverly Schwerin, Eileen Stork, Moira Crowley, Anthony J Piazza, Micheal L Heard, Netsanet Gebregziabher, William Fadel, Robert Bartlett
OBJECTIVE: To describe the outcome of young adults treated for hypoxemic respiratory failure with extracorporeal membrane oxygenation as neonates. DESIGN: The study was designed as a multisite, cross sectional survey. SETTING: The survey was completed electronically or on paper by subjects and stored in a secure data base. SUBJECTS: Subjects were surviving neonatal extracorporeal membrane oxygenation patients from eight institutions who were18 years old or older...
November 1, 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27807138/critical-role-of-atp-induced-atp-release-for-ca2-signaling-in-nonsensory-cell-networks-of-the-developing-cochlea
#11
Federico Ceriani, Tullio Pozzan, Fabio Mammano
Spatially and temporally coordinated variations of the cytosolic free calcium concentration ([Ca(2+)]c) play a crucial role in a variety of tissues. In the developing sensory epithelium of the mammalian cochlea, elevation of extracellular adenosine trisphosphate concentration ([ATP]e) triggers [Ca(2+)]c oscillations and propagation of intercellular inositol 1,4,5-trisphosphate (IP3)-dependent Ca(2+) waves. What remains uncertain is the relative contribution of gap junction channels and connexin hemichannels to these fundamental mechanisms, defects in which impair hearing acquisition...
November 2, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27800104/epidemiological-and-clinical-aspects-of-ear-nose-and-throat-sensorineural-emergencies-in-the-yaound%C3%A3-reference-hospital
#12
François Djomou, Yves Christian Andjock Nkouo, Eko David Mindja, Choffor Nchinda, Luc Meka, Emilia Mbamyah-Lyonga, Alexis Ndjolo
INTRODUCTION: Sensorineural emergencies (SNE) are rare clinical situations. Few patients consult early explaining subsequent difficulty in having accurate data and management. Three clinical conditions are considered SNE in otolaryngology; they include sudden sensorineural hearing loss (SSHL), Bell's palsy and acute vertigo. There is very little data available on sensorineural emergencies in our setting. The aim of this study was to provide preliminary data on the management of Ear Nose and Throat (ENT) sensorineural emergency cases in Yaoundé Reference Hospital...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27796557/systematic-review-to-evaluate-the-safety-efficacy-and-economical-outcomes-of-the-vibrant-soundbridge-for-the-treatment-of-sensorineural-hearing-loss
#13
REVIEW
Karl-Ludwig Bruchhage, Anke Leichtle, Rainer Schönweiler, Ingo Todt, Wolf-Dieter Baumgartner, Henning Frenzel, Barbara Wollenberg
Introduced in the late 90s, the active middle ear implant Vibrant Soundbridge (VSB) is nowadays used for hearing rehabilitation in patients with mild to severe sensorineural hearing loss (SNHL) unable to tolerate conventional hearing aids. In experienced hands, the surgical implantation is fast done, safe and highly standardized. Here, we present a systematic review, after more than 15 years of application, to determine the efficacy/effectiveness and cost-effectiveness, as well as patient satisfaction with the VSB active middle ear implant in the treatment of mild to severe SNHL...
October 31, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27765115/-pathological-changes-of-temporal-bone-after-cochlear-implantation-and-the-countermeasures
#14
P Han, X R Niu, X Y Du, Z C Chen, Y Xu, Y Cheng, Y Gao, Q Zhang, M Xu
Cochlear implant (CI) is an artificial electronic device which can provide a sense of sound to a patient with severe or profound hearing loss. Pathological changes have been observed after CI surgery, which might influence the effectiveness of the CI procedure. In this review, we divided the postoperative pathological changes of the temporal bone into two categories according to different stages: immediate trauma and delayed side effects. Immediate trauma might arise from traumatic insertion of the electrode during CI surgery, which included trauma at cochleostomy site, lateral wall trauma, basilar membrane injury, osseous lamina fracture and modiolar injury...
October 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/27734979/the-audiological-and-take-results-of-perichondrium-attached-cartilage-island-graft-in-tympanoplasty-pacit
#15
F Solmaz, D Akduman, M Haksever, E Gündoğdu, M Yanılmaz, A Mescioğlu
Cartilage is one of the most preferable grafts for tympanoplasty (TPL). The anatomical and audiological results and take rates of perichondrium attached cartilage island graft in tympanoplasty (PACIT) are presented herein. One hundred ninety four ears of 191 patients (108 male, 83 female) were evaluated retrospectively in terms of the type of surgery, graft take rate and hearing results. Type I, II, and III TPL were performed in 127 (65.46%), 45 (23.20%), and 22 (11.34%) ears, respectively. The overall mean preoperative pure tone average-air bone gaps (PTA-ABGs) for TPL types were 33...
August 2016: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/27723017/hyperbaric-oxygen-therapy-in-the-treatment-of-sudden-sensorineural-hearing-loss-a-retrospective-analysis-of-outcomes
#16
Susannah Sherlock, Kenneth Thistlethwaite, Alessandro Rubini, Mohsina Khatun, Christopher Perry, Alexis Tabah
OBJECTIVE: To analyse predictive factors affecting outcome after treatment with hyperbaric oxygen (HBOT) in patients with idiopathic sudden sensorineural hearing loss (ISSHL). METHODS: This is a retrospective audit of outcome in 77 consecutive patients referred for consideration of HBOT for ISSHL for either adjunctive treatment or after failure of steroid therapy. The hearing measured from the pre- and post-HBOT pure-tone audiogram (PTA4) at four frequencies; 500 Hz, 1 kHz, 2 kHz and 4 kHz, was averaged and compared...
September 2016: Diving and Hyperbaric Medicine: the Journal of the South Pacific Underwater Medicine Society
https://www.readbyqxmd.com/read/27716598/clinical-analysis-of-paper-patch-myringoplasty-in-patients-with-tympanic-membrane-perforations
#17
Dong Hee Lee, Jisun Kim, Eunhye Shin, Yulgyun Kim, Yesun Cho
OBJECTIVE: The aims of our study were to evaluate the outcome of office-based paper patch grafting in tympanic membrane (TM) perforation regardless of the disease duration or etiology and to compare the clinical factors influencing the successful closure of perforation between the success and failure groups of paper patch myringoplasty. MATERIALS AND METHODS: This is a retrospective study of 114 patients that underwent paper patch myringoplasty in an outpatient setting (success group, 83 cases; failure group, 31 cases)...
August 2016: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/27635185/an-overlapping-case-of-alport-syndrome-and-thin-basement-membrane-disease
#18
Mashriq Alganabi, Ahmad Eter
We report a case of a 48-year-old male who presented with hematuria of at least 10 years, and has a daughter with hematuria as well. The patient has a history of degenerative hearing loss, decreased vision and cataract formation, but no diabetes, hypertension or proteinuria. A full serology and urology workup was negative for any abnormality. A kidney biopsy for the patient revealed a diagnosis of Alport syndrome but was unable to rule out thin basement membrane disease. The biopsy was inconclusive in making the diagnosis but the patient's clinical presentation led to the diagnosis of Alport syndrome...
October 2016: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/27627812/x-linked-and-autosomal-recessive-alport-syndrome-pathogenic-variant-features-and-further-genotype-phenotype-correlations
#19
Judith Savige, Helen Storey, Hae Il Cheong, Hee Gyung Kang, Eujin Park, Pascale Hilbert, Anton Persikov, Carmen Torres-Fernandez, Elisabet Ars, Roser Torra, Jens Michael Hertz, Mads Thomassen, Lev Shagam, Dongmao Wang, Yanyan Wang, Frances Flinter, Mato Nagel
Alport syndrome results from mutations in the COL4A5 (X-linked) or COL4A3/COL4A4 (recessive) genes. This study examined 754 previously- unpublished variants in these genes from individuals referred for genetic testing in 12 accredited diagnostic laboratories worldwide, in addition to all published COL4A5, COL4A3 and COL4A4 variants in the LOVD databases. It also determined genotype-phenotype correlations for variants where clinical data were available. Individuals were referred for genetic testing where Alport syndrome was suspected clinically or on biopsy (renal failure, hearing loss, retinopathy, lamellated glomerular basement membrane), variant pathogenicity was assessed using currently-accepted criteria, and variants were examined for gene location, and age at renal failure onset...
2016: PloS One
https://www.readbyqxmd.com/read/27614962/parental-views-on-otitis-media-systematic-review-of-qualitative-studies
#20
REVIEW
Shingisai Chando, Christian Young, Jonathan C Craig, Hasantha Gunasekera, Allison Tong
UNLABELLED: This study aims to describe parental experiences and perspectives of caring for a child with otitis media. We conducted a systematic review of qualitative studies on parental perspectives on caring for a child with otitis media. We searched electronic databases to July 2015. Seventeen studies involving 284 participants from six countries were included. We identified seven themes: diminishing competency (guilt over failure to identify symptoms, helpless and despairing, fear of complications, disempowered and dismissed); disrupting life schedules (disturbing sleep, interfering with work, burden on family); social isolation (stigma and judgement, sick consciousness); threatening normal development (delaying growth milestones, impairing interpersonal skills, impeding education); taking ownership (recognising symptoms, diagnostic closure, working the system, protecting against physical trauma, contingency planning); valuing support (needing respite, depending on community, clinician validation); and cherishing health (relief with treatment success, inspiring resilience)...
October 2016: European Journal of Pediatrics
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