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Pediatric hearing loss

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https://www.readbyqxmd.com/read/28184249/atlantoaxial-rotatory-fixation-as-a-rare-complication-from-head-positioning-in-otologic-surgery-report-of-two-cases-in-young-children
#1
Hiroshi Sakaida, Koji Akeda, Akihiro Sudo, Kazuhiko Takeuchi
BACKGROUND: Atlantoaxial rotatory fixation is a condition in which the first and second vertebrae of the cervical spine become interlocked in a rotated position. This condition can result in serious consequences and thus have a significant impact on patients, especially when diagnosis and treatment are delayed. Some cases of atlantoaxial rotatory fixation have been described in association with otologic surgery or plastic surgery involving the ear. We present the cases of two pediatric patients who developed atlantoaxial rotatory fixation following otologic surgery and we review the relevant literature...
2017: Patient Safety in Surgery
https://www.readbyqxmd.com/read/28167016/lateral-skull-base-surgery-in-a-pediatric-population-a-25-year-experience-in-a-referral-skull-base-center
#2
Golda Grinblat, Sampath Chandra Prasad, Andrea Fulcheri, Melissa Laus, Alessandra Russo, Mario Sanna
OBJECTIVE: To analyze the pathology and surgical outcomes of lateral skull base (LSB) procedures in a pediatric population. STUDY DESIGN: Retrospective case review in a referral skull base center. METHODS: Charts of pediatric patients who underwent defined LSB procedures from 1983 to 2015 for various pathologies were evaluated at our center. A systematic review of literature was performed and our results were compared with the literature. RESULTS: 63 patients presented with 65 diseased ears...
March 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28141652/tracheal-cartilaginous-sleeve-in-syndromic-craniosynostosis-an-underrecognized-source-of-significant-morbidity-and-mortality
#3
Brent B Pickrell, Jesse D Meaike, Karina T Cañadas, Binoy M Chandy, Edward P Buchanan
Tracheal cartilaginous sleeve (TCS) is a rare and previously unrecognized source of morbidity and mortality in patients with certain craniosynostosis syndromes. There is a paucity of reporting on this airway anomaly, and the true incidence of TCS is largely unknown. The purpose of this study was to investigate the incidence of TCS among patients with syndromic craniosynostosis at our institution. Patients with syndromic craniosynostosis who underwent direct bronchoscopy and laryngoscopy were evaluated retrospectively by pediatric otolaryngologists for the presence of TCS and associated anomalies...
January 30, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28109500/is-routine-audiometric-testing-necessary-for-children-with-isolated-preauricular-lesions
#4
Grace T Wu, Conor Devine, Allen Xu, Katie Geelan-Hansen, Samantha Anne
INTRODUCTION: Preauricular lesions, including tags, pits, sinuses, and cysts are commonly seen. Some studies have shown increased incidence of hearing loss in these patients but other studies have failed to corroborate this finding. The purpose of this study is to evaluate the incidence of hearing loss in patients with isolated preauricular lesions. METHODS: Retrospective chart review of all pediatric otolaryngology patients seen at a tertiary academic center between 2008 and 2014...
February 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28109477/clinical-outcomes-following-cochlear-implantation-in-children-with-inner-ear-anomalies
#5
Amal Isaiah, Daniel Lee, Felicity Lenes-Voit, Melissa Sweeney, Walter Kutz, Brandon Isaacson, Peter Roland, Kenneth H Lee
OBJECTIVE: A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported. METHODS: The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1-18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification...
February 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28106625/long-term-hearing-results-after-ossiculoplasty
#6
Matthew D Cox, Aaron Trinidade, James Shep Russell, John L Dornhoffer
OBJECTIVES: To determine if the OOPS index is predictive of long-term hearing results after ossiculoplasty. STUDY DESIGN: Case series with retrospective chart review. SETTING: Tertiary care otology practice. PATIENTS: Adult and pediatric patients (3-88 years of age). INTERVENTIONS: Ossiculoplasty with cartilage tympanoplasty, with or without mastoidectomy. OUTCOME MEASURES: Primary outcome measures included short-term hearing results (pure-tone average air-bone gap [PTA-ABG] measured between 60 days and 1 year after surgery), long-term hearing results (PTA-ABG measured ≥5 years after surgery), and the rate of successful ABG closure to ≤20 dB...
January 18, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28092097/complications-and-parent-satisfaction-in-pediatric-osseointegrated-bone-conduction-hearing-implants
#7
Kenny H Chan, Dexiang Gao, Emily L Jensen, Gregory C Allen, Stephen P Cass
OBJECTIVE: To assess long-term complication rate and parental satisfaction of osseointegrated bone conduction hearing implants (OBCHIs). STUDY DESIGN: Retrospective chart review of children undergoing OBCHIs. METHODS: A retrospective chart review of children undergoing OBCHIs for the treatment of conductive, mixed, and single-sided sensorineural hearing loss in children. RESULTS: Forty-five subjects were identified with 0...
January 16, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28060175/cochlear-nerve-deficiency-and-brain-abnormalities-in-pediatric-patients
#8
Thierry Morlet, Markian Pazuniak, Robert C O'Reilly, Vinay Kandula, Arabinda K Choudhary
HYPOTHESIS: To investigate the intracranial abnormalities present in children with cochlear nerve deficiency (CND), including abnormalities of other cranial nerves, and to describe their auditory abilities. BACKGROUND: The prevalence of CND has increased with the development of high resolution magnetic resonance imaging (MRI). There are varying degrees of CND from true aplasia to hypoplasia. The etiology of CND remains unclear and it may be associated with intracranial abnormalities in some instances...
March 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28048024/su-f-t-116-predicting-iq-and-the-risk-of-hearing-loss-following-proton-versus-photon-radiotherapy-for-pediatric-brain-tumor-patients
#9
D Fortin, A Ng, D Tsang, M Sharpe, N Laperriere, D Hodgson
PURPOSE: The increased sparing of normal tissues in intensity modulated proton therapy (IMPT) compared to photon intensity modulated radiotherapy (IMRT) in brain tumor treatments should translate into improved neurocognitive outcomes. Models were used to estimate the intelligence quotient (IQ) and the risk of hearing loss 5 years post radiotherapy and to compare outcomes of proton against photon in pediatric brain tumors. METHODS: Patients who had received radical IMRT were randomly selected from our retrospective database: 10 cases each of craniopharyngioma, ependymoma and medulloblastoma, and 20 cases of glioma...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28040203/inner-ear-malformations-a-practical-diagnostic-approach
#10
M Mazón, E Pont, A Montoya-Filardi, J Carreres-Polo, F Más-Estellés
Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted...
December 28, 2016: Radiología
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#11
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28027286/the-relationship-of-the-middle-ear-effusion-microbiome-to-secretory-mucin-production-in-pediatric-patients-with-chronic-otitis-media
#12
Anna Krueger, Stéphanie Val, Marcos Pérez-Losada, Karuna Panchapakesan, Joe Devaney, Vanessa Duah, Christine DeMasson, Marian Poley, Mary Rose, Diego Preciado
BACKGROUND: Acute otitis media (AOM), an infection of the middle ear, can become chronic (COM) following multiple episodes. Microbial influence on COM remains unclear. It's been reported that mucin glycoproteins are required for middle ear immune defense against pathogens. We aim to characterize the middle ear effusion (MEE) microbiome using high-throughput sequencing and assess potential associations in microbiome diversity with the presence of the secretory mucins MUC5B and MUC5AC. We hypothesize that MEEs containing MUC5B will exhibit a microbiome largely devoid of typical AOM bacteria...
December 23, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28004468/first-2-cases-with-thiamine-responsive-megaloblastic-anemia-in-the-czech-republic-a-rare-form-of-monogenic-diabetes-mellitus-a-novel-mutation-in-the-thiamine-transporter-slc19a2-gene-intron-1-mutation-c-204-2t-g
#13
Renata Pomahačová, Jana Zamboryová, Josef Sýkora, Petra Paterová, Karel Fiklík, Tomáš Votava, Zdeňka Černá, Petr Jehlička, Václav Lád, Ivan Šubrt, Jiří Dort, Eva Dortová
Thiamine-responsive megaloblastic anemia (TRMA) is a rare autosomal recessive disorder caused by mutations in the SLC19A2 gene. To date at least 43 mutations have been reported for the gene encoding a plasma membrane thiamine transporter protein (THTR-1). TRMA has been reported in less than 80 cases worldwide. Here, we illustrate 2 female patients with TRMA first diagnosed in the Czech Republic and in central Europe being confirmed by sequencing of the THTR-1 gene SLC19A2. Both subjects are compound heterozygotes with 3 different mutations in the SLC19A2 gene...
December 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27956721/otologic-and-audiologic-outcomes-in-pediatric-patients-with-velo-cardio-facial-22q11-deletion-syndrome
#14
Forest W Weir, Sarah A Wallace, David R White, Jonathan L Hatch, Shaun A Nguyen, Ted A Meyer
OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with velo-cardio-facial syndrome (VCFS) and to compare these characteristics with patient demographics and other otologic factors. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGenDB with a diagnosis of velo-cardio-facial syndrome or DiGeorge syndrome...
January 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/27939998/cochlear-implantation-in-autistic-children-with-profound-sensorineural-hearing-loss
#15
Magdalena Lachowska, Agnieszka Pastuszka, Zuzanna Łukaszewicz-Moszyńska, Lidia Mikołajewska, Kazimierz Niemczyk
INTRODUCTION: Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. OBJECTIVE: The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability...
November 19, 2016: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/27925622/relation-between-amniotic-fluid-infection-or-cytokine-levels-and-hearing-screen-failure-in-infants-at-32-wk-gestation-or-less
#16
Eun Young Jung, Byung Yoon Choi, Jihye Rhee, Jaehong Park, Soo-Hyun Cho, Kyo Hoon Park
BACKGROUND: To determine whether the presence of intra-amniotic infection and elevated proinflammatory cytokine levels in amniotic fluid (AF) are associated with failure in the newborn hearing screen (NHS) test in very preterm neonates. METHODS: This is a retrospective cohort study of 112 premature singleton neonates born to women with preterm labor or preterm premature rupture of membranes at ≤32 wk. AF obtained through amniocentesis was cultured, and interleukin-6 (IL-6) and IL-8 levels were determined...
December 7, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27894912/hearing-thresholds-at-high-frequency-in-patients-with-cystic-fibrosis-a-systematic-review
#17
Debora T M Caumo, Lúcia B Geyer, Adriana R Teixeira, Sérgio S M Barreto
INTRODUCTION: High-frequency audiometry may contribute to the early detection of hearing loss caused by ototoxic medications. Many ototoxic drugs are widely used in the treatment of patients with cystic fibrosis. Early detection of hearing loss should allow known harmful drugs to be identified before the damage affects speech frequencies. The damage caused by ototoxicity is irreversible, resulting in important social and psychological consequences. In children, hearing loss, even when restricted to high frequencies, can affect the development of language...
November 9, 2016: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/27886419/functional-characterization-of-a-novel-loss-of-function-mutation-of-prps1-related-to-early-onset-progressive-nonsyndromic-hearing-loss-in-koreans-dfnx1-potential-implications-on-future-therapeutic-intervention
#18
So Young Kim, Ah Reum Kim, Nayoung K D Kim, Chung Lee, Jin Hee Han, Min Young Kim, Eun-Hee Jeon, Woong-Yang Park, Rahul Mittal, Denise Yan, Xue Zhong Liu, Byung Yoon Choi
BACKGROUND: The symptoms of phosphoribosyl pyrophosphate synthetase 1 (PRPS1) deficiency diseases have been reported to be alleviated by medication. In the present study, we report biochemical data that favor PRPS1 deficiency-related hearing loss as a potential target for pharmaceutical treatment. METHODS: We recruited 42 probands from subjects aged less than 15 years with a moderate degree of nonsyndromic autosomal-recessive or sporadic sensorineural hearing loss (SNHL) in at least one side...
November 2016: Journal of Gene Medicine
https://www.readbyqxmd.com/read/27885978/factors-affecting-daily-cochlear-implant-use-in-children-datalogging-evidence
#19
Vijayalakshmi Easwar, Joseph Sanfilippo, Blake Papsin, Karen Gordon
BACKGROUND: Children with profound hearing loss can gain access to sound through cochlear implants (CIs), but these devices must be worn consistently to promote auditory development. Although subjective parent reports have identified several factors limiting long-term CI use in children, it is also important to understand the day-to-day issues which may preclude consistent device use. In the present study, objective measures gathered through datalogging software were used to quantify the following in children: (1) number of hours of CI use per day, (2) practical concerns including repeated disconnections between the external transmission coil and the internal device (termed "coil-offs"), and (3) listening environments experienced during daily use...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27885975/hearing-benefit-and-rated-satisfaction-in-children-with-unilateral-conductive-hearing-loss-using-a-transcutaneous-magnetic-coupled-bone-conduction-hearing-aid
#20
Melissa J Polonenko, Lora Carinci, Karen A Gordon, Blake C Papsin, Sharon L Cushing
BACKGROUND: Bilateral hearing is important for learning, development, and function in complex everyday environments. Children with conductive and mixed hearing loss (HL) have been treated for years with percutaneous coupling through an abutment, which achieves powerful output, but the implant site is susceptible to skin reactions and trauma. To overcome these complications, transcutaneous magnetic coupling systems were recently introduced. PURPOSE: The purpose of the study was to evaluate whether the new transcutaneous magnetic coupling is an effective coupling paradigm for bone-conduction hearing aids (BCHAs)...
November 2016: Journal of the American Academy of Audiology
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