American Academy of Audiology Clinical Practice Guidelines
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Approach to Brain Malformations
Approach to Brain Malformations A General Imaging Approach to Brain CSF spaces. This is the basis for development of the Dandy- Malformations Walker malformation; it requires abnormal development of the cerebellum itself and of the overlying leptomeninges. Whenever an infant or child is referred for imaging because of Looking at the midline image also gives an idea of the relative either seizures or delayed development, the possibility of a head size through assessment of the craniofacial ratio. In the brain malformation should be carefully investigated. If the normal neonate, the ratio of the cranial vault to the face on child appears dysmorphic in any way (low-set ears, abnormal midline images is 5:1 or 6:1. By 2 years, it should be 2.5:1, and facies, hypotelorism), the likelihood of an underlying brain by 10 years, it should be about 1.5:1. malformation is even higher, but a normal appearance is no guarantee of a normal brain. In all such cases, imaging should After looking at the midline, evaluate the brain from outside be geared toward showing a structural abnormality. The to inside. Start with the cerebral cortex. Is the thickness imaging sequences should maximize contrast between gray normal (2-3 mm)? If it is too thick, think of pachygyria or matter and white matter, have high spatial resolution, and be polymicrogyria. Is the cortical white matter junction smooth or acquired as volumetric data whenever possible so that images irregular? If it is irregular, think of polymicrogyria or Brain: Pathology-Based Diagnoses can be reformatted in any plane or as a surface rendering. -
PDF in English
Case Report Article Labyrinthitis Ossificans. Report of One Case and Literature Review Leandro Ricardo Mattiola*, Mark Makowiecky**, Carlos Eduardo Guimarães de Salles**, Marcela Pozzi Cardoso***, Samir Cahali****. * ENT doctor. Post-graduation student in Head and Neck Surgery at HSPE-SP. ** 2nd yr. Resident Doctor in ENT and Head and Neck Surgery at HSPE-SP. *** ENT Doctor. Assistant doctor in the Otology Department at HSPE-SP. **** PhD in Otorhinolaryngology by UNIFESP. Head of ENT and Head and Neck Surgery Department at HSPE-SP. Institution: Hospital do Servidor Público Estadual de São Paulo - FMO. Departamento de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço. São Paulo / SP – Brazil. Address for correspondence: Leandro Ricardo Mattiola – Rua José de Magalhães 600 - Vila Clementino – São Paulo / SP – Brazil - Zip code: 04026-090 – Telephone/ Fax: (+55 11) 5088-8406 - E-mail: [email protected] Article received on May 31st, 2007. Article approved on November 8th, 2007. SUMMARY Introduction: Labyrinthitis ossificans is a pathology characterized by sensorioneural hearing loss; secondary to infectious process, which produces irreversible injury to inner ear. Objective: To report a labyrinthitis ossificans case and review the literature. Case Report: A seven-year-old male patient, with profound hearing loss in tonal audiometry; no response from brainstem audiometry and compatible CT findings. Conclusion: Labyrinthitis ossificans results ossification on the inner ear structures. Pacient presents profound irreversible hearing loss, followed or not by disequilibrium, that can have important implication on educational socio-development. Diagnosis is important for cochlear implantation cases of the selected cases. Key words: labirinthitis, cochlea, hearing loss, osteogenesis 300 Intl. Arch. Otorhinolaryngol., São Paulo, v.12, n.2, p. -
Correlations Between Audiogram and Objective Hearing Tests in Sensorineural Hearing Loss
International Tinnitus Journal, Vol. 5, No.2, 107-112 (1999) Correlations Between Audiogram and Objective Hearing Tests in Sensorineural Hearing Loss L. Bishara,1 J. Ben-David,l L. Podoshin,1 M. Fradis,l C.B. Teszler,l H. Pratt,2 T. Shpack,3 H. Feiglin,3 H. Hafner,3 and N. Herlinger2 I Department of Otolaryngology, Head and Neck Surgery, and 3Institute of Audiology, Bnai-Zion Medical Center, and 2Evoked Potentials Laboratory, Technion, Haifa, Israel Abstract: Owing to its subjective nature, behavioral pure-tone audiometry often is an unre liable testing method in uncooperative subjects, and assessing the true hearing threshold be comes difficult. In such cases, objective tests are used for hearing-threshold determination (i.e., auditory brainstem evoked potentials [ABEP] and frequency-specific auditory evoked potentials: slow negative response at 10 msec [SN-1O]). The purpose of this study was to evaluate the correlation between pure-tone audiogram shape and the predictive accuracy of SN-IO and ABEP in normal controls and in patients suf fering from sensorineural hearing loss (SNHL). One-hundred-and-fifty subjects aged 15 to 70, some with normal hearing and the remainder with SNHL, were tested prospectively in a double-blind design. The battery of tests included pure-tone audiometry (air and bone conduction), speech reception threshold, ABEP, and SN- 10. Patients with SNHL were divided into four categories according to audiogram shape (i.e., flat, ascending, descending, and all other shapes). The results showed that ABEP predicts behavioral thresholds at 3 kHz and 4 kHz in cases of high-frequency hearing loss. -
CASE REPORT 48-Year-Old Man
THE PATIENT CASE REPORT 48-year-old man SIGNS & SYMPTOMS – Acute hearing loss, tinnitus, and fullness in the left ear Dennerd Ovando, MD; J. Walter Kutz, MD; Weber test lateralized to the – Sergio Huerta, MD right ear Department of Surgery (Drs. Ovando and Huerta) – Positive Rinne test and and Department of normal tympanometry Otolaryngology (Dr. Kutz), UT Southwestern Medical Center, Dallas; VA North Texas Health Care System, Dallas (Dr. Huerta) Sergio.Huerta@ THE CASE UTSouthwestern.edu The authors reported no A healthy 48-year-old man presented to our otolaryngology clinic with a 2-hour history of potential conflict of interest hearing loss, tinnitus, and fullness in the left ear. He denied any vertigo, nausea, vomiting, relevant to this article. otalgia, or otorrhea. He had noticed signs of a possible upper respiratory infection, including a sore throat and headache, the day before his symptoms started. His medical history was unremarkable. He denied any history of otologic surgery, trauma, or vision problems, and he was not taking any medications. The patient was afebrile on physical examination with a heart rate of 48 beats/min and blood pressure of 117/68 mm Hg. A Weber test performed using a 512-Hz tuning fork lateral- ized to the right ear. A Rinne test showed air conduction was louder than bone conduction in the affected left ear—a normal finding. Tympanometry and otoscopic examination showed the bilateral tympanic membranes were normal. THE DIAGNOSIS Pure tone audiometry showed severe sensorineural hearing loss in the left ear and a poor speech discrimination score. The Weber test confirmed the hearing loss was sensorineu- ral and not conductive, ruling out a middle ear effusion. -
Hearing Screening Training Manual REVISED 12/2018
Hearing Screening Training Manual REVISED 12/2018 Minnesota Department of Health (MDH) Community and Family Health Division Maternal and Child Health Section 1 2 For more information, contact Minnesota Department of Health Maternal Child Health Section 85 E 7th Place St. Paul, MN 55164-0882 651-201-3760 [email protected] www.health.state.mn.us Upon request, this material will be made available in an alternative format such as large print, Braille or audio recording. 3 Revisions made to this manual are based on: Guidelines for Hearing Screening After the Newborn Period to Kindergarten Age http://www.improveehdi.org/mn/library/files/afternewbornperiodguidelines.pdf American Academy of Audiology, Childhood Screening Guidelines http://www.cdc.gov/ncbddd/hearingloss/documents/AAA_Childhood%20Hearing%2 0Guidelines_2011.pdf American Academy of Pediatrics (AAP), Hearing Assessment in Children: Recommendations Beyond Neonatal Screening http://pediatrics.aappublications.org/content/124/4/1252 4 Contents Introduction .................................................................................................................... 7 Audience ..................................................................................................................... 7 Purpose ....................................................................................................................... 7 Overview of hearing and hearing loss ............................................................................ 9 Sound, hearing, and hearing -
The Sex Pistols: Punk Rock As Protest Rhetoric
UNLV Retrospective Theses & Dissertations 1-1-2002 The Sex Pistols: Punk rock as protest rhetoric Cari Elaine Byers University of Nevada, Las Vegas Follow this and additional works at: https://digitalscholarship.unlv.edu/rtds Repository Citation Byers, Cari Elaine, "The Sex Pistols: Punk rock as protest rhetoric" (2002). UNLV Retrospective Theses & Dissertations. 1423. http://dx.doi.org/10.25669/yfq8-0mgs This Thesis is protected by copyright and/or related rights. It has been brought to you by Digital Scholarship@UNLV with permission from the rights-holder(s). You are free to use this Thesis in any way that is permitted by the copyright and related rights legislation that applies to your use. For other uses you need to obtain permission from the rights-holder(s) directly, unless additional rights are indicated by a Creative Commons license in the record and/ or on the work itself. This Thesis has been accepted for inclusion in UNLV Retrospective Theses & Dissertations by an authorized administrator of Digital Scholarship@UNLV. For more information, please contact [email protected]. INFORMATION TO USERS This manuscript has been reproduced from the microfilm master. UMI films the text directly from the original or copy submitted. Thus, some thesis and dissertation copies are in typewriter face, while others may be from any type of computer printer. The quality of this reproduction is dependent upon the quality of the copy submitted. Broken or indistinct print, colored or poor quality illustrations and photographs, print bleedthrough, substandard margins, and improper alignment can adversely affect reproduction. In the unlikely event that the author did not send UMI a complete manuscript and there are missing pages, these will be noted. -
BMC Ear, Nose and Throat Disorders Biomed Central
BMC Ear, Nose and Throat Disorders BioMed Central Case report Open Access Acute unilateral hearing loss as an unusual presentation of cholesteatoma Daniel Thio*1, Shahzada K Ahmed2 and Richard C Bickerton3 Address: 1Department of Otorhinolaryngology, South Warwickshire General Hospitals NHS Trust Warwick CV34 5BW UK, 2Department of Otorhinolaryngology, South Warwickshire General Hospitals NHS Trust Warwick CV34 5BW UK and 3Department of Otorhinolaryngology, South Warwickshire General Hospitals NHS Trust Warwick CV34 5BW UK Email: Daniel Thio* - [email protected]; Shahzada K Ahmed - [email protected]; Richard C Bickerton - [email protected] * Corresponding author Published: 18 September 2005 Received: 10 July 2005 Accepted: 18 September 2005 BMC Ear, Nose and Throat Disorders 2005, 5:9 doi:10.1186/1472-6815-5-9 This article is available from: http://www.biomedcentral.com/1472-6815/5/9 © 2005 Thio et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Background: Cholesteatomas are epithelial cysts that contain desquamated keratin. Patients commonly present with progressive hearing loss and a chronically discharging ear. We report an unusual presentation of the disease with an acute hearing loss suffered immediately after prolonged use of a pneumatic drill. Case presentation: A 41 year old man with no previous history of ear problems presented with a sudden loss of hearing in his right ear immediately following the prolonged use of a pneumatic drill on concrete. -
Congenital Disorders of Glycosylation from a Neurological Perspective
brain sciences Review Congenital Disorders of Glycosylation from a Neurological Perspective Justyna Paprocka 1,* , Aleksandra Jezela-Stanek 2 , Anna Tylki-Szyma´nska 3 and Stephanie Grunewald 4 1 Department of Pediatric Neurology, Faculty of Medical Science in Katowice, Medical University of Silesia, 40-752 Katowice, Poland 2 Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, 01-138 Warsaw, Poland; [email protected] 3 Department of Pediatrics, Nutrition and Metabolic Diseases, The Children’s Memorial Health Institute, W 04-730 Warsaw, Poland; [email protected] 4 NIHR Biomedical Research Center (BRC), Metabolic Unit, Great Ormond Street Hospital and Institute of Child Health, University College London, London SE1 9RT, UK; [email protected] * Correspondence: [email protected]; Tel.: +48-606-415-888 Abstract: Most plasma proteins, cell membrane proteins and other proteins are glycoproteins with sugar chains attached to the polypeptide-glycans. Glycosylation is the main element of the post- translational transformation of most human proteins. Since glycosylation processes are necessary for many different biological processes, patients present a diverse spectrum of phenotypes and severity of symptoms. The most frequently observed neurological symptoms in congenital disorders of glycosylation (CDG) are: epilepsy, intellectual disability, myopathies, neuropathies and stroke-like episodes. Epilepsy is seen in many CDG subtypes and particularly present in the case of mutations -
Polymicrogyria (PMG) ‘Many–Small–Folds’
Polymicrogyria Dr Andrew Fry Clinical Senior Lecturer in Medical Genetics Institute of Medical Genetics, Cardiff [email protected] Polymicrogyria (PMG) ‘Many–small–folds’ • PMG is heterogeneous – in aetiology and phenotype • A disorder of post-migrational cortical organisation. PMG often appears thick on MRI with blurring of the grey-white matter boundary Normal PMG On MRI PMG looks thick but the cortex is actually thin – with folded, fused gyri Courtesy of Dr Jeff Golden, Pen State Unv, Philadelphia PMG is often confused with pachygyria (lissencephaly) Thick cortex (10 – 20mm) Axial MRI 4 cortical layers Lissencephaly Polymicrogyria Cerebrum Classical lissencephaly is due Many small gyri – often to under-migration. fused together. Axial MRI image at 7T showing morphological aspects of PMG. Guerrini & Dobyns Malformations of cortical development: clinical features and genetic causes. Lancet Neurol. 2014 Jul; 13(7): 710–726. PMG - aetiology Pregnancy history • Intrauterine hypoxic/ischemic brain injury (e.g. death of twin) • Intrauterine infection (e.g. CMV, Zika virus) TORCH, CMV PCR, [+deafness & cerebral calcification] CT scan • Metabolic (e.g. Zellweger syndrome, glycine encephalopathy) VLCFA, metabolic Ix • Genetic: Family history Familial recurrence (XL, AD, AR) Chromosomal abnormalities (e.g. 1p36 del, 22q11.2 del) Syndromic (e.g. Aicardi syndrome, Kabuki syndrome) Examin - Monogenic (e.g. TUBB2B, TUBA1A, GPR56) Array ation CGH Gene test/Panel/WES/WGS A cohort of 121 PMG patients Aim: To explore the natural history of PMG and identify new genes. Recruited: • 99 unrelated patients • 22 patients from 10 families 87% White British, 53% male ~92% sporadic cases (NB. ascertainment bias) Sporadic PMG • Array CGH, single gene and gene panel testing - then a subset (n=57) had trio-WES. -
Vestibular Neuritis, Labyrinthitis, and a Few Comments Regarding Sudden Sensorineural Hearing Loss Marcello Cherchi
Vestibular neuritis, labyrinthitis, and a few comments regarding sudden sensorineural hearing loss Marcello Cherchi §1: What are these diseases, how are they related, and what is their cause? §1.1: What is vestibular neuritis? Vestibular neuritis, also called vestibular neuronitis, was originally described by Margaret Ruth Dix and Charles Skinner Hallpike in 1952 (Dix and Hallpike 1952). It is currently suspected to be an inflammatory-mediated insult (damage) to the balance-related nerve (vestibular nerve) between the ear and the brain that manifests with abrupt-onset, severe dizziness that lasts days to weeks, and occasionally recurs. Although vestibular neuritis is usually regarded as a process affecting the vestibular nerve itself, damage restricted to the vestibule (balance components of the inner ear) would manifest clinically in a similar way, and might be termed “vestibulitis,” although that term is seldom applied (Izraeli, Rachmel et al. 1989). Thus, distinguishing between “vestibular neuritis” (inflammation of the vestibular nerve) and “vestibulitis” (inflammation of the balance-related components of the inner ear) would be difficult. §1.2: What is labyrinthitis? Labyrinthitis is currently suspected to be due to an inflammatory-mediated insult (damage) to both the “hearing component” (the cochlea) and the “balance component” (the semicircular canals and otolith organs) of the inner ear (labyrinth) itself. Labyrinthitis is sometimes also termed “vertigo with sudden hearing loss” (Pogson, Taylor et al. 2016, Kim, Choi et al. 2018) – and we will discuss sudden hearing loss further in a moment. Labyrinthitis usually manifests with severe dizziness (similar to vestibular neuritis) accompanied by ear symptoms on one side (typically hearing loss and tinnitus). -
Bedside Neuro-Otological Examination and Interpretation of Commonly
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.054478 on 24 November 2004. Downloaded from BEDSIDE NEURO-OTOLOGICAL EXAMINATION AND INTERPRETATION iv32 OF COMMONLY USED INVESTIGATIONS RDavies J Neurol Neurosurg Psychiatry 2004;75(Suppl IV):iv32–iv44. doi: 10.1136/jnnp.2004.054478 he assessment of the patient with a neuro-otological problem is not a complex task if approached in a logical manner. It is best addressed by taking a comprehensive history, by a Tphysical examination that is directed towards detecting abnormalities of eye movements and abnormalities of gait, and also towards identifying any associated otological or neurological problems. This examination needs to be mindful of the factors that can compromise the value of the signs elicited, and the range of investigative techniques available. The majority of patients that present with neuro-otological symptoms do not have a space occupying lesion and the over reliance on imaging techniques is likely to miss more common conditions, such as benign paroxysmal positional vertigo (BPPV), or the failure to compensate following an acute unilateral labyrinthine event. The role of the neuro-otologist is to identify the site of the lesion, gather information that may lead to an aetiological diagnosis, and from there, to formulate a management plan. c BACKGROUND Balance is maintained through the integration at the brainstem level of information from the vestibular end organs, and the visual and proprioceptive sensory modalities. This processing takes place in the vestibular nuclei, with modulating influences from higher centres including the cerebellum, the extrapyramidal system, the cerebral cortex, and the contiguous reticular formation (fig 1). -
Sudden Bilateral Simultaneous Deafness with Vertigo As a Sole Manifestation of Vertebrobasilar Insufficiency H Lee, H a Yi, R W Baloh
539 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.4.539 on 1 April 2003. Downloaded from SHORT REPORT Sudden bilateral simultaneous deafness with vertigo as a sole manifestation of vertebrobasilar insufficiency H Lee, H A Yi, R W Baloh ............................................................................................................................. J Neurol Neurosurg Psychiatry 2003;74:539–541 CASE REPORT A 68 year old woman presented with bilateral sudden A 68 year old woman with long standing non-insulin depend- simultaneous hearing loss and transient spontaneous ent diabetes mellitus and hypertension suddenly developed vertigo as a sole manifestation of vertebrobasilar vertigo, vomiting, and bilateral tinnitus. Five minutes later, insufficiency. Extensive investigation to exclude other she noticed bilateral hearing loss. She had difficulty hearing causes was unremarkable. Magnetic resonance imaging her husband speak. She did not have dysarthria, numbness, of the brain, including diffusion images, showed no abnor- weakness, visual distortion, or incoordination. The hearing malities. A magnetic resonance angiogram showed severe loss persisted, but vertigo resolved over a few hours. Two days stenosis of the middle third of the basilar artery. A pure previously, she had had two episodes of transient vertigo and tone audiogram showed moderate sensorineural-type hearing loss involving the left ear that lasted no more than a hearing loss bilaterally. The localisation and mechanism of few minutes without any accompanying neurological symp- an isolated cochleovestibular dysfunction are discussed. toms. The patient had no previous history of hearing difficulty. There was no prior history of temporal bone fracture, menin- gitis, autoimmune disease, or exposure to ototoxic drugs. On admission, she had persistent bilateral hearing loss and ertebrobasilar insufficiency (VBI) can cause sudden non-specific dizziness with lightheadedness.