scholarly journals The Role of Prestin in the Generation of Electrically Evoked Otoacoustic Emissions in Mice

2008 ◽  
Vol 99 (4) ◽  
pp. 1607-1615 ◽  
Author(s):  
Markus Drexl ◽  
Marcia M. Mellado Lagarde ◽  
Jian Zuo ◽  
Andrei N. Lukashkin ◽  
Ian J. Russell
Keyword(s):  
Hair Cells ◽  
Otoacoustic Emissions ◽  
Temporal Structure ◽  
Organ Of Corti ◽  
Outer Hair Cells ◽  
Tectorial Membrane ◽  
Wild Type ◽  
Short Delay ◽  
Delay Component

Electrically evoked otoacoustic emissions are sounds emitted from the inner ear when alternating current is injected into the cochlea. Their temporal structure consists of short- and long-delay components and they have been attributed to the motile responses of the sensory-motor outer hair cells of the cochlea. The nature of these motile responses is unresolved and may depend on either somatic motility, hair bundle motility, or both. The short-delay component persists after almost complete elimination of outer hair cells. Outer hair cells are thus not the sole generators of electrically evoked otoacoustic emissions. We used prestin knockout mice, in which the motor protein prestin is absent from the lateral walls of outer hair cells, and Tecta ΔENT/ΔENT mice, in which the tectorial membrane, a structure with which the hair bundles of outer hair cells normally interact, is vestigial and completely detached from the organ of Corti. The amplitudes and delay spectra of electrically evoked otoacoustic emissions from Tecta ΔENT/ΔENT and Tecta +/+ mice are very similar. In comparison with prestin +/+ mice, however, the short-delay component of the emission in prestin −/− mice is dramatically reduced and the long-delay component is completely absent. Emissions are completely suppressed in wild-type and Tecta ΔENT/ΔENT mice at low stimulus levels, when prestin-based motility is blocked by salicylate. We conclude that near threshold, the emissions are generated by prestin-based somatic motility.

scholarly journals Amplification lags nonlinearity in the recovery from reduced endocochlear potential

2020 ◽  
Author(s):  
C. Elliott Strimbu ◽  
Yi Wang ◽  
Elizabeth S. Olson
Keyword(s):  
Hair Cells ◽  
Otoacoustic Emissions ◽  
Basilar Membrane ◽  
Frequency Tuning ◽  
Organ Of Corti ◽  
Endocochlear Potential ◽  
Outer Hair Cells ◽  
Frequency Resolution ◽  
Operating Condition ◽  
Distortion Product

ABSTRACTThe mammalian hearing organ, the cochlea, contains an active amplifier to boost the vibrational response to low level sounds. Hallmarks of this active process are sharp location-dependent frequency tuning and compressive nonlinearity over a wide stimulus range. The amplifier relies on outer hair cell (OHC) generated forces driven in part by the endocochlear potential (EP), the ~ +80 mV potential maintained in scala media, generated by the stria vascularis. We transiently eliminated the EP in vivo by an intravenous injection of furosemide and measured the vibrations of different layers in the cochlea’s organ of Corti using optical coherence tomography. Distortion product otoacoustic emissions (DPOAE) were monitored at the same times. Following the injection, the vibrations of the basilar membrane lost the best frequency (BF) peak and showed broad tuning similar to a passive cochlea. The intra-organ of Corti vibrations measured in the region of the OHCs lost their BF peak and showed low-pass responses, but retained nonlinearity, indicating that OHC electromotility was still operational. Thus, while electromotility is presumably necessary for amplification, its presence is not sufficient for amplification. The BF peak recovered nearly fully within 2 hours, along with a non-monotonic DPOAE recovery that suggests that physical shifts in operating condition are a final step in the recovery process.SIGNIFICANCEThe endocochlear potential, the +80 mV potential difference across the fluid filled compartments of the cochlea, is essential for normal mechanoelectrical transduction, which leads to receptor potentials in the sensory hair cells when they vibrate in response to sound. Intracochlear vibrations are boosted tremendously by an active nonlinear feedback process that endows the cochlea with its healthy sensitivity and frequency resolution. When the endocochlear potential was reduced by an injection of furosemide, the basilar membrane vibrations resembled those of a passive cochlea, with broad tuning and linear scaling. The vibrations in the region of the outer hair cells also lost the tuned peak, but retained nonlinearity at frequencies below the peak, and these sub-BF responses recovered fairly rapidly. Vibration responses at the peak recovered nearly fully over 2 hours. The staged vibration recovery and a similarly staged DPOAE recovery suggests that physical shifts in operating condition are a final step in the process of cochlear recovery.

Role of the Tectorial Membrane Revealed by Otoacoustic Emissions Recorded From Wild-Type and Transgenic TectaΔENT/ΔENT Mice

2004 ◽  
Vol 91 (1) ◽  
pp. 163-171 ◽  
Author(s):  
Andrei N. Lukashkin ◽  
Victoria A. Lukashkina ◽  
P. Kevin Legan ◽  
Guy P. Richardson ◽  
Ian J. Russell
Keyword(s):  
Otoacoustic Emissions ◽  
Low Frequency ◽  
Tectorial Membrane ◽  
Local Minima ◽  
Wild Type ◽  
Distortion Product ◽  
Growth Functions ◽  
In The Wild ◽  
Surrounding Fluid

Distortion product otoacoustic emissions (DPOAE) were recorded from wild-type mice and mutant TectaΔ ENT/Δ ENT mice with detached tectorial membranes (TM) under combined ketamine/xylaxine anesthesia. In TectaΔ ENT/Δ ENT mice, DPOAEs could be detected above the noise floor only when the levels of the primary tones exceeded 65 dB SPL. DPOAE amplitude decreased with increasing frequency of the primaries in TectaΔ ENT/Δ ENT mice. This was attributed to hair cell excitation via viscous coupling to the surrounding fluid and not by interaction with the TM as in the wild-type mice. Local minima and corresponding phase transitions in the DPOAE growth functions occurred at higher DPOAE levels in wild-type than in TectaΔ ENT/Δ ENT mice. In less-sensitive TectaΔ ENT/Δ ENT mice, the position of the local minima varied nonsystematically with frequency or no minima were observed. A bell-like dependence of the DPOAE amplitude on the ratio of the primaries was recorded in both wild-type and TectaΔ ENT/Δ ENT mice. However, the pattern of this dependence was different in the wild-type and TectaΔ ENT/Δ ENT mice, an indication that the bell-like shape of the DPOAE was produced by a combination of different mechanisms. A nonlinear low-frequency resonance, revealed by nonmonotonicity of the phase behavior, was seen in the wild-type but not in TectaΔ ENT/Δ ENT mice.

scholarly journals Elmod3 knockout leads to progressive hearing loss and abnormalities in cochlear hair cell stereocilia

2019 ◽  
Vol 28 (24) ◽  
pp. 4103-4112 ◽  
Author(s):  
Wu Li ◽  
Yong Feng ◽  
Anhai Chen ◽  
Taoxi Li ◽  
Sida Huang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Actin Cytoskeleton ◽  
Hearing Impairment ◽  
Hair Cells ◽  
Organ Of Corti ◽  
Vestibular Function ◽  
Outer Hair Cells ◽  
Auditory Function ◽  
Cochlear Hair Cells

Abstract ELMOD3, an ARL2 GTPase-activating protein, is implicated in causing hearing impairment in humans. However, the specific role of ELMOD3 in auditory function is still far from being elucidated. In the present study, we used the CRISPR/Cas9 technology to establish an Elmod3 knockout mice line in the C57BL/6 background (hereinafter referred to as Elmod3−/− mice) and investigated the role of Elmod3 in the cochlea and auditory function. Elmod3−/− mice started to exhibit hearing loss from 2 months of age, and the deafness progressed with aging, while the vestibular function of Elmod3−/− mice was normal. We also observed that Elmod3−/− mice showed thinning and receding hair cells in the organ of Corti and much lower expression of F-actin cytoskeleton in the cochlea compared with wild-type mice. The deafness associated with the mutation may be caused by cochlear hair cells dysfunction, which manifests with shortening and fusion of inner hair cells stereocilia and progressive degeneration of outer hair cells stereocilia. Our finding associates Elmod3 deficiencies with stereocilia dysmorphologies and reveals that they might play roles in the actin cytoskeleton dynamics in cochlear hair cells, and thus relate to hearing impairment.

Blebs in inner and outer hair cells: a pathophysiological hypothesis

2008 ◽  
Vol 122 (11) ◽  
pp. 1151-1155 ◽  
Author(s):  
R Ramírez-Camacho ◽  
J R García-Berrocal ◽  
A Trinidad ◽  
J M Verdaguer ◽  
J Nevado
Keyword(s):  
Hair Cells ◽  
Outer Hair Cell ◽  
Ganglion Cells ◽  
Stria Vascularis ◽  
Spiral Ganglion ◽  
Organ Of Corti ◽  
Outer Hair Cells ◽  
Inner Hair Cells ◽  
Hearing Thresholds

AbstractIntroduction:The ototoxic effects of cisplatin include loss of outer hair cells, degeneration of the stria vascularis and a decrease in the number of spiral ganglion cells. Scanning microscopy has shown balloon-like protrusions (blebs) of the plasma membrane of inner hair cells following cisplatin administration. The present study was undertaken to identify the possible role of inner and outer hair cell blebs in the pathogenesis of cisplatin-induced ototoxicity.Materials and methods:Twenty-five guinea pigs were injected with cisplatin and their hearing tested at different time-points, before sacrifice and examination with scanning electron microscopy.Results and analysis:Seven animals showed blebs in the inner hair cells at different stages. Hearing thresholds were lower in animals showing blebs.Discussion:Cisplatin seems to be able to induce changes in inner hair cells as well as in other structures in the organ of Corti. Blebbing observed in animals following cisplatin administration could play a specific role in the regulation of intracellular pressure.

scholarly journals Vanilloid Receptors in Hearing: Altered Cochlear Sensitivity by Vanilloids and Expression of TRPV1 in the Organ of Corti

2003 ◽  
Vol 90 (1) ◽  
pp. 444-455 ◽  
Author(s):  
Jiefu Zheng ◽  
Chunfu Dai ◽  
Peter S. Steyger ◽  
Youngki Kim ◽  
Zoltan Vass ◽  
...  
Keyword(s):  
Hair Cells ◽  
Otoacoustic Emissions ◽  
Transient Receptor Potential ◽  
Basilar Membrane ◽  
Organ Of Corti ◽  
Receptor Potential ◽  
Compound Action Potential ◽  
Outer Hair Cells ◽  
Vanilloid Receptors ◽  
Cochlear Blood Flow

Capsaicin, the vanilloid that selectively activates vanilloid receptors (VRs) on sensory neurons for noxious perception, has been reported to increase cochlear blood flow (CBF). VR-related receptors have also been found in the inner ear. This study aims to address the question as to whether VRs exist in the organ of Corti and play a role in cochlear physiology. Capsaicin or the more potent VR agonist, resiniferatoxin (RTX), was infused into the scala tympani of guinea pig cochlea, and their effects on cochlear sensitivity were investigated. Capsaicin (20 μM) elevated the threshold of auditory nerve compound action potential and reduced the magnitude of cochlear microphonic and electrically evoked otoacoustic emissions. These effects were reversible and could be blocked by a competitive antagonist, capsazepine. Application of 2 μM RTX resulted in cochlear sensitivity alterations similar to that by capsaicin, which could also be blocked by capsazepine. A desensitization phenomenon was observed in the case of prolonged perfusion with either capsaicin or RTX. Brief increase of CBF by capsaicin was confirmed, and the endocochlear potential was not decreased. Basilar membrane velocity (BM) growth functions near the best frequency and BM tuning were altered by capsaicin. Immunohistochemistry study revealed the presence of vanilloid receptor type 1 of the transient receptor potential channel family in the hair cells and supporting cells of the organ of Corti and the spiral ganglion cells of the cochlea. The results indicate that the main action of capsaicin is on outer hair cells and suggest that VRs in the cochlea play a role in cochlear homeostasis.

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.

Melatonin and other antioxidants prolong the postmortem activity of the outer hair cells of the organ of Corti: Its relation to the type of death

1999 ◽  
Vol 27 (2) ◽  
pp. 73-77 ◽  
Author(s):  
Miguel A. Lopez-Gonzalez ◽  
Juan M. Guerrero ◽  
Francisco Rojas ◽  
Carmen Osuna ◽  
Francisco Delgado
Keyword(s):  
Hair Cells ◽  
Organ Of Corti ◽  
Outer Hair Cells

High-Voltage Electron Microscopic Study of the Inner Ear: Technique and Preliminary Results

1983 ◽  
Vol 92 (1_suppl) ◽  
pp. 3-12 ◽  
Author(s):  
Tomonori Takasaka ◽  
Hideich Shinkawa ◽  
Kozo Watanuki ◽  
Sho Hashimoto ◽  
Kazutomo Kawamoto
Keyword(s):  
Electron Microscopy ◽  
Hair Cell ◽  
Inner Ear ◽  
High Voltage ◽  
Hair Cells ◽  
Outer Hair Cells ◽  
Tectorial Membrane ◽  
Preliminary Results ◽  
Voltage Electron ◽  
Cuticular Plate

The technique and some preliminary results of the application of high-voltage electron microscopy (HVEM) to the study of inner ear morphology in the guinea pig are reported in this paper. The main advantage of HVEM is that sharp images of thicker specimens can be obtained because of the greater penetrating power of high energy electrons. The optimum thickness of the sections examined with an accelerating voltage of 1,000 kV was found to be between 500 to 800 nm. The sections below 500 nm in thickness often had insufficient contrast, while those above 800 nm were rather difficult to interpret due to overlap of images of the organelles. The whole structure of the sensory hairs from the tip to the rootlet was more frequently observed in the 800-nm thick sections. Thus the fine details of the hair attachment to the tectorial membrane as well as the hair rootlet extension into the cuticular plate could be thoroughly studied in the HVEM. In specimens fixed in aldehyde containing 2% tannic acid, the attachment of the tips of the outer hair cell stereocilia to the tectorial membrane was observed. For the inner hair cells, however, the tips of the hairs were separated from the undersurface of the tectorial membrane. The majority of the rootlets of the outer hair cells terminated at the midportion of the cuticular plate, while most of the inner hair cell rootlets traversed the entire width of the cuticular plate and extended into the apical cytoplasm. These differences in ultrastructural appearance may indicate that the two kinds of hair cells play different roles in the acoustic transduction process. The three-dimensional arrangement of the nerve endings on the hair cells was also studied by the serial thick-sectioning technique in the HVEM. In general, an entire arrangement of the nerve endings was almost completely cut in less than ten 800-nm thick sections instead of the 50- to 100-ultrathin (ie, less than 100 nm) conventional sections for transmission electron microscopy. The present study confirms an earlier report that the first row outer hair cells in the third cochlear turn are innervated by nearly equal numbers of efferent and afferent endings, the average number being nine.

Innervation Densities of Inner and Outer Hair Cells of the Human Organ of Corti

ORL ◽  
1988 ◽  
Vol 50 (6) ◽  
pp. 363-370 ◽  
Author(s):  
Joseph B. Nadol, Jr.
Keyword(s):  
Hair Cells ◽  
Organ Of Corti ◽  
Outer Hair Cells ◽  
Human Organ

scholarly journals A detection method for lactic dehydrogenase activity in the inner ear.

1978 ◽  
Vol 26 (4) ◽  
pp. 313-317 ◽  
Author(s):  
T Omata ◽  
I Ohtani ◽  
K Ohtsuki ◽  
J Ouchi
Keyword(s):  
Hair Cells ◽  
Osmium Tetroxide ◽  
Light And Electron Microscopy ◽  
Organ Of Corti ◽  
Lactic Dehydrogenase ◽  
Outer Hair Cells ◽  
Water Soluble ◽  
Reaction Products ◽  
Frozen Sections ◽  
Membranous Labyrinth

A method for the detection of lactic dehydrogenase enzymatic activity in outer hair cells of the rabbit is described. The membranous labyrinth with temporal bone was prefixed in glutaraldehyde. After being placed into the incubation medium, it was postfixed in osmium tetroxide. Specimens of the organ of Corti were removed. Then the specimens were embedded in water-soluble glycol and cut with a cryostat for light microscopy, and also they were embedded in Epon and cut for light and electron microscopy. Sectioning of the membranous labyrinth was very easily made when the specimens were embedded in both the water-soluble glycol and the Epon. The structures of the frozen sections as well as the Epon-embedded ones were well preserved. In the frozen sections the preservation and localization of reaction products were thoroughly kept, but monoformazan of the Epon-embedded sections was soluble.

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