The Membranous Labyrinth in Vivo from High-Resolution Temporal CT Data
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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). -
Cochlear Partition Anatomy and Motion in Humans Differ from The
Cochlear partition anatomy and motion in humans differ from the classic view of mammals Stefan Raufera,b,1, John J. Guinan Jra,b,c, and Hideko Heidi Nakajimaa,b,c aEaton-Peabody Laboratories, Massachusetts Eye and Ear, Boston, MA 02114; bSpeech and Hearing Bioscience and Technology Program, Harvard University, Cambridge, MA 02138; and cDepartment of Otolaryngology, Harvard Medical School, Boston, MA 02115 Edited by Christopher A. Shera, University of Southern California, Los Angeles, CA, and accepted by Editorial Board Member Thomas D. Albright June 6, 2019 (received for review January 16, 2019) Mammals detect sound through mechanosensitive cells of the assume there is no OSL motion (10–13). Additionally, the attach- cochlear organ of Corti that rest on the basilar membrane (BM). ment of the OSL to the BM and the attachment of the TM to Motions of the BM and organ of Corti have been studied at the spiral limbus (which sits above the OSL) are also consid- the cochlear base in various laboratory animals, and the assump- ered stationary. In contrast to this view, there have been reports tion has been that the cochleas of all mammals work similarly. of sound-induced OSL movement, but these reports have been In the classic view, the BM attaches to a stationary osseous spi- largely ignored in overviews of cochlear mechanics and the for- ral lamina (OSL), the tectorial membrane (TM) attaches to the mation of cochlear models (10–13). Von B´ek´esy (14), using static limbus above the stationary OSL, and the BM is the major mov- pressure, found that the CP “bent like an elastic rod that was free ing element, with a peak displacement near its center. -
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. -
Balance and Equilibrium, I: the Vestibule and Semicircular Canals
Anatomic Moment Balance and Equilibrium, I: The Vestibule and Semicircular Canals Joel D. Swartz, David L. Daniels, H. Ric Harnsberger, Katherine A. Shaffer, and Leighton Mark In this, our second temporal bone installment, The endolymphatic duct arises from the en- we will emphasize the vestibular portion of the dolymphatic sinus and passes through the ves- labyrinth, that relating to balance and equilib- tibular aqueduct of the osseous labyrinth to rium. Before proceeding, we must again remind emerge from an aperture along the posterior the reader of the basic structure of the labyrinth: surface of the petrous pyramid as the endolym- an inner membranous labyrinth (endolym- phatic sac. phatic) surrounded by an outer osseous laby- The utricle and saccule are together referred rinth with an interposed supportive perilym- to as the static labyrinth, because their function phatic labyrinth. We recommend perusal of the is to detect the position of the head relative to first installment before continuing if there are gravity (5–7). They each have a focal concen- any uncertainties in this regard. tration of sensory receptors (maculae) located The vestibule, the largest labyrinthine cavity, at right angles to each other and consisting of measures 4 to 6 mm maximal diameter (1–3) ciliated hair cells and tiny crystals of calcium (Figs 1–3). The medial wall of the vestibule is carbonate (otoliths) embedded in a gelatinous unique in that it contains two distinct depres- mass. These otoliths respond to gravitational sions (Fig 4). Posterosuperiorly lies the elliptical pull; therefore, changes in head position distort recess, where the utricle is anchored. -
Characteristic Anatomical Structures of Rat Temporal Bone
HOSTED BY Available online at www.sciencedirect.com ScienceDirect Journal of Otology 10 (2015) 118e124 www.journals.elsevier.com/journal-of-otology/ Characteristic anatomical structures of rat temporal bone Peng Li a,b, Kelei Gao b,c, Dalian Ding a,b,c,*, Richard Salvi b,c a Department of Otolaryngology, Head and Neck Surgery, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510630, China b Center for Hearing and Deafness, State University of New York at Buffalo, Buffalo, NY 14214, USA c Department of Otolaryngology, Head and Neck Surgery, Xiangya Hospital, Central South University, Hunan 410013, China Abstract As most gene sequences and functional structures of internal organs in rats have been well studied, rat models are widely used in experi- mental medical studies. A large number of descriptions and atlas of the rat temporal bone have been published, but some detailed anatomy of its surface and inside structures remains to be studied. By focusing on some unique characteristics of the rat temporal bone, the current paper aims to provide more accurate and detailed information on rat temporal bone anatomy in an attempt to complete missing or unclear areas in the existed knowledge. We also hope this paper can lay a solid foundation for experimental rat temporal bone surgeries, and promote information exchange among colleagues, as well as providing useful guidance for novice researchers in the field of hearing research involving rats. Copyright © 2015 The Authors. Production & hosting by Elsevier (Singapore) Pte Ltd On behalf of PLA General Hospital Department of Otolaryngology Head and Neck Surgery. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/ by-nc-nd/4.0/). -
Physiology of the Inner Ear Balance
§ Te xt § Important Lecture § Formulas No.15 § Numbers § Doctor notes “Life Is Like Riding A § Notes and explanation Bicycle. To Keep Your Balance, You Must Keep Moving” 1 Physiology of the inner ear balance Objectives: 1. Understand the sensory apparatus of the inner ear that helps the body maintain its postural equilibrium. 2. The mechanism of the vestibular system for coordinating the position of the head and the movement of the eyes. 3. The function of semicircular canals (rotational movements, angular acceleration). 4. The function of the utricle and saccule within the vestibule (respond to changes in the position of the head with respect to gravity (linear acceleration). 5. The connection between the vestibular system and other structure (eye, cerebellum, brain stem). 2 Control of equilibrium } Equilibrium: Reflexes maintain body position at rest & movement through receptors of postural reflexes: 1. Proprioceptive system (Cutaneous sensations). 2. Visual (retinal) system. 3. Vestibular system (Non auditory membranous labyrinth1). 4. Cutaneous sensation. } Cooperating with vestibular system wich is present in the semicircular canals in the inner ear. 3 1: the explanation in the next slide. • Ampulla or crista ampullaris: are the dilations at the end of the semicircular canals and they affect the balance. • The dilations connect the semicircular canals to the cochlea utricle Labyrinth and saccule: contain the vestibular apparatus (maculla). Bony labyrinth • bony cochlea, vestibule & 3 bony semicircular canals. • Enclose the membranous labyrinth. Labyrinth a. Auditory (cochlea for hearing). b. Non-auditory for equilibrium (Vestibular apparatus). composed of two parts: • Vestibule: (Utricle and Saccule). • Semicircular canals “SCC”. Membranous labyrinth • Membranous labyrinth has sensory receptors for hearing and equilibrium • Vestibular apparatus is responsible for equilibrium 4 Macula (otolith organs) of utricle and saccule } Hair cell synapse with endings of the vestibular nerve. -
Mmubn000001 20756843X.Pdf
PDF hosted at the Radboud Repository of the Radboud University Nijmegen The following full text is a publisher's version. For additional information about this publication click this link. http://hdl.handle.net/2066/147581 Please be advised that this information was generated on 2021-10-07 and may be subject to change. CATION TRANSPORT AND COCHLEAR FUNCTION CATION TRANSPORT AND COCHLEAR FUNCTION PROMOTORES: Prof. Dr. S. L. BONTING EN Prof. Dr. W. F. B. BRINKMAN CATION TRANSPORT AND COCHLEAR FUNCTION PROEFSCHRIFT TER VERKRIJGING VAN DE GRAAD VAN DOCTOR IN DE WISKUNDE EN NATUURWETENSCHAPPEN AAN DE KATHOLIEKE UNIVERSITEIT TE NIJMEGEN, OP GEZAG VAN DE RECTOR MAGNIFICUS DR. G. BRENNINKMEIJER, HOOGLERAAR IN DE FACULTEIT DER SOCIALE WETENSCHAPPEN, VOLGENS BESLUIT VAN DE SENAAT IN HET OPENBAAR TE VERDEDIGEN OP VRIJDAG 19 DECEMBER 1969 DES NAMIDDAGS TE 2 UUR DOOR WILLIBRORDUS KUIJPERS GEBOREN TE KLOOSTERZANDE 1969 CENTRALE DRUKKERIJ NIJMEGEN I am greatly indebted to Dr. J. F. G. Siegers for his interest and many valuable discussions throughout the course of this investigation. The technical assistance of Miss A. C. H. Janssen, Mr. A. C. van der Vleuten and Mr. P. Spaan and co-workers was greatly appreciated. I also wish to express my gratitude to Miss. A. E. Gonsalvcs and Miss. G. Kuijpers for typing and to Mrs. M. Duncan for correcting the ma nuscript. The diagrams were prepared by Mr W. Maas and Mr. C. Reckers and the micro- photographs by Mr. A. Reijnen of the department of medical illustration. Aan mijn Ouders, l Thea, Annemarie, Katrien en Michiel. CONTENTS GENERAL INTRODUCTION ... -
The Nervous System: General and Special Senses
18 The Nervous System: General and Special Senses PowerPoint® Lecture Presentations prepared by Steven Bassett Southeast Community College Lincoln, Nebraska © 2012 Pearson Education, Inc. Introduction • Sensory information arrives at the CNS • Information is “picked up” by sensory receptors • Sensory receptors are the interface between the nervous system and the internal and external environment • General senses • Refers to temperature, pain, touch, pressure, vibration, and proprioception • Special senses • Refers to smell, taste, balance, hearing, and vision © 2012 Pearson Education, Inc. Receptors • Receptors and Receptive Fields • Free nerve endings are the simplest receptors • These respond to a variety of stimuli • Receptors of the retina (for example) are very specific and only respond to light • Receptive fields • Large receptive fields have receptors spread far apart, which makes it difficult to localize a stimulus • Small receptive fields have receptors close together, which makes it easy to localize a stimulus. © 2012 Pearson Education, Inc. Figure 18.1 Receptors and Receptive Fields Receptive Receptive field 1 field 2 Receptive fields © 2012 Pearson Education, Inc. Receptors • Interpretation of Sensory Information • Information is relayed from the receptor to a specific neuron in the CNS • The connection between a receptor and a neuron is called a labeled line • Each labeled line transmits its own specific sensation © 2012 Pearson Education, Inc. Interpretation of Sensory Information • Classification of Receptors • Tonic receptors -
Inner Ear Defects and Hearing Loss in Mice Lacking the Collagen Receptor
Laboratory Investigation (2008) 88, 27–37 & 2008 USCAP, Inc All rights reserved 0023-6837/08 $30.00 Inner ear defects and hearing loss in mice lacking the collagen receptor DDR1 Angela M Meyer zum Gottesberge1, Oliver Gross2, Ursula Becker-Lendzian1, Thomas Massing1 and Wolfgang F Vogel3 Discoidin domain receptor 1 (DDR1) is a tyrosine kinase receptor that is activated by native collagen. The physiological functions of DDR1 include matrix homeostasis and cell growth, adhesion, branching, and migration, but the specific role of DDR1 in the development and function of the inner ear has not been analyzed. Here, we show that deletion of the DDR1 gene in mouse is associated with a severe decrease in auditory function and substantial structural alterations in the inner ear. Immunohistochemical analysis demonstrated DDR1 expression in several locations in the cochlea, mostly associated with basement membrane and fibrillar collagens; in particular in basal cells of the stria vascularis, type III fibrocytes, and cells lining the basilar membrane of the organ of Corti. In the stria vascularis, loss of DDR1 function resulted in altered morphology of the basal cells and accumulation of electron-dense matrix within the strial epithelial layer in conjunction with a focal and progressive deterioration of strial cells. Cell types in proximity to the basilar membrane, such as Claudius’, inner and outer sulcus cells, also showed marked ultrastructural alterations. Changes in the organ of Corti, such as deterioration of the supporting cells, specifically the outer hair cells, Deiters’, Hensen’s and bordering cells, are likely to interfere with mechanical properties of the organ and may be responsible for the hearing loss observed in DDR1-null mice. -
Otic Capsule Or Bony Labyrinth
DEVELOPMENT OF EAR BY DR NOMAN ULLAH WAZIR DEVELOPMENT OF EAR The ears are composed of three anatomic parts: External ear: • Consisting of the auricle , external acoustic meatus, and the external layer of the tympanic membrane. Middle ear: • The internal layer of the tympanic membrane, and three small auditory ossicles, which are connected to the oval windowsof the internal ear. • Internal ear: Consisting of the vestibulocochlear organ, which is concerned with hearing and balance. • The external and middle parts of the ears are concerned with the transference of sound waves to the internal ears, which convert the waves into nerve impulses and registers changes in equilibrium. DEVELOPMENT OF INTERNALEAR The internal ears are the first to develop. • Otic placode: Early in the 4th week, a thickening of surface ectoderm takes place on each side of the myelencephalon,the caudal part of thehindbrain. • Inductive signals from the paraxial mesoderm and notochord stimulate the surface ectoderm to form theplacodes. • Each otic placode soon invaginates and sinks deep to the surface ectoderm into the underlying mesenchyme. • In so doing, it forms an otic pit. • The edges of the pit come together and fuse to forman otic vesicle the primordium of the membranous labyrinth. • The otic vesicle soon loses its connection with the surface ectoderm. • A diverticulum (endolymohatic appendage) grows from the vesicle and elongates to form the endolymphatic duct and sac. the rest of the oticvesicle differentiates into an expanded pars superior (Ventral saccularparts, which give rise to the sacculeand cochlearducts) and an initially tapered pars inferior (Dorsal utricular parts, from which thesmall endolymphaticducts, utricles and semicircular ductsarise). -
Microstructures of the Osseous Spiral Laminae in the Bat Cochlea: a Scanning Electron Microscopic Study
Arch. Histol. Cytol., Vol. 55, No. 3 (1992) p. 315-319 Microstructures of the Osseous Spiral Laminae in the Bat Cochlea: A Scanning Electron Microscopic Study Babi r KUcUK2 and Kazuhiro ABE1 Department of Anatomy, Hokkaido University School of Medicine, Sapporo, Hokkaido; and Department of Otolaryngology2, Tokai University School of Medicine, Isehara, Kanagawa, Japan Received May 22, 1992 Summary. The architecture and surface structures of dary osseous spiral laminae. High frequency sounds the primary and secondary osseous spiral laminae in the vibrate the membrane in the basal regions of the cochlea of the bat, an animal able to hear high fre- cochlear duct, while relatively lower frequency quency sounds, were examined by scanning electron sounds vibrate the membrane in the apical regions microscopy to understand the micromechanical adapta- (BEKESY, 1960) . Using the mouse cochlea, we have tions of the bony supportive elements in the inner ear to suggested that the regional vibration pattern of the the specific hearing function. The bat used was Myotis frater kaguyae. basilar membrane is closely related to the base-to- The myotis bat cochlea was seen to consist of a hook apex variations in the morphology of the osseous and a spiral portion with one and three-quarter turns spiral laminae (KUcUK and ABE, 1989). It is known and was characterized by: 1) a distinct ridge-like pro- that the bat cochlea is sensitive to sounds in the very jection running spirally along the middle line on the high frequency range. In fact, the frequencies of the vestibular leaf of the primary osseous spiral lamina; 2) sounds that stimulate the basilar membrane in the a wide secondary osseous spiral lamina; and 3) a narrow bat cochlea are higher than those that stimulate the spiral fissure between the primary and secondary osse- basal regions of the basilar membrane in the mouse ous spiral laminae. -
Otoancorin, an Inner Ear Protein Restricted To
Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22 Ingrid Zwaenepoel*, Mirna Mustapha*, Michel Leibovici*, Elisabeth Verpy*, Richard Goodyear†, Xue Zhong Liu‡, Sylvie Nouaille*, Walter E. Nance§, Moien Kanaan¶, Karen B. Avrahamʈ, Fredj Tekaia**, Jacques Loiselet††, Marc Lathrop‡‡, Guy Richardson†, and Christine Petit*¶¶ *Unite´deGe´ne´ tique des De´ficits Sensoriels, Centre National de la Recherche Scientifique, Unite´de Recherche Associe´e 1968, and **Unite´deGe´ne´ tique Mole´culaire des Levures, Centre National de la Recherche Scientifique, Unite´de Recherche Associe´e 2171, Institut Pasteur, 25 rue du Dr. Roux, 75724 Paris Cedex 15, France; †School of Biological Sciences, The University of Sussex, Falmer, Brighton, BN1 9QG, United Kingdom; ‡Department of Otolaryngology, University of Miami, Miami, FL 33101; §Department of Human Genetics, Virginia Commonwealth University, 1101 East Marshall Street, Richmond, VA 23219; ¶Molecular Genetics, Life Science Department, Bethlehem University POB 9, Palestinian Authority; ʈDepartment of Human Genetics and Molecular Medicine, Tel Aviv University, Tel Aviv 69978, Israel; ††Laboratoire de Biologie Mole´culaire, Faculte´deMe´ decine, Universite´Saint-Joseph, Beyrouth, Lebanon; and ‡‡Centre National de Ge´notypage, 91057 Evry, France Edited by Thaddeus P. Dryja, Harvard Medical School, Boston, MA, and approved February 21, 2002 (received for review October 1, 2001) A 3,673-bp murine cDNA predicted to encode a glycosylphosphati- collagenase-insensitive striated-sheet matrix (6). Otogelin is asso- dylinositol-anchored protein of 1,088 amino acids was isolated ciated mainly with the collagen fibril bundles (3, 7), whereas ␣- and during a study aimed at identifying transcripts specifically ex- -tectorin are major components of the striated-sheet matrix (8, 9).