scholarly journals The development of semicircular canals in the inner ear: role of FGFs in sensory cristae

Development ◽  
2004 ◽  
Vol 131 (17) ◽  
pp. 4201-4211 ◽  
Author(s):  
W. Chang
Keyword(s):  
Inner Ear ◽  
Semicircular Canals

scholarly journals The Nuclear Receptor Nor-1 Is Essential for Proliferation of the Semicircular Canals of the Mouse Inner Ear

2002 ◽  
Vol 22 (3) ◽  
pp. 935-945 ◽  
Author(s):  
Tiia Ponnio ◽  
Quiana Burton ◽  
Fred A. Pereira ◽  
Doris K. Wu ◽  
Orla M. Conneely
Keyword(s):  
Inner Ear ◽  
Nuclear Receptor ◽  
Semicircular Canals ◽  
Mammalian Development ◽  
Genomic Locus ◽  
Membranous Labyrinth ◽  
Spatiotemporal Expression ◽  
Proliferative Growth ◽  
Precise Role

ABSTRACT Nor-1 belongs to the nur subfamily of nuclear receptor transcription factors. The precise role of Nor-1 in mammalian development has not been established. However, recent studies indicate a function for this transcription factor in oncogenesis and apoptosis. To examine the spatiotemporal expression pattern of Nor-1 and the developmental and physiological consequences of Nor-1 ablation, Nor-1-null mice were generated by insertion of the lacZ gene into the Nor-1 genomic locus. Disruption of the Nor-1 gene results in inner ear defects and partial bidirectional circling behavior. During early otic development, Nor-1 is expressed exclusively in the semicircular canal forming fusion plates. After formation of the membranous labyrinth, Nor-1 expression in the vestibule is limited to nonsensory epithelial cells localized at the inner edge of the semicircular canals and to the ampullary and utricular walls. In the absence of Nor-1, the vestibular walls fuse together as normal; however, the endolymphatic fluid space in the semicircular canals is diminished and the roof of the ampulla appears flattened due to defective continual proliferative growth of the semicircular canals.

scholarly journals Origins of Vertigo

2021 ◽  
Vol 20 ◽  
Author(s):  
JMS Pearce ◽  
Keyword(s):  
Inner Ear ◽  
Cold Water ◽  
Semicircular Canals ◽  
Vital Role ◽  
Classical Period ◽  
Vestibular Apparatus ◽  
Excessive Heat ◽  
The Brain

The words for vertigo: ‘dinos’, ‘ilinggous’ ,‘skotomatikoi,’ date back to the classical period of Plato and Hippocrates. They were followed by the Latin ‘vertigine’ and ‘scotomia’. Excessive heat or blood in the brain was the original causes of vertigo, followed by Galen’s vaporous pneuma. Not until Flourens demonstration of circling movement in pigeons in which he had destroyed the semicircular canals, and Brown-Séquard’s observation of vertigo in man after syringing the ear with cold water, did the vital role of the vestibular apparatus appear. Subsequent syndromes described by Ménière and Bárány showed the role of the inner ear in causing vertigo in man.

Involvement of programmed cell death in morphogenesis of the vertebrate inner ear

Development ◽  
1997 ◽  
Vol 124 (12) ◽  
pp. 2451-2461 ◽  
Author(s):  
D.M. Fekete ◽  
S.A. Homburger ◽  
M.T. Waring ◽  
A.E. Riedl ◽  
L.F. Garcia
Keyword(s):  
Cell Death ◽  
Epithelial Cells ◽  
Programmed Cell Death ◽  
Inner Ear ◽  
Three Dimensional ◽  
Semicircular Canals ◽  
Otic Vesicle ◽  
New Evidence

An outstanding challenge in developmental biology is to reveal the mechanisms underlying the morphogenesis of complex organs. A striking example is the developing inner ear of the vertebrate, which acquires a precise three-dimensional arrangement of its constituent epithelial cells to form three semicircular canals, a central vestibule and a coiled cochlea (in mammals). In generating a semicircular canal, epithelial cells seem to ‘disappear’ from the center of each canal. This phenomenon has been variously explained as (i) transdifferentiation of epithelium into mesenchyme, (ii) absorption of cells into the expanding canal or (iii) programmed cell death. In this study, an in situ DNA-end labeling technique (the TUNEL protocol) was used to map regions of cell death during inner ear morphogenesis in the chicken embryo from embryonic days 3.5-10. Regions of cell death previously identified in vertebrate ears have been confirmed, including the ventromedial otic vesicle, the base of the endolymphatic duct and the fusion plates of the semicircular canals. New regions of cell death are also described in and around the sensory organs. Reducing normal death using retrovirus-mediated overexpression of human bcl-2 causes abnormalities in ear morphogenesis: hollowing of the center of each canal is either delayed or fails entirely. These data provide new evidence to explain the role of cell death in morphogenesis of the semicircular canals.

The Role of the Endolymphatic Sac in Inner Ear Immunity

1987 ◽  
Vol 103 (5) ◽  
pp. 182-188 ◽  
Author(s):  
Shunichi Tomiyama ◽  
Jeffrey Harris
Keyword(s):  
Inner Ear ◽  
Endolymphatic Sac

scholarly journals Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Xuewen Wu ◽  
Li Zhang ◽  
Yihui Li ◽  
Wenjuan Zhang ◽  
Jianjun Wang ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Mouse Model ◽  
Inner Ear ◽  
Viral Vectors ◽  
Survival Rates ◽  
Semicircular Canals ◽  
Dependent Manner ◽  
Syndrome Type ◽  
Dose Dependent

AbstractMutations in voltage-gated potassium channel KCNE1 cause Jervell and Lange-Nielsen syndrome type 2 (JLNS2), resulting in congenital deafness and vestibular dysfunction. We conducted gene therapy by injecting viral vectors using the canalostomy approach in Kcne1−/− mice to treat both the hearing and vestibular symptoms. Results showed early treatment prevented collapse of the Reissner’s membrane and vestibular wall, retained the normal size of the semicircular canals, and prevented the degeneration of inner ear cells. In a dose-dependent manner, the treatment preserved auditory (16 out of 20 mice) and vestibular (20/20) functions in mice treated with the high-dosage for at least five months. In the low-dosage group, a subgroup of mice (13/20) showed improvements only in the vestibular functions. Results supported that highly efficient transduction is one of the key factors for achieving the efficacy and maintaining the long-term therapeutic effect. Secondary outcomes of treatment included improved birth and litter survival rates. Our results demonstrated that gene therapy via the canalostomy approach, which has been considered to be one of the more feasible delivery methods for human inner ear gene therapy, preserved auditory and vestibular functions in a dose-dependent manner in a mouse model of JLNS2.

Inner Ear Pathology and Otitis Media

1980 ◽  
Vol 89 (3_suppl) ◽  
pp. 249-253 ◽  
Author(s):  
Michael M. Paparella ◽  
Marcos V. Goycoolea ◽  
William L. Meyerhoff
Keyword(s):  
Inner Ear ◽  
Otitis Media ◽  
Round Window ◽  
Round Window Membrane

Insidious inner ear complications of otitis media have been and are being studied in our laboratory. The purpose of this paper is to review these studies, coordinate, capsulize and highlight the results with emphasis on the transport role of the round window membrane.

The Role of the Endolymphatic Sac in Inner Ear Immunity

1987 ◽  
Vol 103 (3-4) ◽  
pp. 182-188 ◽  
Author(s):  
Shunichi Tomiyama ◽  
Jeffrey P. Harris
Keyword(s):  
Inner Ear ◽  
Endolymphatic Sac

scholarly journals Role of microRNAs in inner ear development and hearing loss

Gene ◽  
2019 ◽  
Vol 686 ◽  
pp. 49-55 ◽  
Author(s):  
Rahul Mittal ◽  
George Liu ◽  
Sai P. Polineni ◽  
Nicole Bencie ◽  
Denise Yan ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Ear Development ◽  
Inner Ear Development
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