Mechanoelectrical transduction of adult outer hair cells studied in a gerbil hemicochlea

Nature ◽  
2004 ◽  
Vol 429 (6993) ◽  
pp. 766-770 ◽  
Author(s):  
David Z. Z. He ◽  
Shuping Jia ◽  
Peter Dallos
Keyword(s):  
Hair Cells ◽  
Outer Hair Cells ◽  
Mechanoelectrical Transduction

scholarly journals A Tmc1 mutation reduces calcium permeability and expression of mechanoelectrical transduction channels in cochlear hair cells

2019 ◽  
Vol 116 (41) ◽  
pp. 20743-20749 ◽  
Author(s):  
Maryline Beurg ◽  
Amanda Barlow ◽  
David N. Furness ◽  
Robert Fettiplace
Keyword(s):  
Hair Cells ◽  
Single Channel ◽  
Maximum Amplitude ◽  
Outer Hair Cells ◽  
Single Amino Acid ◽  
Wild Type ◽  
Cochlear Hair Cells ◽  
Proximate Cause ◽  
Mechanoelectrical Transduction ◽  
Calcium Permeability

Mechanoelectrical transducer (MET) currents were recorded from cochlear hair cells in mice with mutations of transmembrane channel-like protein TMC1 to study the effects on MET channel properties. We characterized a Tmc1 mouse with a single-amino-acid mutation (D569N), homologous to a dominant human deafness mutation. Measurements were made in both Tmc2 wild-type and Tmc2 knockout mice. By 30 d, Tmc1 pD569N heterozygote mice were profoundly deaf, and there was substantial loss of outer hair cells (OHCs). MET current in OHCs of Tmc1 pD569N mutants developed over the first neonatal week to attain a maximum amplitude one-third the size of that in Tmc1 wild-type mice, similar at apex and base, and lacking the tonotopic size gradient seen in wild type. The MET-channel Ca2+ permeability was reduced 3-fold in Tmc1 pD569N homozygotes, intermediate deficits being seen in heterozygotes. Reduced Ca2+ permeability resembled that of the Tmc1 pM412K Beethoven mutant, a previously studied semidominant mouse mutation. The MET channel unitary conductance, assayed by single-channel recordings and by measurements of current noise, was unaffected in mutant apical OHCs. We show that, in contrast to the Tmc1 M412K mutant, there was reduced expression of the TMC1 D569N channel at the transduction site assessed by immunolabeling, despite the persistence of tip links. The reduction in MET channel Ca2+ permeability seen in both mutants may be the proximate cause of hair-cell apoptosis, but changes in bundle shape and protein expression in Tmc1 D569N suggest another role for TMC1 apart from forming the channel.

Functional effects of a monoclonal antibody on mechanoelectrical transduction in outer hair cells

2002 ◽  
Vol 164 (1-2) ◽  
pp. 190-205 ◽  
Author(s):  
Claudia C. Schulte ◽  
Jens Meyer ◽  
David N. Furness ◽  
Carole M. Hackney ◽  
Thomas R. Kleyman ◽  
...  
Keyword(s):  
Monoclonal Antibody ◽  
Hair Cells ◽  
Outer Hair Cells ◽  
Mechanoelectrical Transduction

scholarly journals Distinct roles of stereociliary links in the nonlinear sound processing and noise resistance of cochlear outer hair cells

2020 ◽  
Vol 117 (20) ◽  
pp. 11109-11117
Author(s):  
Woongsu Han ◽  
Jeong-Oh Shin ◽  
Ji-Hyun Ma ◽  
Hyehyun Min ◽  
Jinsei Jung ◽  
...  
Keyword(s):  
Hair Cells ◽  
Otoacoustic Emissions ◽  
Essential Role ◽  
High Sensitivity ◽  
Outer Hair Cells ◽  
Tectorial Membrane ◽  
Sound Processing ◽  
Mechanoelectrical Transduction ◽  
Distortion Product ◽  
Membrane Attachment

Outer hair cells (OHCs) play an essential role in hearing by acting as a nonlinear amplifier which helps the cochlea detect sounds with high sensitivity and accuracy. This nonlinear sound processing generates distortion products, which can be measured as distortion-product otoacoustic emissions (DPOAEs). The OHC stereocilia that respond to sound vibrations are connected by three kinds of extracellular links: tip links that connect the taller stereocilia to shorter ones and convey force to the mechanoelectrical transduction channels, tectorial membrane-attachment crowns (TM-ACs) that connect the tallest stereocilia to one another and to the overlying TM, and horizontal top connectors (HTCs) that link adjacent stereocilia. While the tip links have been extensively studied, the roles that the other two types of links play in hearing are much less clear, largely because of a lack of suitable animal models. Here, while analyzing genetic combinations of tubby mice, we encountered models missing both HTCs and TM-ACs or HTCs alone. We found that the tubby mutation causes loss of both HTCs and TM-ACs due to a mislocalization of stereocilin, which results in OHC dysfunction leading to severe hearing loss. Intriguingly, the addition of the modifier allele modifier of tubby hearing 1 in tubby mice selectively rescues the TM-ACs but not the HTCs. Hearing is significantly rescued in these mice with robust DPOAE production, indicating an essential role of the TM-ACs but not the HTCs in normal OHC function. In contrast, the HTCs are required for the resistance of hearing to damage caused by noise stress.

scholarly journals The contribution of TMC1 to adaptation of mechanoelectrical transduction channels in cochlear outer hair cells

2019 ◽  
Vol 597 (24) ◽  
pp. 5949-5961
Author(s):  
Adam C. Goldring ◽  
Maryline Beurg ◽  
Robert Fettiplace
Keyword(s):  
Hair Cells ◽  
Outer Hair Cells ◽  
Mechanoelectrical Transduction

scholarly journals A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics

2017 ◽  
Vol 8 (1) ◽  
Author(s):  
Victoria A. Lukashkina ◽  
Snezana Levic ◽  
Andrei N. Lukashkin ◽  
Nicola Strenzke ◽  
Ian J. Russell
Keyword(s):  
Gap Junctions ◽  
Hair Cells ◽  
High Frequency ◽  
Basilar Membrane ◽  
Frequency Tuning ◽  
Outer Hair Cells ◽  
Mechanoelectrical Transduction ◽  
Age Related ◽  
Junction Protein ◽  
Cochlear Amplification

Abstract Accelerated age-related hearing loss disrupts high-frequency hearing in inbred CD-1 mice. The p.Ala88Val (A88V) mutation in the gene coding for the gap-junction protein connexin30 (Cx30) protects the cochlear basal turn of adult CD-1Cx30 A88V/A88V mice from degeneration and rescues hearing. Here we report that the passive compliance of the cochlear partition and active frequency tuning of the basilar membrane are enhanced in the cochleae of CD-1Cx30 A88V/A88V compared to CBA/J mice with sensitive high-frequency hearing, suggesting that gap junctions contribute to passive cochlear mechanics and energy distribution in the active cochlea. Surprisingly, the endocochlear potential that drives mechanoelectrical transduction currents in outer hair cells and hence cochlear amplification is greatly reduced in CD-1Cx30 A88V/A88V mice. Yet, the saturating amplitudes of cochlear microphonic potentials in CD-1Cx30 A88V/A88V and CBA/J mice are comparable. Although not conclusive, these results are compatible with the proposal that transmembrane potentials, determined mainly by extracellular potentials, drive somatic electromotility of outer hair cells.

Further studies on the ultrastructure of the cochlea

1990 ◽  
Vol 48 (3) ◽  
pp. 440-441
Author(s):  
Zhixian Wang ◽  
Pinjin Zhu ◽  
Jianhe Sun ◽  
Xuezheng Song
Keyword(s):  
Hair Cells ◽  
Military Medicine ◽  
Outer Hair Cells ◽  
Apical Surface ◽  
Epithelial Surface ◽  
Hearing Research ◽  
New Findings ◽  
Fine Structures ◽  
Temporal Bones ◽  
Accurate Observation

Hearing research is important not only for clinical, professional and military medicine, but also for toxicology, gerontology and genetics. Ultrastructure of the cochlea attracts much attention of electron microscopists, (1―3) but the research lags far behind that of the other parts of the organnism. On the basis of careful microdissection, technical improvment and accurate observation, we have got some new findings which have not been reported in the literature.We collected four cochleas from human corpses. Temporal bones dissected 1 h after death and cochleas perfused with fixatives 4 h after death were good enough in terms of preservation of fine structures. SEM:The apical surface of OHCs (Outer hair cells) and DTs (Deiters cells) is narrower than that of IPs (Inner pillar cells). The mosaic configuration of the reticular membrane is not typical. The stereocilia of IHCs (Inner hair cells) are not uniform and some kinocilia could be seen on the OHCs in adults. The epithelial surface of RM (Reissner’s membrane) is not smooth and no mesh could be seen on the mesothelial surface of RM. TEM.

Pengaruh sisplatin dosis tinggi terhadap penurunan fungsi sel rambut luar koklea

2013 ◽  
Vol 40 (2) ◽  
Author(s):  
Asti Kristianti ◽  
Teti Madiadipoera ◽  
Bogi Soeseno
Keyword(s):  
Hair Cells ◽  
Malignant Neoplasm ◽  
Otoacoustic Emission ◽  
Outer Hair Cells ◽  
High Dose ◽  
Distortion Product Otoacoustic Emission ◽  
Inclusion Criteria ◽  
Cochlear Hair Cells ◽  
Distortion Product ◽  
Bilateral Sensorineural Hearing Loss

Background: Chemotherapy is worldwide used nowadays, and its toxicity still remain a problemespecially toxicity to the ear (ototoxicity). Cisplatin (cis-diamminedichloroplatinum) is one of themost commonly used chemotherapy and highly potent in treating epithelial malignancies. Ototoxicitycaused by cisplatin is irreversible, progressive, bilateral, sensorineural hearing loss especially on highfrequency (4-8 KHz) accompanied by tinnitus. Purpose: To observe the cochlear outer hair cells damagein malignancies patients treated with cisplatin. Methods: This study is an observational analytic studywith prospective design to determine the influence of high dose cisplatin on cochlear outer hair cellsfunction. The research was carried out at the ENT-HNS Department, Hasan Sadikin General HospitalBandung, from November 2007 until June 2008. Audiometry, tympanometry, and distortion productotoacoustic emission (DPOAE) examinations were conducted before chemotherapy and DPOAE, andtimpanometry was again measured three days after first and second cycles of cisplatin administration. McNemar test was performed to calculate the effects of high-dose cisplatin to the cochlear outer haircells function. To compare pre and post-cisplatin on alteration of cochlear hair cells function, Wilcoxontest was used. Results: In this study 60 ears from 30 subjects that meet the inclusion criteria, consistedof 25 man (83.3%) and 5 women (16.7%). The prevalence of damaged cochlear outer hair cells were63% at first cycle and 70% at second cycle of cisplatin administration. The decline of cochlear outerhair cells function was significant (p<0.001). Conclusion: High-dose cisplatin decreases cochlear outerhair cells function in patients with malignant neoplasm. Abstrak : Latar belakang: Kemoterapi sekarang rutin digunakan secara klinis di seluruh dunia. Sejalan denganhal tersebut toksisitas kemoterapi, khususnya terhadap telinga saat ini menjadi perhatian. Sisplatin(cis-diamminedichloroplatinum) adalah salah satu obat kemoterapi yang paling banyak digunakandan paling manjur untuk terapi keganasan epitelial. Efek ototoksik sisplatin yaitu terjadi gangguandengar sensorineural yang irreversible, progresif, bilateral pada frekuensi tinggi (4-8 kHz), dan disertaidengan tinitus. Tujuan: Untuk menilai penurunan fungsi sel rambut luar koklea pada penderita tumorganas sesudah pemberian sisplatin dosis tinggi dengan menggunakan DPOAE. Metode: Studi analitikobservasional dengan rancangan prospektif di Bagian IK. THT-KL RS. Hasan Sadikin Bandung mulaibulan November 2007 sampai dengan Juni 2008. Pada penelitian ini dilakukan pemeriksaan audiometrinada murni, timpanometri, dan distortion product otoacoustic emission (DPOAE) prakemoterapi, kemudianDPOAE dan timpanometri diulang tiga hari sesudah siklus pertama dan kedua kemoterapi sisplatin. Datayang diperoleh diuji dengan uji McNemar dan uji Wilcoxon. Hasil: Dari penelitian didapat 60 telingadari 30 subjek penelitian yang memenuhi kriteria inklusi yang terdiri dari 25 laki-laki (83,3%) dan 5perempuan (16,7%). Insidens penurunan fungsi sel rambut luar koklea sebesar 63% (38 kasus) sesudahsiklus pertama dan 70% (42 kasus) sesudah siklus kedua. Hubungan penurunan fungsi sel rambut luarkoklea memberikan nilai yang sangat bermakna sejak pemberian siklus pertama (p<0,001). Kesimpulan:Pemberian sisplatin dosis tinggi pada penderita tumor ganas menyebabkan penurunan fungsi sel rambutluar koklea.Kata kunci: kemoterapi, sisplatin dosis tinggi, sel rambut luar koklea.

scholarly journals Optimization of spectral-domain optical coherence tomography with a supercontinuum source for in vivo motion detection of low reflective outer hair cells in guinea pig cochleae

2021 ◽  
Author(s):  
Fumiaki Nin ◽  
Samuel Choi ◽  
Takeru Ota ◽  
Zhang Qi ◽  
Hiroshi Hibino
Keyword(s):  
Optical Coherence Tomography ◽  
High Resolution ◽  
Guinea Pig ◽  
Hair Cells ◽  
Sensory Epithelium ◽  
Outer Hair Cells ◽  
Acquisition Time ◽  
Total Acquisition Time ◽  
Sd Oct

AbstractSound evokes sub-nanoscale vibration within the sensory epithelium. The epithelium contains not only immotile cells but also contractile outer hair cells (OHCs) that actively shrink and elongate synchronously with the sound. However, the in vivo motion of OHCs has remained undetermined. The aim of this work is to perform high-resolution and -accuracy vibrometry in live guinea pigs with an SC-introduced spectral-domain optical coherence tomography system (SD-OCT). In this study, to reveal the effective contribution of SC source in the recording of the low reflective materials with the short total acquisition time, we compare the performances of the SC-introduced SD-OCT (SCSD-OCT) to that of the conventional SD-OCT. As inanimate comparison objects, we record a mirror, a piezo actuator, and glass windows. For the measurements in biological materials, we use in/ex vivo guinea pig cochleae. Our study achieved the optimization of a SD-OCT system for high-resolution in vivo vibrometry in the cochlear sensory epithelium, termed the organ of Corti, in mammalian cochlea. By introducing a supercontinuum (SC) light source and reducing the total acquisition time, we improve the axial resolution and overcome the difficulty in recording the low reflective material in the presence of biological noise. The high power of the SC source enables the system to achieve a spatial resolution of 1.72 ± 0.00 μm on a mirror and reducing the total acquisition time contributes to the high spatial accuracy of sub-nanoscale vibrometry. Our findings reveal the vibrations at the apical/basal region of OHCs and the extracellular matrix, basilar membrane.

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