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Sensorineural hearing loss

Thayse Bienert Goetze, Pricila Sleifer, Rafael Fabiano Machado Rosa, Alessandra Pawelec da Silva, Carla Graziadio, Paulo Ricardo Gazzola Zen
Oculoauriculovertebral spectrum (OAVS), also known as Goldenhar syndrome, is considered a condition associated to failing of embryogenesis involving the first and second branchial arches, leading to structural abnormalities arising from it. The aim of this study is to verify the hearing features presented by patients with OAVS and provide additional information that may contribute to improvement of speech therapy. The sample consisted of 10 individuals diagnosed with OAVS and cared for by the Clinical Genetics Service...
October 24, 2016: American Journal of Medical Genetics. Part A
Xiaohui Duan, Weihong Gu, Ying Hao, Renbin Wang, Hong Wen, Shaojie Sun, Jinsong Jiao, Dongsheng Fan
Myelin protein zero (MPZ) is a major component of compact myelin in peripheral nerves. Mutations in MPZ have been associated with different Charcot-Marie-Tooth disease (CMT) phenotypes (CMT1B, CMT2I/J, CMTDI), Dejerine-Sottas syndrome, and congenital hypomyelination neuropathy. Here, we report phenotypic variability in a four-generation Chinese family with the MPZ mutation Asp121Asn. Genetic testing was performed on nine family members and 200 controls. Clinical, electrophysiological and skeletal muscle MRI assessments were available for review in six family members...
2016: Frontiers in Aging Neuroscience
A R Fetoni, R Rolesi, F Paciello, S L M Eramo, C Grassi, D Troiani, G Paludetti
Experimental and human investigations have raised the level of concern about the potential ototoxicity of organic solvents and their interaction with noise. The main objective of this study was to characterize the effects of the combined noise and styrene exposure on hearing focusing on the mechanism of damage on the sensorineural cells and supporting cells of the organ of Corti and neurons of the ganglion of Corti. The impact of single and combined exposures on hearing was evaluated by auditory functional testing and histological analyses of cochlear specimens...
October 18, 2016: Free Radical Biology & Medicine
Melinda Vass, Ágnes Diószegi, Norbert Németh, Viktória Sógor, Sándor Baráth, Eszter Szalai, László Módis, Soltész Pál
Rheopheresis is an extracorporal selective double-filtration procedure. In the first part of the treatment the blood is passes through the plasma filter, which separates blood cells from the plasma. Then the plasma flow to a second filter called MONET (Membranefiltration Optimised Novel Extracorporal Treatment). The MONET filter retains high molecular weight proteins such LDL, Lp(a), fibrinogen, α2 macroglobulin, vWF and IgM. Hereby the whole blood and plasma viscosity decrease, improves microcirculation, and has a positive effect on lipid profile as well...
October 15, 2016: Clinical Hemorheology and Microcirculation
Poornapriya Ramamurthy, Joshua B White, Joong Yull Park, Richard I Hume, Fumi Ebisu, Flor Mendez, Shuichi Takayama, Kate F Barald
BACKGROUND: To send meaningful information to the brain, an inner ear cochlear implant (CI) must become closely coupled to as large and healthy a population of remaining Spiral Ganglion Neurons (SGN) as possible. Inner ear gangliogenesis depends on macrophage migration inhibitory factor (MIF), a directionally attractant neurotrophic cytokine made by both Schwann and supporting cells (Bank et al., 2012). MIF-induced mouse embryonic stem cell (mESC)-derived "neurons" could potentially substitute for lost or damaged SGN...
October 19, 2016: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Lianhua Sun, Xiaohua Li, Jun Shi, Xiuhong Pang, Yechen Hu, Xiaowen Wang, Hao Wu, Tao Yang
Waardenburg syndrome (WS) characterized by sensorineural hearing loss and pigmentary abnormalities is genetically heterogeneous and phenotypically variable. This study investigated the molecular etiology and genotype-phenotype correlation of WS in 36 Chinese Han deaf probands and 16 additional family members that were clinically diagnosed with WS type I (WS1, n = 8) and type II (WS2, n = 42). Mutation screening of six WS-associated genes detected PAX3 mutations in 6 (86%) of the 7 WS1 probands. Among the 29 WS2 probands, 13 (45%) and 10 (34%) were identified with SOX10 and MITF mutations, respectively...
October 19, 2016: Scientific Reports
John Patton, Carmen Brewer, Wade Chien, Jennifer J Johnston, Andrew J Griffith, Leslie G Biesecker
Variants in the unconventional myosin gene, MYO1A, have been reported to cause non-syndromic sensorineural hearing loss with a pattern of autosomal dominant inheritance. Others have challenged this association. We used a genotypic ascertainment study design to test the association of MYO1A variants with hearing loss. We evaluated MYO1A variants from a cohort of 951 individuals with exome sequencing who were not ascertained for hearing loss. Five individuals had one of two variants claimed to be associated with sensorineural hearing loss in a prior study and 33 individuals had one of 13 predicted deleterious variants...
October 19, 2016: European Journal of Human Genetics: EJHG
Israel Pena, Erin Y Chew, Barcleigh P Landau, Joseph T Breen, Jose P Zevallos, Jeffrey T Vrabec
OBJECTIVE: To investigate the prevalence of vestibular schwannoma (VS) and asymmetric sensorineural hearing loss in the Veterans Administration hospital population and analyze a more efficient method of diagnosing VS in a population with significant noise exposure. STUDY DESIGN: Retrospective review of South Central (VISN 16) Veterans Administration hospitals. METHODS: Record query for ICD-9 codes for asymmetric sensorineural hearing loss or VS between 1999 and 2012...
October 14, 2016: Otology & Neurotology
Reuven Ishai, Christopher F Halpin, Jennifer J Shin, Michael J McKenna, Alicia M Quesnel
OBJECTIVES: 1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. STUDY DESIGN: Retrospective audiometric database and chart review. SETTING: Tertiary referral center. PATIENTS: Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram...
October 12, 2016: Otology & Neurotology
Jason Fleming, Dianna Quan
We present a patient with congenital spinal muscular atrophy associated with pain, subjective sensory loss, right talipes equinovarus, delayed walking, and progressive gait impairment. A sister and niece reportedly had Charcot-Marie-Tooth 1A, but the patient's electromyogram showed an axonal motor neuropathy or neuronopathy. We identified a c.806G>A TRPV4 gene mutation causing an Arg269His amino acid substitution. TRPV4 mutations cause variable phenotypes including axonal sensorimotor neuropathy and motor neuropathy or neuronopathy...
September 16, 2016: Neuromuscular Disorders: NMD
Michael C Topf, David W Hsu, Douglas R Adams, Tingting Zhan, Stanley Pelosi, Thomas O Willcox, Brian McGettigan, Kyle W Fisher
PURPOSE: To determine the rate of persistent tympanic membrane perforation after intratympanic steroid injection. To determine which comorbid conditions and risk factors are associated with prolonged time to perforation closure following intratympanic steroid injection. MATERIALS AND METHODS: Clinical data were gathered for patients who had undergone intratympanic steroid injection to treat sudden sensorineural hearing loss or Ménière's disease. Primary outcomes analysis included rate of persistent tympanic membrane perforation, defined as perforation at least 90days following last injection, and time to perforation healing...
September 28, 2016: American Journal of Otolaryngology
Yuka Morita, Kuniyuki Takahashi, Shuji Izumi, Yamato Kubota, Shinsuke Ohshima, Arata Horii
OBJECTIVE: Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. STUDY DESIGN: Retrospective chart review...
September 30, 2016: Otology & Neurotology
Nikolaus E Wolter, Sharon L Cushing, Luis D Vilchez Madrigal, Adrian L James, Jennifer Campos, Blake C Papsin, Karen A Gordon
OBJECTIVE: To determine if children with unilateral sensorineural hearing loss (UHL) demonstrate impaired balance compared with their normal hearing (NH) peers. STUDY DESIGN: Prospective, case-control study. METHODS: Balance was assessed in14 UHL and 14 NH children using the Bruininks-Oseretsky Test-2 (BOT-2) and time to fall (TTF) in an immersive, virtual-reality laboratory. Postural control was quantified by center of pressure (COP) using force plates...
September 30, 2016: Otology & Neurotology
Thomas Sené, Olivier Lidove, Joel Sebbah, Jean-Marc Darondel, Hervé Picard, Laurent Aaron, Olivier Fain, Thierry Zenone, Dominique Joly, Philippe Charron, Jean-Marc Ziza
The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy...
October 2016: Medicine (Baltimore)
Yi Zhang, Bo Liu, Rui Wang, Ruo Jia, Xin Gu
BACKGROUND: Meniere's disease is a unique, progressive disease of the inner ear. The complex manifestation presents diagnostic challenges. The cochlear symptoms often present before vertigo and tend to be ignored. This study aimed to analyze the characteristics of cochlear symptoms and functions associated with Meniere's disease to investigate the regularity of the development of this disorder. METHODS: One-hundred fifteen patients who were diagnosed with definite unilateral Meniere's disease at the Hearing and Vestibular Clinic of the Department of Otorhinolaryngology of Beijing Tongren Hospital from August 2013 to November 2015 were recruited in this retrospective study...
2016: Chinese Medical Journal
A W Van Der Wal, P J A M Van Ooij, J A De Ru
OBJECTIVE: Sudden sensorineural hearing loss in divers may be caused by either inner-ear barotrauma or inner-ear decompression sickness. There is no consensus on the best treatment option. This study aimed to evaluate the therapeutic value of hyperbaric oxygen therapy for sudden sensorineural hearing loss in divers. METHOD: A literature review and three cases of divers with sudden sensorineural hearing loss treated with hyperbaric oxygen therapy are presented. RESULTS: Hyperbaric oxygen therapy resulted in hearing improvement in 80 per cent of patients: 39 per cent had hearing improvement and 41 per cent had full recovery...
October 17, 2016: Journal of Laryngology and Otology
Erika Barioni Mantello, Carla Dias da Silva, Eduardo Tanaka Massuda, Miguel Angelo Hyppolito, Ana Cláudia Mirândola Barbosa Dos Reis
Introduction Hearing difficulties can be minimized by the use of hearing aids. Objective The objective of this study is to assess the speech perception and satisfaction of hearing aids users before and after aid adaptation and to determine whether these measures are correlated. Methods The study was conducted on 65 individuals, 54% females and 46% males aged 63 years on average, after the systematic use of hearing aids for at least three months. We characterized subjectś personal identification data, the degree, and configuration of hearing loss, as well as aspects related to adaptation...
October 2016: International Archives of Otorhinolaryngology
Stephanie Kletke, Vaishnavi Batmanabane, Tianyang Dai, Ajoy Vincent, Shuning Li, Karen A Gordon, Blake C Papsin, Sharon L Cushing, Elise Héon
The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having Usher syndrome. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was done to determine the ocular phenotype...
October 15, 2016: Clinical Genetics
Neil S Patel, Jacob B Hunter, Brendan P O'Connell, Natalie M Bertrand, George B Wanna, Matthew L Carlson
OBJECTIVE: Patients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time. STUDY DESIGN: Retrospective review at two tertiary care academic referral centers. METHODS: Adults analyzed were diagnosed with SSCD by high-resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms...
October 14, 2016: Laryngoscope
I M Villarreal, D Méndez, J M Duque Silva, P Ortega Del Álamo
Introduction. Labyrinthine concussion is a term used to describe a rare cause of sensorineural hearing loss with or without vestibular symptoms occurring after head trauma. Isolated damage to the inner ear without involving the vestibular organ would be designated as a cochlear labyrinthine concussion. Hearing loss is not a rare finding in head trauma that involves petrous bone fractures. Nevertheless it generally occurs ipsilateral to the side of the head injury and extraordinarily in the contralateral side and moreover without the presence of a fracture...
2016: Case Reports in Otolaryngology
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