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https://www.readbyqxmd.com/read/28324246/opinions-of-hearing-parents-about-the-causes-of-hearing-impairment-of-their-children-with-biallelic-gjb2-mutations
#1
Aisen V Solovyev, Lilya U Dzhemileva, Olga L Posukh, Nikolay A Barashkov, Marita S Bady-Khoo, Semen L Lobov, Natalya Yu Popova, Georgii P Romanov, Nikolay N Sazonov, Alexander A Bondar, Igor V Morozov, Mikhail I Tomsky, Sardana A Fedorova, Elza K Khusnutdinova
Hereditary hearing impairment (HI) caused by recessive GJB2 mutations is a frequent sensory disorder. The results of the molecular-based studies of HI are widely used in various genetic test systems. However, the ethical aspects are less described than the genetic aspects. The concerns expressed by individuals from groups with genetic risks must be included in the counseling of patients and their families. For evaluation of subjective opinions of hearing parents about the presumed causes of HI of their children, we analyze the cohort of parents having children with confirmed hereditary HI caused by biallelic recessive GJB2 mutations (in a homozygous or a compound heterozygous state)...
March 21, 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28323747/supporting-a-youth-with-cerebellar-ataxia-into-adolescence
#2
Veronica Meneses, Zurisadai Gonzalez-Castillo, Veronica B Edgar, Marilyn Augustyn
Zoe, a 13-year-old white girl, presents as a new patient to your pediatric clinic with complaints of frequent emesis, anxiety, and learning problems, and previous diagnosis of cerebellar ataxia. Parents accompany Zoe and state, "it is really hard for her to go out, she gets sick and falls easily." She was born full term by vaginal delivery without complications. Given globally delayed milestones, she received early intervention services. Feeding problems began at infancy, including gastroesophageal reflux and aspiration pneumonia...
March 17, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28320186/neurological-complications-after-tuberculous-meningitis-in-a-multi-state-cohort-in-the-united-states
#3
Alexander E Merkler, Alexandra S Reynolds, Gino Gialdini, Nicholas A Morris, Santosh B Murthy, Kiran Thakur, Hooman Kamel
BACKGROUND AND PURPOSE: To assess the rate of neurological complications and mortality after tuberculous meningitis in the United States. METHODS: The authors performed a retrospective cohort study of all patients 18years or older hospitalized for tuberculous meningitis in California between 2005 and 2010, New York between 2006 and 2012, and Florida between 2005 and 2012. Outcomes of interest were mortality and the following neurological complications: stroke, seizure, hydrocephalus requiring a ventriculoperitoneal shunt, vision impairment, and hearing impairment...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28319495/endoscopic-stapes-surgery-a-comparison-with-microscopic-surgery
#4
Rhona Sproat, Constantina Yiannakis, Arunachalam Iyer
OBJECTIVE: To investigate postoperative audiological outcomes and complication rates for fully endoscopic and microscopic stapes surgery carried out by a single surgeon in one center. PATIENTS: All patients having undergone endoscopic and non-endoscopic stapes surgery for otosclerosis from September 2009 to August 2016 under a single surgeon. INTERVENTION(S): Stapedotomy using either an operating microscope or endoscope for visualization. Stapedotomy was carried out using a standard approach...
March 17, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28319479/brainstem-evoked-potential-indices-of-subcortical-auditory-processing-after-mild-traumatic-brain-injury
#5
Kathy R Vander Werff, Brian Rieger
OBJECTIVES: The primary aim of this study was to assess subcortical auditory processing in individuals with chronic symptoms after mild traumatic brain injury (mTBI) by measuring auditory brainstem responses (ABRs) to standard click and complex speech stimuli. Consistent with reports in the literature of auditory problems after mTBI (despite normal-hearing thresholds), it was hypothesized that individuals with mTBI would have evidence of impaired neural encoding in the auditory brainstem compared to noninjured controls, as evidenced by delayed latencies and reduced amplitudes of ABR components...
March 17, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28319315/maxillofacial-features-and-systemic-malformations-in-expanded-spectrum-hemifacial-microsomia
#6
Noah Cohen, Erica Cohen, Alberto Gaiero, Silvia Zecca, Graziella Fichera, Federica Baldi, Joseph Felix Giordanetto, Jacques Marie Mercier, Amnon Cohen
Hemifacial microsomia (HFM) is a rare, multisystemic congenital disease with estimated frequency of 1/26370 births in Europe. Most cases are sporadic and caused by unilateral abnormal morphogenesis of the first and second pharyngeal arches. The aim of this study is to define the types and frequency of maxillofacial and systemic malformations in HFM patients. This is a case series study of patients with HFM evaluated at a single institution. Data were acquired through history, physical examination, photographs, diagnostic radiology, and laboratory and analyzed by the FileMakerPro database on 95 patients (54F; 41M) of which 89 met the inclusion criteria...
March 20, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28318969/auditory-perceptual-evaluation-of-voice-quality-of-cochlear-implanted-and-normal-hearing-individuals-a-reliability-study
#7
Ana Cristina Coelho, Alcione Ghedini Brasolotto, Ana Carolina Nascimento Fernandes, Daniela Malta de Souza Medved, Eduardo Magalhães da Silva, Fayez Bahmad Júnior
OBJECTIVE: This study aimed to present an experience in rating voices of adults with normal hearing and adults with cochlear implants and critically examine the outcomes, discussing pros and cons of the methodology used. STUDY DESIGN: This is a cross-sectional, prospective study. METHODS: One hundred and fifty voice samples, consisting of 50 sustained vowels, 50 samples of connected speech, and 50 samples of conversational speech, belonging to 25 adults with hearing impairment with cochlear implants and 25 adults with normal hearing, were perceptually analyzed for inter-rater agreement and intra-rater reliability...
March 16, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28318810/incomplete-partition-type-iii-a-rare-and-difficult-cochlear-implant-surgical-indication
#8
Levent Sennaroğlu, Münir Demir Bajin
OBJECTIVE: Presenting the clinical features and treatment options for incomplete partition type-III. METHODS: Nine primary and 1 revision incomplete partition type-III cochlear implant cases treated between 2004 and 2015 in Hacettepe University Department of Otolaryngology were included in the study. Treatment options and particularly cochlear implantation tecnique were described. RESULTS: Nine primary and 1 revison cases were all succesfully implanted...
March 16, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28318405/the-association-between-age-related-hearing-impairment-and-metabolic-syndrome-in-korean-women-5-year-follow-up-observational-study
#9
Tae Su Kim, Eun Hui Kim, Jong Woo Chung
BACKGROUND: Although several observational studies showed a relationship between various conditions of metabolic syndrome (MetS) and hearing threshold, there are no studies about longitudinal audiometric results related MetS. The aim of this study was to investigate the association between MetS and age-related hearing impairment (ARHI) through a large, average 5-year longitudinal follow-up, clinical comparative analysis. MATERIALS AND METHODS: We recruited 1381 women older than 50 years who were enrolled in 2007 and reevaluated in 2012...
March 20, 2017: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/28317321/development-of-a-home-based-auditory-training-to-improve-speech-recognition-on-the-telephone-for-patients-with-cochlear-implants-a-randomized-trial
#10
Friedrich Ihler, Jenny Blum, Gino Steinmetz, Bernhard G Weiss, Stefan Zirn, Martin Canis
OBJECTIVES: Speech recognition on the telephone poses a challenge for patients with cochlear implants due to a reduced bandwidth of transmission. This trial evaluates a home-based auditory training with telephone-specific filtered speech material to improve sentence recognition. DESIGN: Randomized controlled parallel double-blind. SETTING: One tertiary referral center. PARTICIPANTS: 20 postlingually deafened patients with cochlear implants...
March 20, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28316922/miracle-cells-for-natural-dentistry-a-review
#11
REVIEW
Rani Somani, Shipra Jaidka, Neeti Bajaj, Sameksha Arora
Stem cells are undifferentiated cells that can differentiate into specialized cells. Recently, enormous growth has been seen in the recognition of stem cell-based therapies, which have the potential to ameliorate the life of patients with conditions that span from Parkinson's disease to cardiac ischemia to bone or tooth loss. This research has produced new but unexplored possibilities in the regeneration of different organs and tissues. Presently, research is focused on the proficiency of stem cells and their utilization in dentistry, which is gaining interest...
January 2017: Journal of Oral Biology and Craniofacial Research
https://www.readbyqxmd.com/read/28314816/characterization-of-a-human-point-mutation-of-vglut3-p-a211v-in-the-rodent-brain-suggests-a-non-uniform-distribution-of-the-transporter-in-synaptic-vesicles
#12
Lauriane Ramet, Johannes Zimmermann, Tiphaine Bersot, Odile Poirel, Stéphanie De Gois, Katlin Silm, Diana Yae Sakae, Nina Mansouri-Guilani, Marie-Josée Bourque, Louis-Eric Trudeau, Nicolas Pietrancosta, Stéphanie Daumas, Véronique Bernard, Christian Rosenmund, Salah El Mestikawy
The atypical vesicular glutamate transporter type 3 (VGLUT3) is expressed by sub-populations of neurons using acetylcholine, GABA or serotonin as neurotransmitters. In addition, VGLUT3 is expressed in the inner hair cells of the auditory system. A mutation (p.A211V) in the gene that encodes VGLUT3 is responsible for progressive deafness in two unrelated families. In this study, we investigated the consequences of the p.A211V mutation in cell cultures and in the central nervous system (CNS) of a mutant mouse...
March 17, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28314420/sport-injuries-of-the-ear-and-temporal-bone
#13
REVIEW
L Mariel Osetinsky, Grant S Hamilton, Matthew L Carlson
In cases of head trauma, the ear should be evaluated in all of its components. A good understanding of otologic and skull base anatomy enables a thorough trauma assessment of this complex anatomic region. Auricular laceration, abrasion, avulsion, hematoma, frostbite, otitis externa, exostosis, tympanic membrane perforation, ossicular discontinuity, perilymphatic fistula, labyrinthine concussion, temporal bone fracture, facial nerve paresis, and sensorineural hearing loss are a few of the more common otologic injuries seen in active patients...
April 2017: Clinics in Sports Medicine
https://www.readbyqxmd.com/read/28306710/are-intratympanic-corticosteroids-effective-for-m%C3%A3-ni%C3%A3-re%C3%A2-s-disease
#14
Ángela Chuang-Chuang, María A Baeza
Ménière’s disease affects the inner ear and its main symptoms are vertigo, hearing loss and fluctuating aural symptoms. Nowadays, there are many therapeutic alternatives, being the use of intratympanic corticosteroids one that has become popular. To answer this question, we searched in Epistemonikos database, which is maintained by screening multiple databases. We identified four systematic reviews including 15 studies overall, of which seven were randomized trials. We extracted data and generated a summary of findings table using the GRADE approach...
March 13, 2017: Medwave
https://www.readbyqxmd.com/read/28306653/new-scoring-system-for-evaluating-patulous-eustachian-tube-patients
#15
Ryoukichi Ikeda, Toshiaki Kikuchi, Hidetoshi Oshima, Hiromitsu Miyazaki, Hiroshi Hidaka, Tetsuaki Kawase, Yukio Katori, Toshimitsu Kobayashi
OBJECTIVE: To assess the efficacy of patulous Eustachian tube handicap inventory (PHI) for patulous Eustachian tube (PET) patients. STUDY DESIGN: Prospective. SETTING: Tertiary referral center. SUBJECTS: A prospective survey of medical records in Sen-En Hospital identified 31 ears of 31 patients with definite PET who received insertion of the silicone plug as surgical treatment group, 29 ears of 29 patients treated with self-instillation of physiological saline solution as conservative treatment group, and 29 ears of 29 patients of sensorineural hearing loss without findings of PET treated between June 2015 and December 2015...
March 16, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28306651/predicting-auditory-outcomes-from-radiological-imaging-in-cochlear-implant-patients-with-cochlear-nerve-deficiency
#16
Xingmei Wei, Yongxin Li, Biao Chen, Yue Gong, Qian-Jie Fu, Ting Liu, Danmo Cui, Qiaotong Su, Ying Shi
OBJECTIVE: To compare imaging and outcome data in cochlear implant (CI) patients with and without cochlear nerve deficiency (CND). STUDY DESIGN: Retrospective study comparing presurgical imaging (via high-resolution computed tomography and three-dimensional magnetic resonance imaging) to postsurgical auditory outcomes in CI patients with and without CND. PATIENTS: Forty-three CI patients with CND diagnosed according to preoperative imaging were included in the CND group...
March 16, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28306650/cochlear-transcriptome-following-acoustic-trauma-and-dexamethasone-administration-identified-by-a-combination-of-rna-seq-and-dna-microarray
#17
Yukihide Maeda, Ryotaro Omichi, Akiko Sugaya, Shin Kariya, Kazunori Nishizaki
AIM: To elucidate molecular mechanisms of noise-induced hearing loss (NIHL) and glucocorticoid therapy in the cochlea. BACKGROUND: Glucocorticoids are used to treat many forms of acute sensorineural hearing loss, but their molecular action in the cochlea remains poorly understood. METHODS: Dexamethasone was administered intraperitoneally immediately following acoustic overstimulation at 120 dB SPL for 2 hours to mice. The whole cochlear transcriptome was analyzed 12 and 24 hours following noise trauma and dexamethasone administration by both next-generation sequencing (RNA-seq) and DNA microarray...
March 16, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28306649/secondary-endolymphatic-hydrops
#18
Ashley P O'Connell Ferster, Sebahattin Cureoglu, Nevra Keskin, Michael M Paparella, Huseyin Isildak
HYPOTHESIS: A review of the most recent literature will provide clinicians with an update of secondary endolymphatic hydrops, aiding in diagnosis and treatment of affected patients. BACKGROUND: Secondary endolymphatic hydrops is a pathologic finding of the inner ear resulting in episodic vertigo and intermittent hearing loss. It is a finding for which extensive research is being performed. METHODS: A review of the most recent literature on secondary endolymphatic hydrops was performed using PubMed literature search...
March 16, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28304096/insertion-depth-impacts-speech-perception-and-hearing-preservation-for-lateral-wall-electrodes
#19
Brendan P O'Connell, Jacob B Hunter, David S Haynes, Jourdan T Holder, Matt M Dedmon, Jack H Noble, Benoit M Dawant, George B Wanna
OBJECTIVES: 1) Examine angular insertion depths (AID) and scalar location of Med-El (GmbH Innsbruck, Austria) electrodes; and 2) determine the relationship between AID and audiologic outcomes controlling for scalar position. STUDY DESIGN: Retrospective review. METHODS: Postlingually deafened adults undergoing cochlear implantation with Flex 24, Flex 28, and Standard electrode arrays (Med-El) were identified. Patients with preoperative and postoperative computed tomography scans were included so that electrode location and AID could be determined...
March 17, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28304075/steroid-intracochlear-distribution-differs-by-administration-method-systemic-versus-intratympanic-injection
#20
Jong Joo Lee, Jeong Hun Jang, Oak-Sung Choo, Hye Jin Lim, Yun-Hoon Choung
OBJECTIVE: Steroids have been widely used to treat inner-ear diseases such as sudden sensorineural hearing loss, tinnitus, and Meniere's disease. They can be given via either systemic or intratympanic (IT) injection. The purpose of the present study was to explore differences in intracochlear steroid distribution by the administration method employed (systemic vs. IT injection). STUDY DESIGN: Animal study. METHODS: Twenty-three Sprague-Dawley rats were given fluorescein isothiocyanate-labeled dexamethasone (FITC-DEX) three times (on successive days) via intraperitoneal (IP) or IT injection...
March 17, 2017: Laryngoscope
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