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Hearing Loss: A general term for the complete or partial loss of the ability to hear from one or both ears.

Pleiotropy

JoVE 10780

Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes, which are involved in pigmentation and also in the early development of the ear. SOX10 is also expressed in nerve tissue that eventually contributes to the enteric nervous system in the gut, which controls the contractions necessary for waste elimination. In this way, SOX10 exhibits pleiotropic effects, because it influences multiple phenotypes. Pleiotropy can arise through several mechanisms. Gene pleiotropy occurs when a gene has various functions due to encoding a product that interacts with multiple proteins or catalyzes multiple reactions. For example, in humans, an abnormal copy of the SOX10 gene, in which a region is deleted, can lead to developmental defects that include a white forelock, different-colored irises (e.g., one blue and one brown), and regions of unpigmented skin. These traits are all symptoms of a di

 Core: Classical and Modern Genetics

Cranial Nerves Exam II (VII-XII)

JoVE 10005

Source:Tracey A. Milligan, MD; Tamara B. Kaplan, MD; Neurology, Brigham and Women's/Massachusetts General Hospital, Boston, Massachusetts, USA


The cranial nerve examination follows the mental status evaluation in a neurological exam. However, the examination begins with observations made upon greeting…

 Physical Examinations III

Ear Exam

JoVE 10148

Source: Richard Glickman-Simon, MD, Assistant Professor, Department of Public Health and Community Medicine, Tufts University School of Medicine, MA


This video describes the examination of the ear, beginning with a review of its surface and interior anatomy (Figure 1). The cartilaginous auricle consists of the helix,…

 Physical Examinations II

The Staircase Procedure for Finding a Perceptual Threshold

JoVE 10231

Source: Laboratory of Jonathan Flombaum—Johns Hopkins University


Psychophysics is the name for a set of methods in perceptual psychology designed in order to relate the actual intensity of stimuli to their perceptual intensity. One important aspect of psychophysics involves the measurement of perceptual thresholds: How bright does a…

 Sensation and Perception

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

1Experimental Neuropsychopharmacology, Federal Institute for Drugs and Medical Devices, 2KBRwyle GmbH, 3Cognitive Neurophysiology, Department of Psychiatry and Psychotherapy, Faculty of Medicine, University of Cologne, 4Molecular and Cellular Cognition, German Center for Neurodegenerative Diseases (DZNE), 5Institute of Neurophysiology, Faculty of Medicine, University of Cologne, 6Federal Institute for Drugs and Medical Devices (BfArM), 7Thescon GmbH

JoVE 59200

 Neuroscience

A Unified Methodological Framework for Vestibular Schwannoma Research

1Eaton Peabody Laboratories, Department of Otolaryngology, Massachusetts Eye and Ear, 2Department of Otolaryngology, Harvard Medical School, 3Department of Otolaryngology, Vienna General Hospital, Medical University of Vienna, 4Program in Speech and Hearing Bioscience and Technology, Harvard Medical School

JoVE 55827

 Cancer Research

A Comparative Study of Drug Delivery Methods Targeted to the Mouse Inner Ear: Bullostomy Versus Transtympanic Injection

1Instituto de Investigaciones Biomédicas (IIBm) Alberto Sols CSIC-UAM, 2Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III (ISCIII), 3Instituto de Investigación Sanitaria La Paz (IdiPAZ), 4Facultad de Veterinaria, Universidad Complutense de Madrid, 5Departmento de Otorrino laringología, Hospital Universitario La Paz

JoVE 54951

 Biology

Extracellular Recording of Neuronal Activity Combined with Microiontophoretic Application of Neuroactive Substances in Awake Mice

1Auditory Neuroscience Laboratory, Institute of Neuroscience of Castilla y León, University of Salamanca, 2Neural Systems Laboratory, Institute for Systems Research, University of Maryland, 3Medical Research Council Institute of Hearing Research, 4Department of Cell Biology and Pathology, Faculty of Medicine, University of Salamanca

JoVE 53914

 Neuroscience

Proton Therapy Delivery and Its Clinical Application in Select Solid Tumor Malignancies

1Department of Radiation Oncology, University of Maryland School of Medicine, 2Department of Radiation Oncology, University of Maryland Medical Center, 3Department of Medicine, University of Maryland School of Medicine, University of Maryland Greenebaum Comprehensive Cancer Center

JoVE 58372

 Medicine

A Multimodal Imaging- and Stimulation-based Method of Evaluating Connectivity-related Brain Excitability in Patients with Epilepsy

1Department of Neurology, Harvard Medical School, 2Department of Neurology, Beth Israel Deaconess Medical Center, 3Berenson-Allen Center for Noninvasive Brain Stimulation, Beth Israel Deaconess Medical Center, 4Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, 5Department of Neurology, Massachusetts General Hospital

JoVE 53727

 Medicine

Simple Surgical Induction of Conductive Hearing Loss with Verification Using Otoscope Visualization and Behavioral Clap Startle Response in Rat

1Instituto de Neurobiología, Universidad Nacional Autónoma de México, México, 2State Key Laboratory of Marine Pollution (SKLMP), City University of Hong Kong, Hong Kong, 3Department of Biomedical Sciences, College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Hong Kong, 4Department of Physics, City University of Hong Kong, Hong Kong, 5Department of Clinical Oncology, Queen Elizabeth Hospital, Hong Kong, 6Department of Materials Science and Engineering, City University of Hong Kong, Hong Kong

Video Coming Soon

JoVE 57993

 JoVE In-Press

A Method for Tracking the Time Evolution of Steady-State Evoked Potentials

1Advanced Center for Electrical and Electronic Engineering (AC3E), Universidad Técnica Federico Santa María, 2Department of Electronic Engineering, Universidad Técnica Federico Santa María, 3Neuroinformatics Department, Cuban Neuroscience Center, 4Centro de Investigación y Desarrollo en IngenierÍa, Universidad de Valparaíso

JoVE 59898

 Neuroscience

The Endoscope-Assisted Minimally Invasive Retro-Sigmoid Approach (EAMIRSA): A Combination of Techniques for Improving Middle Skull Base Surgery

1Permanent Temporal Bone Dissection Laboratory, University of Perugia, 2Otolaryngology Department, Meyer Children Hospital, 3Highland Instruments, 4Otolaryngology Department, Cochlear Implant Unit, Santobono Children Hospital, 5Otolaryngology Department, Ospedale Civile of Mantua

Video Coming Soon

JoVE 58522

 JoVE In-Press

In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears

1Department of Radiology, University Hospital, LMU Munich, 2Department of Otorhinolaryngology - Head and Neck Surgery, SLK-Kliniken Heilbronn GmbH, 3Department of Otorhinolaryngology Head and Neck Surgery, Ludwig-Maximilians-University Hospital Munich, German Centre for Vertigo and Balance Disorder

JoVE 57091

 Medicine

A Surgical Procedure for the Administration of Drugs to the Inner Ear in a Non-Human Primate Common Marmoset (Callithrix jacchus)

1Division of Regenerative Medicine, Jikei University School of Medicine, 2Department of Otorhinolaryngology, Jikei University School of Medicine, 3Department of Otorhinolaryngology, Head and Neck Surgery, Keio University School of Medicine, 4Laboratory Animal Facilities, Jikei University School of Medicine

JoVE 56574

 Medicine

Calcification of Vascular Smooth Muscle Cells and Imaging of Aortic Calcification and Inflammation

1Anesthesia Center for Critical Care Research of the Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, 2Cardiovascular Research Center and Cardiology Division of the Department of Medicine, Massachusetts General Hospital, 3Cardiovascular Division, Brigham and Women's Hospital, 4Harvard Medical School, 5Department of Anesthesiology, Uniklinik RWTH Aachen, RWTH Aachen University, 6Center for Immunology and Inflammatory Diseases and the Division of Rheumatology, Allergy, and Immunology of the Department of Medicine, Massachusetts General Hospital

JoVE 54017

 Medicine

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

1Department of Integrative Physiology, National Institute for Physiological Sciences, 2Department of Otolaryngology, Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences and Medical School, 3Department of Otorhinolaryngology, Kansai Rosai Hospital, 4Institute for Biomagnetism and Biosignalanalysis, University of Muenster, 5Institute for Epidemiology and Social Medicine, University of Muenster, 6Sokendai Graduate University for Advanced Studies

JoVE 53264

 Behavior
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