scholarly journals Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function

10.1038/ng842 ◽  
2002 ◽  
Vol 30 (3) ◽  
pp. 277-284 ◽  
Author(s):  
Kiyoto Kurima ◽  
Linda M. Peters ◽  
Yandan Yang ◽  
Saima Riazuddin ◽  
Zubair M. Ahmed ◽  
...  
Keyword(s):  
Hair Cell ◽  
Cell Function ◽  
Cochlear Hair Cell ◽  
Novel Gene

scholarly journals Piccolo is essential for the maintenance of mouse retina but not cochlear hair cell function

Aging ◽  
2021 ◽  
Author(s):  
Peipei Li ◽  
Zhuchun Lin ◽  
Yachun An ◽  
Jing Lin ◽  
Aizhen Zhang ◽  
...  
Keyword(s):  
Hair Cell ◽  
Cell Function ◽  
Mouse Retina ◽  
Cochlear Hair Cell

Histological evaluation of cochlear hair cell damage from noise-induced hearing loss in chinchillas

1990 ◽  
Vol 48 (3) ◽  
pp. 332-333
Author(s):  
R.V. Harrison ◽  
R.J. Mount ◽  
P. White ◽  
N. Fukushima
Keyword(s):  
Hair Cell ◽  
Evoked Potential ◽  
Cell Damage ◽  
Acoustic Trauma ◽  
Histological Evaluation ◽  
Cell Integrity ◽  
Functional Changes ◽  
Cochlear Hair Cell ◽  
Left And Right ◽  
Contralateral Ear

In studies which attempt to define the influence of various factors on recovery of hair cell integrity after acoustic trauma, an experimental and a control ear which initially have equal degrees of damage are required. With in a group of animals receiving an identical level of acoustic trauma there is more symmetry between the ears of each individual, in respect to function, than between animals. Figure 1 illustrates this, left and right cochlear evoked potential (CAP) audiograms are shown for two chinchillas receiving identical trauma. For this reason the contralateral ear is used as control.To compliment such functional evaluations we have devised a scoring system, based on the condition of hair cell stereocilia as revealed by scanning electron microscopy, which permits total stereociliar damage to be expressed numerically. This quantification permits correlation of the degree of structural pathology with functional changes. In this paper wereport experiments to verify the symmetry of stereociliar integrity between two ears, both for normal (non-exposed) animals and chinchillas in which each ear has received identical noise trauma.

scholarly journals Ceramide/sphingomyelin cycle involvement in gentamicin-induced cochlear hair cell death

2014 ◽  
Vol 89 (3) ◽  
pp. 415-421 ◽  
Author(s):  
Le Nguyen Uyen Chi ◽  
Keiji Tabuchi ◽  
Mariko Nakamagoe ◽  
Masahiro Nakayama ◽  
Bungo Nishimura ◽  
...  
Keyword(s):  
Cell Death ◽  
Hair Cell ◽  
Cochlear Hair Cell ◽  
Hair Cell Death ◽  
Sphingomyelin Cycle

scholarly journals Effects of cochlear hair cell ablation on spatial learning/memory

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Z. Jason Qian ◽  
Anthony J. Ricci
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Spatial Learning ◽  
Hair Cells ◽  
Noise Exposure ◽  
Memory Errors ◽  
Cochlear Hair Cells ◽  
Cochlear Hair Cell ◽  
Cell Ablation ◽  
Spatial Learning Memory

AbstractCurrent clinical interest lies in the relationship between hearing loss and cognitive impairment. Previous work demonstrated that noise exposure, a common cause of sensorineural hearing loss (SNHL), leads to cognitive impairments in mice. However, in noise-induced models, it is difficult to distinguish the effects of noise trauma from subsequent SNHL on central processes. Here, we use cochlear hair cell ablation to isolate the effects of SNHL. Cochlear hair cells were conditionally and selectively ablated in mature, transgenic mice where the human diphtheria toxin (DT) receptor was expressed behind the hair-cell specific Pou4f3 promoter. Due to higher Pou4f3 expression in cochlear hair cells than vestibular hair cells, administration of a low dose of DT caused profound SNHL without vestibular dysfunction and had no effect on wild-type (WT) littermates. Spatial learning/memory was assayed using an automated radial 8-arm maze (RAM), where mice were trained to find food rewards over a 14-day period. The number of working memory errors (WME) and reference memory errors (RME) per training day were recorded. All animals were injected with DT during P30–60 and underwent the RAM assay during P90–120. SNHL animals committed more WME and RME than WT animals, demonstrating that isolated SNHL affected cognitive function. Duration of SNHL (60 versus 90 days post DT injection) had no effect on RAM performance. However, younger age of acquired SNHL (DT on P30 versus P60) was associated with fewer WME. This describes the previously undocumented effect of isolated SNHL on cognitive processes that do not directly rely on auditory sensory input.

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