Computed Tomography Petrous Bone Otosclerosis
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Direct Sagittal CT in the Evaluation of Temporal Bone Disease
371 Direct Sagittal CT in the Evaluation of Temporal Bone Disease 1 Mahmood F. Mafee The human temporal bone is an extremely complex structure. Direct axial and coronal Arvind Kumar2 CT sections are quite satisfactory for imaging the anatomy of the temporal bone; Christina N. Tahmoressi1 however, many relationships of the normal and pathologic anatomic detail of the Barry C. Levin2 temporal bone are better seen with direct sagittal CT sections. The sagittal projection Charles F. James1 is of interest to surgeons, as it has the advantage of following the plane of surgical approach. This article describes the advantages of using direct sagittal sections for Robert Kriz 1 1 studying various diseases of the temporal bone. The CT sections were obtained with Vlastimil Capek the aid of a new headholder added to our GE CT 9800 scanner. The direct sagittal projection was found to be extremely useful for evaluating diseases involving the vertical segment of the facial nerve canal, vestibular aqueduct, tegmen tympani, sigmoid sinus plate, sinodural angle, carotid canal, jugular fossa, external auditory canal, middle ear cavity, infra- and supra labyrinthine air cells, and temporo mandibular joint. CT has contributed greatly to an understanding of the complex anatomy and spatial relationship of the minute structures of the hearing and balance organs, which are packed into a small pyramid-shaped petrous temporal bone [1 , 2]. In the past 6 years, high-resolution CT scanning has been rapidly replacing standard tomography and has proved to be the diagnostic imaging method of choice for studying the normal and pathologic details of the temporal bone [3-14]. -
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. -
Bachmann.Pdf
Pediatric Vestibular Dysfunction: More Than a Balancing Act Kay Bachmann, PhD 1 2 Introduction Childhood Hearing Loss 1. The learner will be able to identify 3 causes of vestibular • 1.4 in 1,000 newborns have hearing loss (CDC) disorders in children. • 5 in 1,000 children have hearing loss ages 3-17 yrs (CDC) 2. The learner will be able to identify at least 3 common symptoms of vestibular dysfunction in children. 3. The learner will be able to administer a short screening to assess balance function in children. 3 4 1 Childhood Vestibular Loss • Balance disorders may make up .45% of chief complaints per chart review in a Pediatric ENT department. (O’Reilly et al 2010) • Children with hearing impairment are twice as likely to have vestibular loss than healthy children • Studies show vestibular loss in 30-79% of children with HL • There is 10% increase in vestibular loss as a result of trauma from receiving a CI (Jacot et al, 2009) 5 6 Common Disorders with Hearing Loss and Vestibular Abnormalities • Syndromes What Does a Typically Functioning Vestibular System Do? – Usher Syndrome Type 1 – Pendred Syndrome (also non syndromic Enlarged vestibular Aqueduct Syndrome) Two Main Reflexes: – Branchio-oto-renal Syndrome Vestibular Ocular Reflex- Helps hold an object – CHARGE association steady when the head/body are in motion. • Cochlear Malformations • VIII Nerve Defects • Cytomegalovirus (CMV) Vestibular Spinal Reflex- Helps keep our posture • Meningitis • Cochlear implant patients and body steady when it senses movement. 7 8 2 What Does an -
Etiologies and Characteristics of Deaf-Blindness
Etiologies and Characteristics of Deaf-Blindness Kathryn Wolff Heller, R.N., Ph.D. Geor gia State Uni ver sity Cheryl Ken nedy Uni ver sity of Pitts burgh Edited by: Louis Cooper , M.D. Law rence T. Eschelman, M.D., P.C. James W. Long, M.D., P.C. Rosanne K. Silberman, Ed., D. Contents Pref ace··························3 Sec tion I Foun da tions ······················5 Chap ter 1 Def i ni tions and Ter mi nol ogy Per tain ing to Deaf-Blind ness ···················6 Chap ter 2 Nor mal Anatomy of the Eye and Ear ········16 Chap ter 3 Common Disor ders of the Eye and Ear ·······22 Sec tion II Causes of Deaf-Blind ness ···············36 Chap ter 4 He red i tary Syn dromes and Dis or ders········37 Chap ter 5 Con gen i tal In fec tions and Teratogens ········50 Chap ter 6 Prematurity and Small for Gesta tional Age·····65 Chap ter 7 Ad ven ti tious Con di tions ···············68 APPENDIX Ref er ences························75 Pref ace here are sev eral dis or ders, syn dromes, in fec tious dise ases, and ad ven ti tious con - di tions that may re sult in an in di vid ual be ing deaf-blind. As a re sult of these var i - Tous eti ol o gies, an in di vid ual who is deaf-blind may ex hibit a range of vi sion and hear ing losses. Sen sory loss may range from mild im pair ment to to tal loss of the abil ity to see and/or hear. -
CONGENITAL MALFORMATIONS of the INNER EAR Malformaciones Congénitas Del Oído Interno
topic review CONGENITAL MALFORMATIONS OF THE INNER EAR Malformaciones congénitas del oído interno. Revisión de tema Laura Vanessa Ramírez Pedroza1 Hernán Darío Cano Riaño2 Federico Guillermo Lubinus Badillo2 Summary Key words (MeSH) There are a great variety of congenital malformations that can affect the inner ear, Ear with a diversity of physiopathologies, involved altered structures and age of symptom Ear, inner onset. Therefore, it is important to know and identify these alterations opportunely Hearing loss Vestibule, labyrinth to lower the risks of all the complications, being of great importance, among others, Cochlea the alterations in language development and social interactions. Magnetic resonance imaging Resumen Existe una gran variedad de malformaciones congénitas que pueden afectar al Palabras clave (DeCS) oído interno, con distintas fisiopatologías, diferentes estructuras alteradas y edad Oído de aparición de los síntomas. Por lo anterior, es necesario conocer e identificar Oído interno dichas alteraciones, con el fin de actuar oportunamente y reducir el riesgo de las Pérdida auditiva Vestíbulo del laberinto complicaciones, entre otras —de gran importancia— las alteraciones en el área del Cóclea lenguaje y en el ámbito social. Imagen por resonancia magnética 1. Epidemiology • Hyperbilirubinemia Ear malformations occur in 1 in 10,000 or 20,000 • Respiratory distress from meconium aspiration cases (1). One in every 1,000 children has some degree • Craniofacial alterations (3) of sensorineural hearing impairment, with an average • Mechanical ventilation for more than five days age at diagnosis of 4.9 years. The prevalence of hearing • TORCH Syndrome (4) impairment in newborns with risk factors has been determined to be 9.52% (2). -
Genetic Determinants of Non-Syndromic Enlarged Vestibular Aqueduct: a Review
Review Genetic Determinants of Non-Syndromic Enlarged Vestibular Aqueduct: A Review Sebastian Roesch 1 , Gerd Rasp 1, Antonio Sarikas 2 and Silvia Dossena 2,* 1 Department of Otorhinolaryngology, Head and Neck Surgery, Paracelsus Medical University, 5020 Salzburg, Austria; [email protected] (S.R.); [email protected] (G.R.) 2 Institute of Pharmacology and Toxicology, Paracelsus Medical University, 5020 Salzburg, Austria; [email protected] * Correspondence: [email protected]; Tel.: +43-(0)662-2420-80564 Abstract: Hearing loss is the most common sensorial deficit in humans and one of the most common birth defects. In developed countries, at least 60% of cases of hearing loss are of genetic origin and may arise from pathogenic sequence alterations in one of more than 300 genes known to be involved in the hearing function. Hearing loss of genetic origin is frequently associated with inner ear malformations; of these, the most commonly detected is the enlarged vestibular aqueduct (EVA). EVA may be associated to other cochleovestibular malformations, such as cochlear incomplete partitions, and can be found in syndromic as well as non-syndromic forms of hearing loss. Genes that have been linked to non-syndromic EVA are SLC26A4, GJB2, FOXI1, KCNJ10, and POU3F4. SLC26A4 and FOXI1 are also involved in determining syndromic forms of hearing loss with EVA, which are Pendred syndrome and distal renal tubular acidosis with deafness, respectively. In Caucasian cohorts, approximately 50% of cases of non-syndromic EVA are linked to SLC26A4 and a large fraction of Citation: Roesch, S.; Rasp, G.; patients remain undiagnosed, thus providing a strong imperative to further explore the etiology of Sarikas, A.; Dossena, S. -
ANATOMY of EAR Basic Ear Anatomy
ANATOMY OF EAR Basic Ear Anatomy • Expected outcomes • To understand the hearing mechanism • To be able to identify the structures of the ear Development of Ear 1. Pinna develops from 1st & 2nd Branchial arch (Hillocks of His). Starts at 6 Weeks & is complete by 20 weeks. 2. E.A.M. develops from dorsal end of 1st branchial arch starting at 6-8 weeks and is complete by 28 weeks. 3. Middle Ear development —Malleus & Incus develop between 6-8 weeks from 1st & 2nd branchial arch. Branchial arches & Development of Ear Dev. contd---- • T.M at 28 weeks from all 3 germinal layers . • Foot plate of stapes develops from otic capsule b/w 6- 8 weeks. • Inner ear develops from otic capsule starting at 5 weeks & is complete by 25 weeks. • Development of external/middle/inner ear is independent of each other. Development of ear External Ear • It consists of - Pinna and External auditory meatus. Pinna • It is made up of fibro elastic cartilage covered by skin and connected to the surrounding parts by ligaments and muscles. • Various landmarks on the pinna are helix, antihelix, lobule, tragus, concha, scaphoid fossa and triangular fossa • Pinna has two surfaces i.e. medial or cranial surface and a lateral surface . • Cymba concha lies between crus helix and crus antihelix. It is an important landmark for mastoid antrum. Anatomy of external ear • Landmarks of pinna Anatomy of external ear • Bat-Ear is the most common congenital anomaly of pinna in which antihelix has not developed and excessive conchal cartilage is present. • Corrections of Pinna defects are done at 6 years of age. -
The Temporal Bone
Anatomic Moment The Temporal Bone Joel D. Swartz, David L. Daniels, H. Ric Harnsberger, Katherine A. Shaffer, and Leighton Mark Despite the advent of thin-section high-reso- the inner ear structures and the external lution computed tomography and, more re- environment. cently, unique magnetic resonance imaging se- Virtually all textbooks define the inner ear as quences with thin sections, T2 weighting, and a membranous labyrinth housed within an os- maximum-intensity projection techniques, seous labyrinth. The membranous labyrinth three-dimensional neuroanatomy of the tempo- contains endolymph, a fluid rich in potassium ral bone and related structures remains some- and low in sodium, similar to intracellular fluid. what of an enigma to many medical specialists, Interposed between the membranous labyrinth neuroradiologists included. Therefore, we are and the osseous labyrinth resides a supportive undertaking a series of anatomic moments in perilymphatic labyrinth. Perilymph is similar to the hope of solidifying the most important ana- cerebrospinal fluid and other extracellular tissue tomic concepts as they relate to this region. Our fluid. approach will be organized so as to consider the The osseous labyrinth consists of the bony temporal bone to represent the complex inter- edifice for the vestibule, semicircular canals, relationship of three systems: hearing, balance, cochlea, and vestibular aqueduct. The mem- and related neuroanatomic and neurovascular branous labyrinth consists of the utricle and structures. saccule (located within the vestibule), the semi- The ear originated in fish as a water motion circular ducts, the cochlear duct, and the en- detection system (1). The vestibular (balance) dolymphatic duct. The latter is a channel within mechanism becomes more complex as we the vestibular aqueduct with communications to scale the embryologic ladder and endolymph the utricle and saccule. -
We Hear with Our Brain As the Same As Our Ears
Scholarly Journal of Otolaryngology DOI: 10.32474/SJO.2019.02.000133 ISSN: 2641-1709 Opinion We Hear With our Brain as the same as our Ears Alireza Bina* Starwood Audiology, USA *Corresponding author: Alireza Bina, Starwood Audiology, USA, Email: Received: May 13, 2019 Published: May 21, 2019 Abstract There are some studies which confirmed that dysfunction in Central Nervous System (CNS) may cause a malfunction in the Peripheral Auditory system (Cochlea_ Auditory Nerve, Auditory Neuropathy), but the question is could Brain Disorder without any lesion in Cochlea and/or Auditory nerve cause Sensorineural Hearing Loss? It seems that there are a lot of Sensorineural hearing loss which they have neither Sensory nor Neural lesion, Brain is involved causing them. We deal with this subject in this paper and we propose a new theory that External Ear Canal is not the only input of Auditory Signals, Sounds could receive by the head and Cerebral Cortex and approach to the Cochlea (Backward Auditory input of Sounds). Discussion because of correlation between them this occurred, so not only There are some studies that verified Otosclerosis and Meniere disorder in Central Auditory system may cause peripheral hearing Diseases which are Peripheral pathologies initiated from CNS [1,2]. loss malfunction in other parts of Brain may involve in causing Increasing Cortisol Level and lack of Dopamine in Hypothalamus hearing loss [3]. Neurofibromatosis type II is a genetic condition is involved in Meniere Disease and in Otosclerosis infection of CNS which may -
Michigan Ear Institute Otosclerosis
Michigan Ear Institute Michigan Otosclerosis www.michiganear.com 34015-56111-109 BOOK Otosclerosis.indd 1 2/13/18 10:33 AM Dennis I. Bojrab, MD Seilesh C. Babu, MD John J. Zappia, MD, FACS Eric W. Sargent, MD, FACS DOCTORS Eleanor Y. Chan, MD Robert S. Hong, MD Ilka C. Naumann, MD Candice C. Colby, MD Christopher A. Schutt, MD Providence Medical Building 30055 Northwestern Highway Suite 101 Farmington Hills, MI 48334 Beaumont Medical Building LOCATIONS 3555 W. Thirteen Mile Road Suite N-210 Royal Oak, MI 48073 Oakwood Medical Building 18181 Oakwood Blvd. Suite 402 Dearborn, MI 48126 Providence Medical Center 26850 Providence Parkway Suite 130 Novi, MI 48374 248-865-4444 phone 248-865-6161 fax 1 34015-56111-109 BOOK Otosclerosis.indd 1 2/13/18 10:33 AM WELCOME Welcome to the Michigan Ear Institute, one of the nation’s leading surgical groups specializing in hearing, balance and facial nerve disorders. The Michigan Ear Institute is committed to providing you with the highest quality diagnostic and surgical treatment possible. Our highly experienced team of physicians, audiologists and clinical physiologists have established international reputations for their innovative diagnostic and surgical capabilities, and our modern, attractive facility has been designed with patient care and convenience as the foremost criteria. It is our privilege to be able to provide care for your medical problems and we will strive to make your visit to the Michigan Ear Institute a positive and rewarding experience. 3 34015-56111-109 BOOK Otosclerosis.indd 3 2/13/18 10:33 AM OTOSCLEROSIS Otosclerosis is a disease of the middle ear bones and sometimes the inner ear. -
Let's Talk About . . . Otosclerosis
LET’S TALK ABOUT . OTOSCLEROSIS diagnosed with otosclerosis. Pregnancy can cause Key points otosclerosis to advance more quickly. • Otosclerosis affects the bones of the middle Otosclerosis is rare, affecting about 3 in 1,000 ear that conduct sound. people. Research suggests between 25 to 50% of people with otosclerosis have a family history of the • It is one of the most common causes of conductive hearing loss in young adults. condition. • How quickly, or to what extent, hearing will The word otosclerosis comes from Greek. It means be affected is unpredictable. abnormal hardening of body tissue (sclerosis) of the ear (oto). • If otosclerosis goes into the inner ear, you may be troubled by ringing in the ears, dizziness and balance problems. How do we hear? • Hearing aids are usually the preferred first treatment choice. To understand why otosclerosis causes hearing loss, it is important to have a basic understanding of how we hear. For hearing to function normally a What is otosclerosis? sound has to travel through all three parts of the Otosclerosis (oh-toe-skler-OH-suhs) a complex ear: outer, middle and inner. The first two are air disorder of abnormal bone growth in the middle ear. filled; the latter is fluid filled. It most often happens when the tiny stapes (“STAY- The outer ear is made up of the part you can see peez”) bone knits with surrounding bone. on the side of your head (pinna) and the funnel- Otosclerosis usually results in slow, shaped external ear canal. The pinna gathers progressive conductive hearing loss. sound waves (vibrations) and channels them When the stapes is unable to vibrate, hearing through the ear canal to the eardrum (tympanic becomes impaired. -
Otosclerosis : Deafness Amendable to Surgery
University of Nebraska Medical Center DigitalCommons@UNMC MD Theses Special Collections 5-1-1964 Otosclerosis : deafness amendable to surgery Charles E. Evans University of Nebraska Medical Center This manuscript is historical in nature and may not reflect current medical research and practice. Search PubMed for current research. Follow this and additional works at: https://digitalcommons.unmc.edu/mdtheses Part of the Medical Education Commons Recommended Citation Evans, Charles E., "Otosclerosis : deafness amendable to surgery" (1964). MD Theses. 12. https://digitalcommons.unmc.edu/mdtheses/12 This Thesis is brought to you for free and open access by the Special Collections at DigitalCommons@UNMC. It has been accepted for inclusion in MD Theses by an authorized administrator of DigitalCommons@UNMC. For more information, please contact [email protected]. Charles Edward Evans Submitted in Partial Fulfillment for the Degree of Doctor of Medicine College of Medicine, University of Nebraska February 1, 1964 Omaha, Nebraska Page I. Introductioll 1 II. Classification of Deafness 3 III. Pathology 5-11 Phas~s of Development Histopathologically 5 General Histopathologic Picture 7 Effects" of Stapes Illvolvement 9 IV. Etiology 12-16 V. Diagnos.iB 17-29 Incidence 17 Diagnostic Tests 20 Signs and Symptoms 22 Sensorineural loss 25 Effects of Pregnancy 26 Vi. Surgery 30-39 Early development 30 Fenestration 32 Stapes Surgery 36 Complications 38 VII. Summary and Comclusions 40-42 VIII. Bibliogra.phy 43-46 INTRODUCTION Throughout time many people have been forced into a world of silence by a disease known as otosclerosis. Deafness generally as a handicap is not apprecLlted by the "normal" population to the same extent that other handicaps are.