Cochlear amplification, outer hair cells and prestin

Dallos, Peter

doi:10.1016/j.conb.2008.08.016

Cited by 239 publications

(221 citation statements)

References 75 publications

(82 reference statements)

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“…Thus, the base of the cochlea resonates at higher frequencies and the apex at lower frequencies. In addition, the magnitude of the organ of Corti vibration is amplified in a nonlinear fashion because of force production by outer hair cells [2]. This amplifies vibrations at low sound levels more than high sound levels.…”

Section: Introductionmentioning

confidence: 99%

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In vivo vibrometry inside the apex of the mouse cochlea using spectral domain optical coherence tomography

Gao

Raphael

Wang

et al. 2013

Biomed. Opt. Express

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Sound transduction within the auditory portion of the inner ear, the cochlea, is a complex nonlinear process. The study of cochlear mechanics in large rodents has provided important insights into cochlear function. However, technological and experimental limitations have restricted studies in mice due to their smaller cochlea. These challenges are important to overcome because of the wide variety of transgenic mouse strains with hearing loss mutations that are available for study. To accomplish this goal, we used spectral domain optical coherence tomography to visualize and measure sound-induced vibrations of intracochlear tissues. We present, to our knowledge, the first vibration measurements from the apex of an unopened mouse cochlea. propagation and reduced endocochlear potential associated with cochlear dysplasia in the BETA2/NeuroD1 null mouse," J. Assoc. Res. Otolaryngol. 8(4), 447-463 (2007). 22. C. C. Liu, S. S. Gao, T. Yuan, C. Steele, S. Puria, and J. S. Oghalai, "Biophysical mechanisms underlying outer hair cell loss associated with a shortened tectorial membrane," J.

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Section: Introductionmentioning

confidence: 99%

“…Because of the relatively long depth of focus, one downside to this approach is that round window membrane vibration may contaminate the measurements [12]. As a result, the consistency of these measurements has been questioned [2].…”

Section: Introductionmentioning

confidence: 99%

In vivo vibrometry inside the apex of the mouse cochlea using spectral domain optical coherence tomography

Gao

Raphael

Wang

et al. 2013

Biomed. Opt. Express

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“…The IHCs serve as sensory receptors, being innervated mostly by auditory afferents. The OHCs, with scattered afferent innervation, play a specific role in amplification of sounds based on their motility (4).…”

Section: Introductionmentioning

confidence: 99%

The LINC complex is essential for hearing

Horn¹,

Brownstein²,

Lenz³

et al. 2013

J. Clin. Invest.

123

168

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Hereditary hearing loss is the most common sensory deficit. We determined that progressive high-frequency hearing loss in 2 families of Iraqi Jewish ancestry was due to homozygosity for the protein truncating mutation SYNE4 c.228delAT. SYNE4, a gene not previously associated with hearing loss, encodes nesprin-4 (NESP4), an outer nuclear membrane (ONM) protein expressed in the hair cells of the inner ear. The truncated NESP4 encoded by the families' mutation did not localize to the ONM. NESP4 and SUN domain-containing protein 1 (SUN1), which localizes to the inner nuclear membrane (INM), are part of the linker of nucleoskeleton and cytoskeleton (LINC) complex in the nuclear envelope. Mice lacking either Nesp4 or Sun1 were evaluated for hair cell defects and hearing loss. In both Nesp4 -/-and Sun1 -/-mice, OHCs formed normally, but degenerated as hearing matured, leading to progressive hearing loss. The nuclei of OHCs from mutant mice failed to maintain their basal localization, potentially affecting cell motility and hence the response to sound. These results demonstrate that the LINC complex is essential for viability and normal morphology of OHCs and suggest that the position of the nucleus in sensory epithelial cells is critical for maintenance of normal hearing.

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“…As voltage sensors, OHCs emit frequency-specific responses with their structural motor proteins [17] and different motility responses with efferent stimulation [8] . This motor protein, prestin, is necessary for cochlear amplification and sharp frequency tuning [18,19] . Xia et al [13] used a noise exposure protocol to cause hair cell loss localized to the basal region of the cochlea, resulting in high-frequency hearing loss.…”

Section: Discussionmentioning

confidence: 99%

A New Hypothesis on the Frequency Discrimination of the Cochlea

Bulut

Uzun

Öztürk³

et al. 2017

Int Adv Otol

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OBJECTIVE:Medial olivocochlear efferent (MOCE) neurons innervate outer hair cells (OHCs) of the cochlea, which in turn leads to basilar membrane motion. We hypothesized that MOCE-induced alterations in basilar membrane motion, independent of traveling waves, is responsible for the cochlear frequency discrimination of sound. MATERIALS and METHODS:Eleven guinea pigs underwent bilateral otoscopic and audiologic evaluations under general anesthesia. The study comprised two parts. Part I (n=11) included spontaneous otoacoustic emission (SOAE) recordings with or without contralateral pure-tone acoustic stimuli (1 and 8 kHz) at 60 dB sound pressure level (SPL). Part II involved pure-tone (1 or 8 kHz) acoustic trauma in the right ears of two randomly selected subgroups (G1: 1 kHz; n=4 and G8: 8 kHz; n=4). The remaining three animals served as controls. After frequency-specific deafness was confirmed by distortion product otoacoustic emission (DPOAE), SOAEs were recorded in the left ears in the presence of a contralateral pure-tone (1 and 8 kHz) stimulus of 60 dB SPL. Furthermore, the surface of the organ of Corti was examined by scanning electron microscopy (SEM). RESULTS:The contralateral pure tone led to frequency-specific activation in SOAEs in part I (without trauma) and part II (with trauma) measurements. SEM showed heterogeneous OHC damage along the cochlea in traumatized ears with pure tone. CONCLUSION:We suggest that MOCEs convey acoustic information from traumatized ears to intact ears. Traumatized ears can show frequency-specific activation in the presence of diffuse damage in OHCs that excludes the passive transmission of the pressure wave from the perilymph to the basilar membrane.

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Cochlear amplification, outer hair cells and prestin

Cited by 239 publications

References 75 publications

In vivo vibrometry inside the apex of the mouse cochlea using spectral domain optical coherence tomography

In vivo vibrometry inside the apex of the mouse cochlea using spectral domain optical coherence tomography

The LINC complex is essential for hearing

A New Hypothesis on the Frequency Discrimination of the Cochlea

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