About this Research Topic

Background

The mature inner ear consists of a complex labyrinth of elaborate sensory organs set in a finely-organized spatial configuration. Vestibular sensory organs detecting angular acceleration reside within the ampullae of three orthogonal semicircular canals, while changes in gravity and linear acceleration are detected primarily by the maculae utriculi and saccule in the vestibule. All vertebrates maintain body equilibrium in space for quality of life and survival. These basic requirements may explain why the phylogenetically-old vestibular system is highly conserved with all vertebrates sharing a common blueprint for basic inner ear configuration and central vestibular pathways. An accurate control of posture, balance, gaze and their perception requires integrating diverse sensorimotor signals within a single CNS framework. Spatially and temporally precise signals from the vestibular sensory organs are transmitted to widespread areas starting mainly with the brainstem vestibular nuclei where the first level of sensory integration occurs. Signaling then proceeds to other brainstem centers, thalamus, cerebral cortex, cerebellum, and spinal cord.
Despite the evolutionary importance of the labyrinths for survival, malformation is not rare during development. Over 40 genes affect the inner ear with many expressed during key inner-ear developmental stages. Many congenital vestibular disorders (CVDs) are characterized by hypoplasia of the semicircular canals with the most common inner ear defect producing a sac-like structure missing the semicircular canals. Despite limited incidence for individual CVDs, epidemiological studies report that vestibular disorders affect 3.3 million children in the US alone. Due to the central role of the vestibular system in daily living, vestibular symptoms impose life-altering disabilities in CVD children, including disorientation, confusion, fatigue while performing daily tasks, and severe challenges in eye-hand coordination, eye tracking, reading, and language.

The overarching goal of this Research Topic is to bring the topic of congenital vestibular disorders to the attention of otolaryngologists, neurologists, and vestibular neuroscientists to increase research in the field leading to improved treatment for CVD children. This objective can be reached by collecting together within a single Research Topic information already available from clinical testing of CVD children and CVD animal models on the structures in the central and peripheral vestibular system that are malformed and dysfunctional, resulting in the behavioral deficits observed in CVD children.
As such, Topic Editors will welcome studies including, but not limited to, comparisons of different clinical techniques used to test CVD children and animal models, as well as studies trying to identify and describe the animal models used to study the peripheral and central vestibular pathologies underlying CVDs.

Contributions to this Research may include:
• What clinical tests are performed presently to evaluate vestibular behavioral performance in children with CVDs, and what do the tests reveal?
• What new clinical tests could be applied to better evaluate vestibular behavior in CVD children?
• What tests now performed on children should also be carried out on CVD animal models?
• What do imaging approaches reveal about peripheral vestibular structures in CVD children, including the pathological development of the sensory organs, vestibular ganglion, and vestibular nerves?
• What do imaging approaches demonstrate about the central vestibular pathways and centers in children with CVDs?
• What do imaging approaches show about peripheral vestibular structures in CVD animal models?
• What do imaging approaches reveal about the central vestibular pathways and centers in CVD animal models?
• How does the pathology in different CVD animal models compares to that found in CVD children?
• What new approaches could be used on CVD animal models to better detect the malformations that occur in the inner ear, vestibular ganglion, and central vestibular pathways and centers?

Keywords: Animal models, Semicircular canal malformation, Childhood balance disorders, Vestibular prostheses, Gene therapy

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