WORLD REPORT on HEARING the Cover Image Is an Artistic Representation of a Sound Wave Entering the Cochlea
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Screening Guide for Usher Syndrome
Screening Guide for Usher Syndrome Florida Outreach Project for Children and Young Adults Who Are Deaf-Blind Bureau of Exceptional Education and Student Services Florida Department of Education 2012 This publication was produced through the Bureau of Exceptional Education and Student Services (BEESS) Resource and Information Center, Division of Public Schools, Florida Department of Education, and is available online at http://www.fldoe.org/ese/pub-home.asp. For information on available resources, contact the BEESS Resource and Information Center (BRIC). BRIC website: http://www.fldoe.org/ese/clerhome.asp Bureau website: http://fldoe.org/ese/ Email: [email protected] Telephone: (850) 245-0475 Fax: (850) 245-0987 This document was developed by the Florida Instructional Materials Center for the Visually Impaired, Outreach Services for the Blind/Visually Impaired and Deaf/Hard-of-Hearing, and the Resource Materials and Technology Center for the Deaf/Hard-of-Hearing, special projects funded by the Florida Department of Education, Division of Public Schools, BEESS, through federal assistance under the Individuals with Disabilities Education Act (IDEA), Part B, in conjunction with the Florida Outreach Project for Children and Young Adults Who Are Deaf- Blind, H326C990032, which is funded by the Office of Special Education Programs, U.S. Department of Education. Information contained within this publication does not necessarily reflect the views of the U.S. Department of Education. Edited by: Susan Lascek, Helen Keller National Center Emily Taylor-Snell, Florida Project for Children and Young Adults Who Are Deaf-Blind Dawn Saunders, Florida Department of Education Leanne Grillot, Florida Department of Education Adapted with permission from the Nebraska Usher Syndrome Screening Project (2002) Copyright State of Florida Department of State 2012 Authorization for reproduction is hereby granted to the state system of public education consistent with section 1006.03(2), Florida Statutes. -
HEARING LOSS, DEAFNESS Ear32 (1)
HEARING LOSS, DEAFNESS Ear32 (1) Hearing Loss, Deafness Last updated: May 11, 2019 CLASSIFICATION, DIAGNOSIS ................................................................................................................. 1 METHODS OF COMMUNICATION FOR DEAF ............................................................................................ 2 MANAGEMENT ......................................................................................................................................... 2 HEARING AIDS (S. AMPLIFICATION) ....................................................................................................... 2 COCHLEAR IMPLANTS ............................................................................................................................ 2 AUDITORY BRAINSTEM IMPLANTS .......................................................................................................... 4 PREVENTION .......................................................................................................................................... 4 PEDIATRIC HEARING DEFICITS ............................................................................................................... 4 ETIOLOGY .............................................................................................................................................. 4 DIAGNOSIS ............................................................................................................................................. 6 TREATMENT .......................................................................................................................................... -
Hearing Loss in Patients with Extracranial Complications of Chronic Otitis Media
ORIGINAL ARTICLE Hearing loss in patients with extracranial complications of chronic otitis media Authors’ Contribution: BCDE AF A – Study Design Tomasz Przewoźny , Jerzy Kuczkowski B – Data Collection C – Statistical Analysis D – Data Interpretation Department of Otolaryngology, Medical University of Gdańsk, Gdańsk, Poland E – Manuscript Preparation F – Literature Search G – Funds Collection Article history: Received: 13.04.2017 Accepted: 10.04.2017 Published: 15.06.2017 ABSTRACT: Objective: A pure tone audiomety analysis of patients with extracranial complications of chronic suppurative otitis media (ECCSOM). Material and methods: We retrospectively analyzed audiometric data performed before treatment from 63 pa- tients with ECCSOM (56 single, 7 multiple complications) including groups of frequencies. Results: The greatest levels of hearing loss were noted for 6 and 8 kHz (79.0 and 75.7 dBHL) and for the frequency groups high tone average (76.1 dBHL). As regards the severity of hearing impairment in pure tone average the prev- alence of complications was as follows: labyrinthitis (77.8±33.6 dBHL), facial palsy (57.1±14.3 dBHL), perilymphatic fistula (53.9±19.9 dBHL) and mastoiditis (42.2±9.5 dBHL) (p=0.023). Conclusions: Hearing loss in ECCSOM is dominated by mixed, high-tone, moderate type of hearing loss, most pro- found in labyrinthitis. In 11% of patients the complication causes total deafness. KEYWORDS: chronic suppurative otitis media, complications extracranial, hearing loss INTRODUCTION ing discharge from the ear and mixed or conductive hearing loss can be observed [4-5]. Labyrinthitis is associated with Chronic suppurative otitis media (CSOM) is a destructive slowly progressive high-frequency sensorineural hearing loss disease of the ear. -
UPDATE VOLUME 20 • ISSUE 3 the Newsletter of the Council for Accreditation in Occupational Hearing Conservation
Fall 2008 UPDATE VOLUME 20 • ISSUE 3 The Newsletter of the Council for Accreditation in Occupational Hearing Conservation Hearing noise-induced hearing loss and further degradation of communication. If too much hearing protection is provided, Conservation the combined effects of the hearing loss and the attenuation provided by the hearing protector may result in critical sounds for the Hearing- and communication signals becoming inaudible. I’m often asked what type of hearing protector is best for Impaired Worker workers with hearing impairment. Considering that there is no ‘best’ HPD for all workers in any hearing category, it Introduction by Ted Madison should come as no surprise that no single type of device will The prevalence of hearing loss among persons enrolled meet the needs of all those with hearing loss. What seems in occupational hearing conservation programs (HCPs) is to be consistent, however, is that each case is unique, and difficult to determine. Recently, Tak and Calvert (2008) that extra time and effort is required to help these workers estimated that 11.4% of the overall US workforce reports find the right combination of protection, having hearing difficulty of varying communication and auditory awareness. degrees and that approximately ¼ of Consultation with an audiologist or other the hearing difficulty reported can hearing health care professional is also be attributed to employment. These an important step in most cases. estimates are based on analysis of data One valuable resource is the OSHA from the US National Health Interview Safety & Health Information Bulletin Survey (NHIS) that were collected (SHIB) titled “Hearing Conservation from 1997 to 2003. -
Pediatric Sensorineural Hearing Loss, Part 2: Syndromic And
Published May 19, 2011 as 10.3174/ajnr.A2499 Pediatric Sensorineural Hearing Loss, Part 2: REVIEW ARTICLE Syndromic and Acquired Causes B.Y. Huang SUMMARY: This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. C. Zdanski Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and M. Castillo illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. Syndromes discussed include BOR syndrome, CHARGE syndrome, Pendred syndrome, Waardenburg syndrome, and X-linked hearing loss with stapes gusher. We conclude the article with a review of acquired causes of childhood SNHL, including infections, trauma, and neoplasms. ABBREVIATIONS: BOR ϭ branchio-oto-renal; CISS ϭ constructive interference in steady state; IAC ϭ internal auditory canal; NF-2 ϭ neurofibromatosis type II; SCC ϭ semicircular canal; SNHL ϭ sensorineural hearing loss; T1WI ϭ T1-weighted image; T2 WI ϭ T2-weighted image he estimated prevalence of SNHL in patients younger than Table 1: Selected hereditary syndromes commonly associated with 1 T18 years of age is 6 per 1000, making it one of the leading SNHL causes of childhood disability and a common reason for oto- Inner Ear Malformations on Inner Ear Malformations laryngology referrals. Cross-sectional imaging is now rou- Imaging Not Common on Imaging tinely performed in these patients because it provides impor- Alagille syndrome Alport syndrome tant information about potential etiologies for hearing loss, Branchio-oto-renal syndrome Biotinidase deficiency defines the anatomy of the temporal bone and the central au- CHARGE syndrome Jervell and Lange-Nielsen syndrome ditory pathway, and identifies additional intracranial abnor- Klippel-Feil syndrome Norrie syndrome malities that may require further work-up. -
Electromagnetic Field and TGF-Β Enhance the Compensatory
www.nature.com/scientificreports OPEN Electromagnetic feld and TGF‑β enhance the compensatory plasticity after sensory nerve injury in cockroach Periplaneta americana Milena Jankowska1, Angelika Klimek1, Chiara Valsecchi2, Maria Stankiewicz1, Joanna Wyszkowska1* & Justyna Rogalska1 Recovery of function after sensory nerves injury involves compensatory plasticity, which can be observed in invertebrates. The aim of the study was the evaluation of compensatory plasticity in the cockroach (Periplaneta americana) nervous system after the sensory nerve injury and assessment of the efect of electromagnetic feld exposure (EMF, 50 Hz, 7 mT) and TGF‑β on this process. The bioelectrical activities of nerves (pre‑and post‑synaptic parts of the sensory path) were recorded under wind stimulation of the cerci before and after right cercus ablation and in insects exposed to EMF and treated with TGF‑β. Ablation of the right cercus caused an increase of activity of the left presynaptic part of the sensory path. Exposure to EMF and TGF‑β induced an increase of activity in both parts of the sensory path. This suggests strengthening efects of EMF and TGF‑β on the insect ability to recognize stimuli after one cercus ablation. Data from locomotor tests proved electrophysiological results. The takeover of the function of one cercus by the second one proves the existence of compensatory plasticity in the cockroach escape system, which makes it a good model for studying compensatory plasticity. We recommend further research on EMF as a useful factor in neurorehabilitation. Injuries in the nervous system caused by acute trauma, neurodegenerative diseases or even old age are hard to reverse and represent an enormous challenge for modern medicine. -
A Molecular and Genetic Analysis of Otosclerosis
A molecular and genetic analysis of otosclerosis Joanna Lauren Ziff Submitted for the degree of PhD University College London January 2014 1 Declaration I, Joanna Ziff, confirm that the work presented in this thesis is my own. Where information has been derived from other sources, I confirm that this has been indicated in the thesis. Where work has been conducted by other members of our laboratory, this has been indicated by an appropriate reference. 2 Abstract Otosclerosis is a common form of conductive hearing loss. It is characterised by abnormal bone remodelling within the otic capsule, leading to formation of sclerotic lesions of the temporal bone. Encroachment of these lesions on to the footplate of the stapes in the middle ear leads to stapes fixation and subsequent conductive hearing loss. The hereditary nature of otosclerosis has long been recognised due to its recurrence within families, but its genetic aetiology is yet to be characterised. Although many familial linkage studies and candidate gene association studies to investigate the genetic nature of otosclerosis have been performed in recent years, progress in identifying disease causing genes has been slow. This is largely due to the highly heterogeneous nature of this condition. The research presented in this thesis examines the molecular and genetic basis of otosclerosis using two next generation sequencing technologies; RNA-sequencing and Whole Exome Sequencing. RNA–sequencing has provided human stapes transcriptomes for healthy and diseased stapes, and in combination with pathway analysis has helped identify genes and molecular processes dysregulated in otosclerotic tissue. Whole Exome Sequencing has been employed to investigate rare variants that segregate with otosclerosis in affected families, and has been followed by a variant filtering strategy, which has prioritised genes found to be dysregulated during RNA-sequencing. -
NOISE and MILITARY SERVICE Implications for Hearing Loss and Tinnitus
NOISE AND MILITARY SERVICE Implications for Hearing Loss and Tinnitus Committee on Noise-Induced Hearing Loss and Tinnitus Associated with Military Service from World War II to the Present Medical Follow-up Agency Larry E. Humes, Lois M. Joellenbeck, and Jane S. Durch, Editors THE NATIONAL ACADEMIES PRESS Washington, DC www.nap.edu THE NATIONAL ACADEMIES PRESS • 500 Fifth Street, N.W. • Washington, DC 20001 NOTICE: The project that is the subject of this report was approved by the Governing Board of the National Research Council, whose members are drawn from the councils of the National Academy of Sciences, the National Academy of Engineering, and the Insti- tute of Medicine. The members of the committee responsible for the report were chosen for their special competences and with regard for appropriate balance. This study was supported by Contract No. V101(93)P-1637 #29 between the Na- tional Academy of Sciences and the Department of Veterans Affairs. Any opinions, find- ings, conclusions, or recommendations expressed in this publication are those of the author(s) and do not necessarily reflect the view of the organizations or agencies that provided support for this project. Library of Congress Cataloging-in-Publication Data Noise and military service : implications for hearing loss and tinnitus / Committee on Noise-Induced Hearing Loss and Tinnitus Associated with Military Service from World War II to the Present, Medical Follow- up Agency ; Larry E. Humes, Lois M. Joellenbeck, and Jane S. Durch, editors. p. ; cm. Includes bibliographical references. ISBN 0-309-09949-8 — ISBN 0-309-65307-X 1. Deafness—Etiology. -
Instruction Sheet: Otitis Externa
University of North Carolina Wilmington Abrons Student Health Center INSTRUCTION SHEET: OTITIS EXTERNA The Student Health Provider has diagnosed otitis externa, also known as external ear infection, or swimmer's ear. Otitis externa is a bacterial/fungal infection in the ear canal (the ear canal goes from the outside opening of the ear to the eardrum). Water in the ear, from swimming or bathing, makes the ear canal prone to infection. Hot and humid weather also predisposes to infection. Symptoms of otitis externa include: ear pain, fullness or itching in the ear, ear drainage, and temporary loss of hearing. These symptoms are similar to those caused by otitis media (middle ear infection). To differentiate between external ear infection and middle ear infection, the provider looks in the ear with an instrument called an otoscope. It is important to distinguish between the two infections, as they are treated differently: External otitis is treated with drops in the ear canal, while middle ear infection is sometimes treated with an antibiotic by mouth. MEASURES YOU SHOULD TAKE TO HELP TREAT EXTERNAL EAR INFECTION: 1. Use the ear drops regularly, as directed on the prescription. 2. The key to treatment is getting the drops down into the canal and keeping the medicine there. To accomplish this: Lie on your side, with the unaffected ear down. Put three to four drops in the infected ear canal, then gently pull the outer ear back and forth several times, working the medicine deeper into the ear canal. Remain still, good-ear-side-down for about 15 minutes. -
Recreational Noise-Induced Hearing Loss
Hearing loss due to recreational exposure to loud sounds A review World Health Organization Hearing loss due to recreational exposure to loud sounds A review World Health Organization Contributors: Etienne Krug, Maria Alarcos Cieza, Shelly Chadha, Laura Sminkey, Thais Morata, DeWet Swanepoel, Adrian Fuente, Warwick Williams, Joseph Cerquone, Ricardo Martinez, Gretchen Stevens, Margie Peden, Sowmya Rao, Paras Agarwal, Eighmey Zeeck, Anna Bladey, Malachi Arunda, Aileen Ncube. Graphics Credits: INIS Communications WHO Library Cataloguing-in-Publication Data Hearing loss due to recreational exposure to loud sounds: a review. 1.Hearing Loss, Noise-Induced. 2.Music. 3.Noise. 4.Recreation. 5.Noise. Transportation. 6.Adolescent. I.World Health Organization. ISBN 978 92 4 150851 3 (NLM classification: WV 270) © World Health Organization 2015 All rights reserved. Publications of the World Health Organization are available on the WHO website (http://www.who.int) or can be purchased from WHO Press, World Health Organization, 20 Avenue Appia, 1211 Geneva 27, Switzerland (tel.: +41 22 791 3264; fax: +41 22 791 4857; e-mail: [email protected]). Requests for permission to reproduce or translate WHO publications – whether for sale or for non- commercial distribution – should be addressed to WHO Press through the WHO website (http://www.who.int/about/licensing/copyright_form/en/index.html). The designations employed and the presentation of the material in this publication do not imply the expression of any opinion whatsoever on the part of the World Health Organization concerning the legal status of any country, territory, city or area or of its authorities, or concerning the delimitation of its frontiers or boundaries. -
Auditory System & Hearing
Auditory System & Hearing Chapters 9 part II Lecture 17 Jonathan Pillow Sensation & Perception (PSY 345 / NEU 325) Fall 2017 1 Cochlea: physical device tuned to frequency! • place code: tuning of different parts of the cochlea to different frequencies 2 The auditory nerve (AN): fibers stimulated by inner hair cells • Frequency selectivity: Clearest when sounds are very faint 3 Threshold tuning curves for 6 neurons (threshold = lowest intensity that will give rise to a response) Characteristic frequency - frequency to which the neuron is most sensitive threshold(dB) frequency (kHz) 4 Information flow in the auditory pathway • Cochlear nucleus: first brain stem nucleus at which afferent auditory nerve fibers synapse • Superior olive: brainstem region thalamus MGN in the auditory pathway where inputs from both ears converge • Inferior colliculus: midbrain nucleus in the auditory pathway • Medial geniculate nucleus (MGN): part of the thalamus that relays auditory signals to the cortex 5 • Primary auditory cortex (A1): First cortical area for processing audition (in temporal lobe) • Belt & Parabelt areas: areas beyond A1, where neurons respond to more complex characteristics of sounds 6 Basic Structure of the Mammalian Auditory System Comparing overall structure of auditory and visual systems: • Auditory system: Large proportion of processing before A1 • Visual system: Large proportion of processing after V1 7 Basic Structure of the Mammalian Auditory System Tonotopic organization: neurons organized spatially in order of preferred frequency • -
Deafness and Hearing Loss Caroline’S Story
Deafness and Hearing Loss Caroline’s Story Caroline is six years old, A publication of NICHCY with bright brown eyes and, at Disability Fact Sheet #3 June 2010 the moment, no front teeth, like so many other first graders. She also wears a hearing aid in each ear—and has done so since she was three, when she Caroline was immediately Hearing Loss was diagnosed with a moderate fitted with hearing aids. She in Children hearing loss. also began receiving special education and related services Hearing is one of our five For Caroline’s parents, there through the public school senses. Hearing gives us access were many clues along the way. system. Now in the first grade, to sounds in the world around Caroline often didn’t respond she regularly gets speech us—people’s voices, their to her name if her back was therapy and other services, and words, a car horn blown in turned. She didn’t startle at her speech has improved warning or as hello! noises that made other people dramatically. So has her vocabu- jump. She liked the TV on loud. lary and her attentiveness. She When a child has a hearing But it was the preschool she sits in the front row in class, an loss, it is cause for immediate started attending when she was accommodation that helps her attention. That’s because three that first put the clues hear the teacher clearly. She’s language and communication together and suggested to back on track, soaking up new skills develop most rapidly in Caroline’s parents that they information like a sponge, and childhood, especially before the have her hearing checked.