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Endolymphatic Sac Tumors: an Overview Michael B
Endolymphatic Sac Tumors: An Overview Michael B. Gluth, MD, FACS Associate Professor Director, Comprehensive Ear & Hearing Ctr. Section of Otolaryngology-Head & Neck Surgery VHL Alliance, Chicago, 2015 Disclosure • No relevant financial interests or other relevant relationships to disclose • Will discuss off-label use of cochlear implants for single-sided SNHL VHL | 2 Outline • What is an endolymphatic sac tumor? • What are the symptoms? • What is the work-up and treatment? VHL | 3 Endolymphatic Sac Anatomy VHL | 4 Endolymphatic Sac Function • Part of the “membranous labyrinth” • Filled with fluid called endolymph • Secretes locally acting chemical called “saccin” • Involved with inner ear fluid homeostasis, mechanisms not fully understood – ELS is involved with Meniere’s disease VHL | 5 ELS Tumors • Extremely rare tumor originating from the endolymphatic sac, only recognized as a unique entity since 1989 • Benign (not cancer) • Highly destructive, slowly progressive – Destroy bone of inner ear, around cranial vault/skull base, and around facial nerve – Can grow into nerves, pass through dura (sac around brain) and press on cerebellum • May be asymptomatic until inner ear is partially destroyed • Key: if hearing loss is present: high chance dura is invaded VHL | 6 Symptoms • Most common presenting symptoms: – Hearing loss (85%) – Visible mass in the ear (50%) – Ringing in the ear (48%) – Facial paralysis (44%) – Dizziness/imbalance (44%) – Headache (37%) – Other neurologic (cranial nerve) weakness (25%) • If present in both ears, outlook -
Morphological and Functional Changes in a New Animal Model Of
Laboratory Investigation (2013) 93, 1001–1011 & 2013 USCAP, Inc All rights reserved 0023-6837/13 Morphological and functional changes in a new animal model of Me´nie`re’s disease Naoya Egami1, Akinobu Kakigi1, Takashi Sakamoto1, Taizo Takeda2, Masamitsu Hyodo2 and Tatsuya Yamasoba1 The purpose of this study was to clarify the underlying mechanism of vertiginous attacks in Me´nie`re’s disease (MD) while obtaining insight into water homeostasis in the inner ear using a new animal model. We conducted both histopatho- logical and functional assessment of the vestibular system in the guinea-pig. In the first experiment, all animals were maintained 1 or 4 weeks after electrocauterization of the endolymphatic sac of the left ear and were given either saline or desmopressin (vasopressin type 2 receptor agonist). The temporal bones from both ears were harvested and the extent of endolymphatic hydrops was quantitatively assessed. In the second experiment, either 1 or 4 weeks after surgery, animals were assessed for balance disorders and nystagmus after the administration of saline or desmopressin. In the first experiment, the proportion of endolymphatic space in the cochlea and the saccule was significantly greater in ears that survived for 4 weeks after surgery and were given desmopressin compared with other groups. In the second experiment, all animals that underwent surgery and were given desmopressin showed spontaneous nystagmus and balance disorder, whereas all animals that had surgery but without desmopressin administration were asymptomatic. Our animal model induced severe endolymphatic hydrops in the cochlea and the saccule, and showed episodes of balance disorder along with spontaneous nystagmus. -
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. -
Endolymphatic Hydrops Meniere's
ENDOLYMPHATIC HYDROPS MENIERE’S DISEASE Introduction: Meniere’s Disease, or endolymphatic hydrops, is a disorder of the inner ear. This condition occurs because of abnormal fluctuations in the inner ear fluid called endolymph. A system of membranes, called the membranous labyrinth, contains a fluid called endolymph. This fluid bathes the inner ear balance and hearing system sensory cells and allows them to function normally. The membranes can become dilated like a balloon when pressure increases. This is called "hydrops". The amount of fluid is normally kept constant by altering the production and absorption of the fluid. Endolymph also contains a specific concentration of sodium, potassium, chloride, and other electrolytes. If the inner ear is damaged by disease, injury, or other causes, the volume and composition of the inner ear fluid can fluctuate with changes in the body’s fluid and electrolyte levels. This fluctuation in inner ear fluid can cause the symptoms of hydrops, including pressure or fullness of the affected ear, tinnitus, hearing loss, and imbalance or dizziness. Treatment of this condition is geared towards stabilizing the body fluid levels so that fluctuations in the endolymph volume can be avoided. Meniere's episodes may occur in clusters in which several attacks may occur within a short period of time. On the other hand, years may pass between episodes. Between the acute attacks, most people are free of symptoms or note mild imbalance, tinnitus, and/or hearing loss. Meniere’s affects roughly 0.2% of the population, about 200 out of 100,000 people (or in other words, 2/1000). -
Nomina Histologica Veterinaria, First Edition
NOMINA HISTOLOGICA VETERINARIA Submitted by the International Committee on Veterinary Histological Nomenclature (ICVHN) to the World Association of Veterinary Anatomists Published on the website of the World Association of Veterinary Anatomists www.wava-amav.org 2017 CONTENTS Introduction i Principles of term construction in N.H.V. iii Cytologia – Cytology 1 Textus epithelialis – Epithelial tissue 10 Textus connectivus – Connective tissue 13 Sanguis et Lympha – Blood and Lymph 17 Textus muscularis – Muscle tissue 19 Textus nervosus – Nerve tissue 20 Splanchnologia – Viscera 23 Systema digestorium – Digestive system 24 Systema respiratorium – Respiratory system 32 Systema urinarium – Urinary system 35 Organa genitalia masculina – Male genital system 38 Organa genitalia feminina – Female genital system 42 Systema endocrinum – Endocrine system 45 Systema cardiovasculare et lymphaticum [Angiologia] – Cardiovascular and lymphatic system 47 Systema nervosum – Nervous system 52 Receptores sensorii et Organa sensuum – Sensory receptors and Sense organs 58 Integumentum – Integument 64 INTRODUCTION The preparations leading to the publication of the present first edition of the Nomina Histologica Veterinaria has a long history spanning more than 50 years. Under the auspices of the World Association of Veterinary Anatomists (W.A.V.A.), the International Committee on Veterinary Anatomical Nomenclature (I.C.V.A.N.) appointed in Giessen, 1965, a Subcommittee on Histology and Embryology which started a working relation with the Subcommittee on Histology of the former International Anatomical Nomenclature Committee. In Mexico City, 1971, this Subcommittee presented a document entitled Nomina Histologica Veterinaria: A Working Draft as a basis for the continued work of the newly-appointed Subcommittee on Histological Nomenclature. This resulted in the editing of the Nomina Histologica Veterinaria: A Working Draft II (Toulouse, 1974), followed by preparations for publication of a Nomina Histologica Veterinaria. -
Ear Development Ear Development
Ear Development Ear Development The ear can be divided into three parts ◦ External Ear Auricle external auditory canal ◦ Middle Ear tympanic cavity auditory tube auditory ossicles malleus, incus, stapes ◦ Inner Ear Cochlea vestibular apparatus semicircular canals Utricle Saccule Development of Inner Ear All of inner ear derivatives arise from ectoderm Late in 3rd week ◦ otic placode (disc) appears next to hindbrain During 4th week ◦ otic placode form: otic pit otic vesicle or otocyst Young neurons delaminate from ventral otocyst Form statoacoustic (vestibulocochlear) ganglion Rhombencephalon region:formation of otic vesicles Derivatives of the 1st & 2nd branchial arches Rhombencephalon region:otic placode , otic pit and otic vesicle Development of Inner Ear epithelial structures of Otic vesicle develops to membranous labyrinth dorsomedial region elongate to form: ◦ endolymphatic appendage endolymphatic sac rest region expanded to: ◦ pars superior Utricle semicircular canals endolymphatic duct ◦ pars inferior Its ventral tip elongate and coil form cochlear duct differentiate to spiral organ of Corti Its rest gives rise to: Saccule connected to cochlea by ductus reuniens Development of the otic vesicle showing a dorsal utricular portion with endolymphatic duct, and a ventral saccular portion Saccule, cochlea & organ of Corti Outgrowth of cochlear duct-spiral fashion 8th wk.- 21/2 turns Cochlear duct conect with saccule through ductus reuniens Sensory cells of Inner Ear specialized mechanotransducers arise in six prosensory regions within developing otic vesicle ◦ In the cochlea organ of Corti ◦ In the saccule and utricle Maculae ◦ in the semicircular canals Cristae All of these sensory regions innervated by the vestibulocochlear nerve Development of the scala tympani & scala vestibuli Note:the auditory nerve and spiral ganglion Scala media - organ of Corti Hair cells Tectorial mem. -
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. -
The Membranous Labyrinth in Vivo from High-Resolution Temporal CT Data
bioRxiv preprint doi: https://doi.org/10.1101/318030; this version posted May 9, 2018. The copyright holder for this preprint (which was not certified by peer review) is the author/funder, who has granted bioRxiv a license to display the preprint in perpetuity. It is made available under aCC-BY-ND 4.0 International license. The Membranous Labyrinth in vivo from high-resolution Temporal CT data Hisaya Tanioka¹* ¹Department of Radiology, Tanioka Clinic *Corresponding author Hisaya Tanioka, MD, PhD Tanioka Clinic Tanioka Bldg. 3F, 6-24-2 Honkomagome Bunkyo-ku, Tokyo 113-0021 Japan Tel & Fax: 81-3-3945-5199 E-mail: [email protected] bioRxiv preprint doi: https://doi.org/10.1101/318030; this version posted May 9, 2018. The copyright holder for this preprint (which was not certified by peer review) is the author/funder, who has granted bioRxiv a license to display the preprint in perpetuity. It is made available under aCC-BY-ND 4.0 International license. The Membranous Labyrinth in vivo from high-resolution Temporal CT data bioRxiv preprint doi: https://doi.org/10.1101/318030; this version posted May 9, 2018. The copyright holder for this preprint (which was not certified by peer review) is the author/funder, who has granted bioRxiv a license to display the preprint in perpetuity. It is made available under aCC-BY-ND 4.0 International license. ABSTRACT A prerequisite for the modeling and understanding of the inner ear mechanics needs the accurate created membranous labyrinth. I present a semi-automated methodology for accurate reconstruction of the membranous labyrinth in vivo from high-resolution temporal bone CT data of normal human subjects. -
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/). -
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).