Strategies in Cochlear Nerve Regeneration, Guidance and Protection
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Pathophysiological Mechanisms and Functional Hearing Consequences of Auditory Neuropathy
doi:10.1093/brain/awv270 BRAIN 2015: 138; 3141–3158 | 3141 REVIEW ARTICLE Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy Gary Rance1 and Arnold Starr2 The effects of inner ear abnormality on audibility have been explored since the early 20th century when sound detection measures were first used to define and quantify ‘hearing loss’. The development in the 1970s of objective measures of cochlear hair cell Downloaded from function (cochlear microphonics, otoacoustic emissions, summating potentials) and auditory nerve/brainstem activity (auditory brainstem responses) have made it possible to distinguish both synaptic and auditory nerve disorders from sensory receptor loss. This distinction is critically important when considering aetiology and management. In this review we address the clinical and pathophysiological features of auditory neuropathy that distinguish site(s) of dysfunction. We describe the diagnostic criteria for: (i) presynaptic disorders affecting inner hair cells and ribbon synapses; (ii) postsynaptic disorders affecting unmyelinated auditory http://brain.oxfordjournals.org/ nerve dendrites; (iii) postsynaptic disorders affecting auditory ganglion cells and their myelinated axons and dendrites; and (iv) central neural pathway disorders affecting the auditory brainstem. We review data and principles to identify treatment options for affected patients and explore their benefits as a function of site of lesion. 1 Department of Audiology and Speech Pathology, The University of Melbourne, -
Fibroblast Growth Factor (Fgf) Implication in the Neonatal Development of the Cochlear Innervation G
FIBROBLAST GROWTH FACTOR (FGF) IMPLICATION IN THE NEONATAL DEVELOPMENT OF THE COCHLEAR INNERVATION G. Després, I. Jalenques, R. Romand To cite this version: G. Després, I. Jalenques, R. Romand. FIBROBLAST GROWTH FACTOR (FGF) IMPLICATION IN THE NEONATAL DEVELOPMENT OF THE COCHLEAR INNERVATION. Journal de Physique IV Proceedings, EDP Sciences, 1992, 02 (C1), pp.C1-173-C1-176. 10.1051/jp4:1992134. jpa-00251205 HAL Id: jpa-00251205 https://hal.archives-ouvertes.fr/jpa-00251205 Submitted on 1 Jan 1992 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. JOURNAL DE PHYSIQUE IV Colloque C1, suppICment au Journal de Physique 111, Volume 2, avril 1992 FIBROBLAST GROWMI FACTOR (FGF) IMPLICATION IN TIlE NEONATAL DEVELOPMENT OF THE COCIiLEAR INNERVATION G. DESPR~S,I. JALENQUES and R. ROMAND Laboratoire de Neurobiolc@e et Physwlogie du Dkeloppement, Universite'BIaise Pascal, F-63177Aubi2re ceder, France The presence of fibroblast growth factor-like protein has been investigated on cryostat sections from Sprague Dawley rat cochleae and auditory brainstem nuclei of various neonatal stages by indirect immunofluorescence and immunoperoxydase techniques with an antibody directed against the 1-24 amino-acid sequence of brain derived basic FGF. -
5.1. Structure of the Spiral Ganglion
CHAPTER 5. INNERVATION OF THE ORGAN OF CORTI The investigation of nerve components of the acoustic system’s periph- eral part is so difficult methodologically that a whole series of questions which were solved long ago during the investigation of the other sensory system have not yet been clarified. The basic difficulty for the morphologists lies in the fact that the organ of Corti, together with its nerve elements, is located within the osseous tissue. In addition, it is in the shape of a spirally involut- ed geometrical figure. These structural peculiarities create considerable dif- ficulties during the determination of the connections between the types of peripheral and central neuron’s processes and bodies of the spiral ganglion of the cochlea. Therefore, most of the work on the cochlea’s innervation and the computation of the different element’s quantity demands an application of special methods, including graphical reconstruction of the serial sections. The Golgi method was and still remains the basic histological method of the organ of Corti’s innervation study, which has been supplemented by the cochlea’s electron-microscope investigations in the normal conditions and during the experimentally induced degenerations. 5.1. Structure of the spiral ganglion The neurons which innervate the auditory receptor cells form a spiral ganglion: a nerve-knot of the VIII pair’s acoustic part of the craniocerebral nerves. The ganglion fills the Rosental’s canal in the cochlea’s axis and re- peats the number of its spiral turns. The ganglionic neuron has, as a rule, a widened body with two processes: peripheral and central (Diagram 7). -
Auditory Neuropathy After Damage to Cochlear Spiral Ganglion Neurons
www.nature.com/scientificreports OPEN Auditory Neuropathy after Damage to Cochlear Spiral Ganglion Neurons in Mice Resulting from Conditional Received: 27 July 2016 Accepted: 15 June 2017 Expression of Diphtheria Toxin Published online: 25 July 2017 Receptors Haolai Pan1, Qiang Song1, Yanyan Huang1, Jiping Wang1, Renjie Chai2, Shankai Yin1 & Jian Wang 1,3 Auditory neuropathy (AN) is a hearing disorder characterized by normal cochlear amplifcation to sound but poor temporal processing and auditory perception in noisy backgrounds. These defcits likely result from impairments in auditory neural synchrony; such dyssynchrony of the neural responses has been linked to demyelination of auditory nerve fbers. However, no appropriate animal models are currently available that mimic this pathology. In this study, Cre-inducible diphtheria toxin receptor (iDTR+/+) mice were cross-mated with mice containing Cre (Bhlhb5-Cre+/−) specifc to spiral ganglion neurons (SGNs). In double-positive ofspring mice, the injection of diphtheria toxin (DT) led to a 30–40% rate of death for SGNs, but no hair cell damage. Demyelination types of pathologies were observed around the surviving SGNs and their fbers, many of which were distorted in shape. Correspondingly, a signifcant reduction in response synchrony to amplitude modulation was observed in this group of animals compared to the controls, which had a Cre− genotype. Taken together, our results suggest that SGN damage following the injection of DT in mice with Bhlhb5-Cre+/− and iDTR+/− is likely to be a good AN model of demyelination. Auditory neuropathy (AN) is a hearing disorder characterized as having normal cochlear microphonic (CM) potentials and otoacoustic emissions (OAEs), but largely reduced or missing auditory brainstem responses (ABRs). -
Common Vestibular Function Tests
Common Vestibular Function Tests Authors: Barbara Susan Robinson, PT, DPT; Lisa Heusel-Gillig PT DPT NCS Fact Sheet The purpose of Vestibular Function Tests (VFTs) is to determine the health of the vestibular portion of the inner ear. These tests are commonly performed by ENTs, Audiologists, and Otolaryngologists Electronystagmography or Videonystagmography Electronystagmography (ENG test) or Videonystagmography (VNG test) evaluate the inner ear. Both record eye movements during a group of tests in light and dark rooms. During the ENG test, small electrodes are placed on the skin near the eyes to record eye movements. For the VNG test, eye movements are recorded by a video camera mounted inside of goggles that are worn during testing. ENG and VNG tests evaluate eye movements while following a visual target (tracking Produced by test) or during body and head position changes (positional test). The caloric test evaluates eye movements in response to cool or warm air (or water) placed in the ear canal. If there is no response to warm or cool air or water, ice water may be used in order to try to produce a response. The caloric test determines the difference between the function of the left and right inner ear. During this test, you may experience dizziness or nausea. You may be asked questions (math questions, city names, alphabet tasks) to distract you in order to get the best results. A Special Interest Group of Contact us: ANPT Other Common Vestibular Function Tests 5841 Cedar Lake Rd S. The rotary chair test is used along with the VNG to confirm the diagnosis and assess Ste 204 compensation of the vestibular system. -
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). -
Diseases of the Brainstem and Cranial Nerves of the Horse: Relevant Examination Techniques and Illustrative Video Segments
IN-DEPTH: NEUROLOGY Diseases of the Brainstem and Cranial Nerves of the Horse: Relevant Examination Techniques and Illustrative Video Segments Robert J. MacKay, BVSc (Dist), PhD, Diplomate ACVIM Author’s address: Alec P. and Louise H. Courtelis Equine Teaching Hospital, College of Veterinary Medicine, University of Florida, Gainesville, FL 32610; e-mail: mackayr@ufl.edu. © 2011 AAEP. 1. Introduction (pons and cerebellum) and myelencephalon (me- This lecture focuses on the functions of the portions dulla oblongata). Because the diencephalon was of the brainstem caudal to the diencephalon. In discussed in the previous lecture under Forebrain addition to regulation of many of the homeostatic Diseases, it will not be covered here. mechanisms of the body, this part of the brainstem controls consciousness, pupillary diameter, eye 3. Functions (Location) movement, facial expression, balance, prehension, mastication and swallowing of food, and movement Pupillary Light Response, Pupil Size (Midbrain, Cranial and coordination of the trunk and limbs. Dysfunc- Nerves II, III) tion of the brainstem and/or cranial nerves therefore In the normal horse, pupil size reflects the balance of manifests in a great variety of ways including re- sympathetic (dilator) and parasympathetic (con- duced consciousness, ataxia, limb weakness, dys- strictor) influences on the smooth muscle of the phagia, facial paralysis, jaw weakness, nystagmus, iris.2–4 Preganglionic neurons for sympathetic and strabismus. Careful neurologic examination supply to the head arise in the gray matter of the in the field can provide accurate localization of first four thoracic segments of the spinal cord and brainstem and cranial nerve lesions. Recognition subsequently course rostrally in the cervical sympa- of brainstem/cranial nerve dysfunction is an impor- thetic nerve within the vagosympathetic trunk. -
Auditory and Vestibular Systems Objective • to Learn the Functional
Auditory and Vestibular Systems Objective • To learn the functional organization of the auditory and vestibular systems • To understand how one can use changes in auditory function following injury to localize the site of a lesion • To begin to learn the vestibular pathways, as a prelude to studying motor pathways controlling balance in a later lab. Ch 7 Key Figs: 7-1; 7-2; 7-4; 7-5 Clinical Case #2 Hearing loss and dizziness; CC4-1 Self evaluation • Be able to identify all structures listed in key terms and describe briefly their principal functions • Use neuroanatomy on the web to test your understanding ************************************************************************************** List of media F-5 Vestibular efferent connections The first order neurons of the vestibular system are bipolar cells whose cell bodies are located in the vestibular ganglion in the internal ear (NTA Fig. 7-3). The distal processes of these cells contact the receptor hair cells located within the ampulae of the semicircular canals and the utricle and saccule. The central processes of the bipolar cells constitute the vestibular portion of the vestibulocochlear (VIIIth cranial) nerve. Most of these primary vestibular afferents enter the ipsilateral brain stem inferior to the inferior cerebellar peduncle to terminate in the vestibular nuclear complex, which is located in the medulla and caudal pons. The vestibular nuclear complex (NTA Figs, 7-2, 7-3), which lies in the floor of the fourth ventricle, contains four nuclei: 1) the superior vestibular nucleus; 2) the inferior vestibular nucleus; 3) the lateral vestibular nucleus; and 4) the medial vestibular nucleus. Vestibular nuclei give rise to secondary fibers that project to the cerebellum, certain motor cranial nerve nuclei, the reticular formation, all spinal levels, and the thalamus. -
Use of Calcium-Binding Proteins to Map Inputs in Vestibular Nuclei of the Gerbil
THE JOURNAL OF COMPARATIVE NEUROLOGY 386:317–327 (1997) Use of Calcium-Binding Proteins to Map Inputs in Vestibular Nuclei of the Gerbil GOLDA ANNE KEVETTER* AND ROBERT B. LEONARD Departments of Otolaryngology, Anatomy and Neurosciences, and Physiology and Biophysics, University of Texas Medical Branch, Galveston, Texas 77555 ABSTRACT We wished to determine whether calbindin and/or calretinin are appropriate markers for vestibular afferents, a population of neurons in the vestibular nuclear complex, or cerebellar Purkinje inputs. To accomplish this goal, immunocytochemical staining was observed in gerbils after lesions of the vestibular nerve central to the ganglion, the cerebellum, or both. Eleven to fourteen days after recovery, the brain was processed for immunocytochemical identification of calretinin and calbindin. After lesion of the vestibular nerve, no calretinin staining was seen in any of the vestibular nuclei except for a population of intrinsic neurons, which showed no obvious change in number or staining pattern. Calbindin staining was reduced in all nuclei except the dorsal part of the lateral vestibular nuclei. The density of staining of each marker, measured in the magnocellular medial vestibular nucleus, was signifi- cantly reduced. After the cerebellar lesion, no differences in calretinin staining were noted. However, calbindin staining was greatly reduced in all nuclei. The density of staining, measured in the caudal medial vestibular nucleus, was significantly lower. After a combined lesion of the cerebellum and vestibular nerve, the distribution and density of calretinin staining resembled that after vestibular nerve section alone, whereas calbindin staining was no longer seen. This study demonstrates that calretinin and calbindin are effective markers for the identification of vestibular afferents. -
Cranial Nerves 1, 5, 7-12
Cranial Nerve I Olfactory Nerve Nerve fiber modality: Special sensory afferent Cranial Nerves 1, 5, 7-12 Function: Olfaction Remarkable features: – Peripheral processes act as sensory receptors (the other special sensory nerves have separate Warren L Felton III, MD receptors) Professor and Associate Chair of Clinical – Primary afferent neurons undergo continuous Activities, Department of Neurology replacement throughout life Associate Professor of Ophthalmology – Primary afferent neurons synapse with secondary neurons in the olfactory bulb without synapsing Chair, Division of Neuro-Ophthalmology first in the thalamus (as do all other sensory VCU School of Medicine neurons) – Pathways to cortical areas are entirely ipsilateral 1 2 Crania Nerve I Cranial Nerve I Clinical Testing Pathology Anosmia, hyposmia: loss of or impaired Frequently overlooked in neurologic olfaction examination – 1% of population, 50% of population >60 years Aromatic stimulus placed under each – Note: patients with bilateral anosmia often report nostril with the other nostril occluded, eg impaired taste (ageusia, hypogeusia), though coffee, cloves, or soap taste is normal when tested Note that noxious stimuli such as Dysosmia: disordered olfaction ammonia are not used due to concomitant – Parosmia: distorted olfaction stimulation of CN V – Olfactory hallucination: presence of perceived odor in the absence of odor Quantitative clinical tests are available: • Aura preceding complex partial seizures of eg, University of Pennsylvania Smell temporal lobe origin -
Cranial Nerve VIII
Cranial Nerve VIII Color Code Important (The Vestibulo-Cochlear Nerve) Doctors Notes Notes/Extra explanation Please view our Editing File before studying this lecture to check for any changes. Objectives At the end of the lecture, the students should be able to: ✓ List the nuclei related to vestibular and cochlear nerves in the brain stem. ✓ Describe the type and site of each nucleus. ✓ Describe the vestibular pathways and its main connections. ✓ Describe the auditory pathway and its main connections. Due to the difference of arrangement of the lecture between the girls and boys slides we will stick to the girls slides then summarize the pathway according to the boys slides. Ponto-medullary Sulcus (cerebello- pontine angle) Recall: both cranial nerves 8 and 7 emerge from the ventral surface of the brainstem at the ponto- medullary sulcus (cerebello-pontine angle) Brain – Ventral Surface Vestibulo-Cochlear (VIII) 8th Cranial Nerve o Type: Special sensory (SSA) o Conveys impulses from inner ear to nervous system. o Components: • Vestibular part: conveys impulses associated with body posture ,balance and coordination of head & eye movements. • Cochlear part: conveys impulses associated with hearing. o Vestibular & cochlear parts leave the ventral surface* of brain stem through the pontomedullary sulcus ‘at cerebellopontine angle*’ (lateral to facial nerve), run laterally in posterior cranial fossa and enter the internal acoustic meatus along with 7th (facial) nerve. *see the previous slide Auditory Pathway Only on the girls’ slides 04:14 Characteristics: o It is a multisynaptic pathway o There are several locations between medulla and the thalamus where axons may synapse and not all the fibers behave in the same manner. -
Auditory Nerve.Pdf
1 Sound waves from the auditory environment all combine in the ear canal to form a complex waveform. This waveform is deconstructed by the cochlea with respect to time, loudness, and frequency and neural signals representing these features are carried into the brain by the auditory nerve. It is thought that features of the sounds are processed centrally along parallel and hierarchical pathways where eventually percepts of the sounds are organized. 2 In mammals, the neural representation of acoustic information enters the brain by way of the auditory nerve. The auditory nerve terminates in the cochlear nucleus, and the cochlear nucleus in turn gives rise to multiple output projections that form separate but parallel limbs of the ascending auditory pathways. How the brain normally processes acoustic information will be heavily dependent upon the organization of auditory nerve input to the cochlear nucleus and on the nature of the different neural circuits that are established at this early stage. 3 This histology slide of a cat cochlea (right) illustrates the sensory receptors, the auditory nerve, and its target the cochlear nucleus. The orientation of the cut is illustrated by the pink line in the drawing of the cat head (left). We learned about the relationship between these structures by inserting a dye-filled micropipette into the auditory nerve and making small injections of the dye. After histological processing, stained single fibers were reconstruct back to their origin, and traced centrally to determine how they terminated in the brain. We will review the components of the nerve with respect to composition, innervation of the receptors, cell body morphology, myelination, and central terminations.