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1
Munnamalai, Vidhya, Nabilah H. Sammudin, Caryl A. Young, Ankita Thawani, Richard J. Kuhn, and Donna M. Fekete. "Embryonic and Neonatal Mouse Cochleae Are Susceptible to Zika Virus Infection." Viruses 13, no. 9 (September 14, 2021): 1823. http://dx.doi.org/10.3390/v13091823.
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Congenital Zika Syndrome (CZS) is caused by vertical transmission of Zika virus (ZIKV) to the gestating human fetus. A subset of CZS microcephalic infants present with reduced otoacoustic emissions; this test screens for hearing loss originating in the cochlea. This observation leads to the question of whether mammalian cochlear tissues are susceptible to infection by ZIKV during development. To address this question using a mouse model, the sensory cochlea was explanted at proliferative, newly post-mitotic or maturing stages. ZIKV was added for the first 24 h and organs cultured for up to 6 days to allow for cell differentiation. Results showed that ZIKV can robustly infect proliferating sensory progenitors, as well as post-mitotic hair cells and supporting cells. Virus neutralization using ZIKV-117 antibody blocked cochlear infection. AXL is a cell surface molecule known to enhance the attachment of flavivirus to host cells. While Axl mRNA is widely expressed in embryonic cochlear tissues susceptible to ZIKV infection, it is selectively downregulated in the post-mitotic sensory organ by E15.5, even though these cells remain infectible. These findings may offer insights into which target cells could potentially contribute to hearing loss resulting from fetal exposure to ZIKV in humans.
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Kim, Sungwook, Ruchire Wijesinghe, Jaeyul Lee, Muhammad Shirazi, Pilun Kim, Jeong Jang, Mansik Jeon, and Jeehyun Kim. "Multiple Wavelength Optical Coherence Tomography Assessments for Enhanced Ex Vivo Intra-Cochlear Microstructural Visualization." Electronics 7, no. 8 (July 31, 2018): 133. http://dx.doi.org/10.3390/electronics7080133.
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The precise identification of intra-cochlear microstructures is an essential otorhinolaryngological requirement to diagnose the progression of cochlea related diseases. Thus, we demonstrated an experimental procedure to investigate the most optimal wavelength range, which can enhance the visualization of ex vivo intra-cochlear microstructures using multiple wavelengths (i.e., 860 nm, 1060 nm, and 1300 nm) based optical coherence tomography (OCT) systems. The high-resolution tomograms, volumetric, and quantitative evaluations obtained from Basilar membrane, organ of Corti, and scala vestibule regions revealed complementary comparisons between the aforementioned three distinct wavelengths based OCT systems. Compared to 860 nm and 1300 nm wavelengths, 1060 nm wavelength OCT was discovered to be an appropriate wavelength range verifying the simultaneously obtainable high-resolution and reasonable depth range visualization of intra-cochlear microstructures. Therefore, the implementation of 1060 nm OCT can minimize the necessity of two distinct OCT systems. Moreover, the results suggest that the performed qualitative and quantitative analysis procedure can be used as a powerful tool to explore further anatomical structures of the cochlea for future studies in otorhinolaryngology.
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Sekiya, Tetsuji, Ken Kojima, Masahiro Matsumoto, Matthew C. Holley, and Juichi Ito. "REBUILDING LOST HEARING USING CELL TRANSPLANTATION." Neurosurgery 60, no. 3 (March 1, 2007): 417–33. http://dx.doi.org/10.1227/01.neu.0000249189.46033.42.
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Abstract OBJECTIVE The peripheral auditory nervous system (cochlea and auditory nerve) has a complex anatomy, and it has traditionally been thought that once the sensorineural structures are damaged, restoration of hearing is impossible. In the past decade, however, the potential to restore lost hearing has been intensively investigated using molecular and cell biological techniques, and we can now part with such a pessimistic view. In this review, we examine an important field in hearing restoration research: cell transplantation. METHODS Most efforts in this field have been directed to the replacement of hair cells by transplantation to the cochlea. Here, we focus on transplantation to the auditory nerve, from the side of the cerebellopontine angle rather than the cochlea. RESULTS Delivery of cells to the cochlea is potentially damaging, and nerve cells transplanted distally to the Schwann-glial transitional zone (cochlear side) may become inhibited when they reach the transitional zone. The auditory nerve is probably the most suitable route for cell transplantation. CONCLUSION The auditory nerve occupies an important position not only in neurosurgery but also in various diseases in other disciplines, and several lines of recent evidence indicate that it is a key target for hearing restoration. It is familiar to most neurosurgeons, and the recent advances in the molecular and cell biology of inner-ear development are of direct importance to neurorestorative medicine. In this article, we review the anatomy, development, and molecular biology of the auditory nerve and cochlea, with emphasis on the advances in cell transplantation.
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Jackler, Robert K., and William P. Dillon. "Computed Tomography and Magnetic Resonance Imaging of the Inner Ear." Otolaryngology–Head and Neck Surgery 99, no. 5 (November 1988): 494–504. http://dx.doi.org/10.1177/019459988809900508.
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The majority of temporal bone radiographic studies are obtained either for middle ear and mastoid disease or in the evaluation of retrocochlear pathology. With recent technologic advances, diagnostic imaging of the inner ear has developed an increasing role in the evaluation and management of diseases that affect the cochlea, semicircular canals, and the vestibular and cochlear aqueducts. High-resolution computed tomography (CT) provides excellent detail of the osseous labyrinth, whereas magnetic resonance imaging (MRI) generates images derived from the membranous labyrinth and its associated neural elements. Optimal techniques for obtaining high quality CT and MRI images of the normal and diseased inner ear are presented. CT has proved useful in the evaluation of inner ear malformations, cochlear otosclerosis, labyrinthine fistulization from cholesteatoma, translabyrinthine fractures, otic capsule osteodystrophies, in the assessment of cochlear patency before cochlear implantation, and in the localization of prosthetic devices such as stapes wires and cochlear implants. While MRI produces discernible images of the soft tissue and fluid components of the inner ear, it has yet to demonstrate any unique advantages in the evaluation of inner ear disease. However, MRI produces excellent and highly useful images of the audiovestibular and facial nerves, cerebellopontine angle, and brain.
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Guo, Weiwei, Haijin Yi, Zhang Yan, Lili Ren, Lei Chen, Li Dong Zhao, Yu Ning, David Z. Z. He, and Shi-Ming Yang. "The morphological and functional development of the stria vascularis in miniature pigs." Reproduction, Fertility and Development 29, no. 3 (2017): 585. http://dx.doi.org/10.1071/rd15183.
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The purpose of this study was to examine the morphological and functional development of the lateral wall of the scala media of the cochlea in miniature pigs; light and transmission electron microscopy and electrophysiology were used for this purpose. We showed that the lateral wall of the scala media of the cochlea appears at embryonic Day 21 (E21) when the cochlear duct begins to form. From E28 to E49, the lateral wall can be distinguished according to its position along the cochlea. At E56, cells in the lateral wall begin to differentiate into three different types. At E70, three cell types, marginal, intermediate and basal, can be clearly distinguished. At E91, the stria vascularis is adult-like and the organ of Corti is also morphologically mature. The average endocochlear potential measured from the second turn of the cochlea (at E98, postnatal Day 1 (P1), P13 and P30) was 71.4 ± 2.5 (n = 7), 78.8 ± 1.5 (n = 10), 77.3 ± 2.3 (n = 10) and 78.0 ± 2.1 mV (n = 10), respectively. Our results suggest that in miniature pigs the stria vascularis develops during the embryonic period, concurrent with maturation of the organ of Corti. The magnitude of the endocochlear potential reached its mature level when the stria vascularis was morphologically adult-like at E98. These findings provide a morphological and functional basis for future animal studies using the miniature pig model concerning the pathogenesis of various inner-ear diseases.
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Engel, Frank, Rosemarie Blatz, Reinhard Schliebs, Michael Palmer, and Sucharit Bhakdi. "Bacterial Cytolysin Perturbs Round Window Membrane Permeability Barrier In Vivo: Possible Cause of Sensorineural Hearing Loss in Acute Otitis Media." Infection and Immunity 66, no. 1 (January 1, 1998): 343–46. http://dx.doi.org/10.1128/iai.66.1.343-346.1998.
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ABSTRACT The passage of radioiodinated streptolysin-O (SLO) and albumin through the round window membrane (RWM) was studied in vivo. When applied to the middle ear, SLO became quantitatively entrapped in this compartment and no passage to the cochlea occurred. However, flux of radioiodinated albumin through the toxin-damaged RWM was observed. We propose that the passage of noxious macromolecules, such as proteases, from a purulent middle-ear effusion may be facilitated by pore-forming toxins, resulting in cochlear damage and sensorineural hearing loss.
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Sillman, Jonathon S., Michael J. Larouere, Alfred L. Nuttall, Merle Lawrence, and Josef M. Miller. "Recent Advances in Cochlear Blood Flow Measurements." Annals of Otology, Rhinology & Laryngology 97, no. 1 (January 1988): 1–8. http://dx.doi.org/10.1177/000348948809700101.
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Changes in blood flow to the inner ear have been thought to influence or underlie a number of cochlear diseases, including some forms of noise-induced hearing loss, sudden hearing loss, and Meniere's disease. Recently, important advances have been made in two technologies for the study of cochlear blood flow. The first is in the area of vital microscopic studies of cochlear microcirculation, and the second is based on the introduction of laser technology in the form of laser Doppler flowmetry. In this report, measurements are given of changes in cochlear circulation caused by carbon dioxide breathing, intravenous phenylephrine injection, systemic hemodilution, positive end expiratory pressure, and direct electrical stimulation of the cochlea. From these changes, we observe that cochlear blood circulation responds to systemic blood pressure alterations and is subject to local flow control mechanisms. Linearity and speed of response of the laser Doppler instrumentation also are shown. These advances show promise for contributing to our knowledge of control mechanisms of inner ear blood flow and for revealing the influence of various pharmacologic agents of potential clinical value.
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Su, Zhongwu, Hao Xiong, Jiaqi Pang, Hanqing Lin, Lan Lai, Huasong Zhang, Weijian Zhang, and Yiqing Zheng. "LncRNA AW112010 Promotes Mitochondrial Biogenesis and Hair Cell Survival: Implications for Age-Related Hearing Loss." Oxidative Medicine and Cellular Longevity 2019 (October 27, 2019): 1–13. http://dx.doi.org/10.1155/2019/6150148.
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Long noncoding RNA (lncRNA) disorder has been found in many kinds of age-associated diseases. However, the role of lncRNA in the development of age-related hearing loss (AHL) is still largely unknown. This study sought to uncover AHL-associated lncRNAs and the function. RNA-sequencing was conducted to profile lncRNA expression in the cochlea of an early-onset AHL mouse model. RT-qPCR assay was used to validate the expression pattern of lncRNAs. ATP assay, JC-1 assay, mitochondrial probe staining, CCK-8 assay, Western blot, and immunocytochemistry were performed to detect the effects of lncRNA AW112010 in HEI-OC1 cells and the mouse cochlea. We identified 88 significantly upregulated lncRNAs and 46 significantly downregulated lncRNAs in the cochlea of aged C57BL/6 mice. We focused on the significantly upregulated AW112010. Silencing of AW112010 decreased the ATP level, mitochondrial membrane potential, and cell viability and increased mitochondrial ROS generation under oxidative stress in HEI-OC1 cells. AW112010 overexpression promoted cell survival in HEI-OC1 cells. AW112010 knockdown reduced mitochondrial mass and impaired mitochondrial biogenesis in HEI-OC1 cells. Activation of mitochondrial biogenesis by resveratrol and STR1720 promoted cell survival. The mitochondrial biogenesis process was activated in the cochlea of aged mice. Moreover, AW112010 regulated AMPK signaling in HEI-OC1 cells. Transcription factor Arid5b elevated in the aged cochlea and induced AW112010 expression and mitochondrial biogenesis in HEI-OC1 cells. Taken together, lncRNAs are dysregulated with aging in the cochlea of C57BL/6 mice. The Arid5b/AW112010 signaling was induced in the aged mouse cochlea and positively modulated the mitochondrial biogenesis to maintain mitochondrial function.
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Meli, Damian N., Roney S. Coimbra, Dominik G. Erhart, Gerard Loquet, Caroline L. Bellac, Martin G. Täuber, Ulf Neumann, and Stephen L. Leib. "Doxycycline Reduces Mortality and Injury to the Brain and Cochlea in Experimental Pneumococcal Meningitis." Infection and Immunity 74, no. 7 (July 2006): 3890–96. http://dx.doi.org/10.1128/iai.01949-05.
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ABSTRACT Bacterial meningitis is characterized by an inflammatory reaction to the invading pathogens that can ultimately lead to sensorineural hearing loss, permanent brain injury, or death. The matrix metalloproteinases (MMPs) and tumor necrosis factor alpha-converting enzyme (TACE) are key mediators that promote inflammation, blood-brain barrier disruption, and brain injury in bacterial meningitis. Doxycycline is a clinically used antibiotic with anti-inflammatory effects that lead to reduced cytokine release and the inhibition of MMPs. Here, doxycycline inhibited TACE with a 50% inhibitory dose of 74 μM in vitro and reduced the amount of tumor necrosis factor alpha released into the cerebrospinal fluid by 90% in vivo. In an infant rat model of pneumococcal meningitis, a single dose of doxycycline (30 mg/kg) given as adjuvant therapy in addition to ceftriaxone 18 h after infection significantly reduced the mortality, the blood-brain barrier disruption, and the extent of cortical brain injury. Adjuvant doxycycline (30 mg/kg given subcutaneously once daily for 4 days) also attenuated hearing loss, as assessed by auditory brainstem response audiometry, and neuronal death in the cochlear spiral ganglion at 3 weeks after infection. Thus, doxycycline, probably as a result of its anti-inflammatory properties, had broad beneficial effects in the brain and the cochlea and improved survival in this model of pneumococcal meningitis in infant rats.
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El Ganzoury, Mona M., Terez B. Kamel, Lobna H. Khalil, and A. M. Seliem. "Cochlear Dysfunction in Children following Cardiac Bypass Surgery." ISRN Pediatrics 2012 (July 1, 2012): 1–6. http://dx.doi.org/10.5402/2012/375038.
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Background. Sensorineural hearing loss after procedures including extracorporeal circulation and hypothermia is greater than general population. Mild hypothermia has a protective role on cochlea; however, deep hypothermia may result in cochlear injury. This research aimed at assessing auditory function in children after open heart surgery in relation to different hypothermic techniques. Subjects and Methods. Forty children with acyanotic heart diseases who underwent open heart surgery were included: group I: twenty patients subjected to mild hypothermia (33° to 37°C), group II: twenty patients subjected to moderate hypothermia (28° to 32°C). Audiological assessment included basic evaluation and otoacoustic emissions. Results. Both groups had distortion-product otoacoustic emissions (DPOAEs) amplitude >3 dB SPL at all frequencies. However, group II showed lower amplitude at overall and at high frequencies (4.416–8.837 KHz) than group I. Transient evoked otoacoustic emissions (TEOAEs) showed partial pass in three patients of group I (15%) and in 15 patients of group II (75%). Moreover, group II showed statistical significant reduction in overall TEOAEs amplitude as well as at high frequencies (2–4 KHz). Conclusions. Patients exposed to moderate hypothermic technique had subtle cochlear dysfunction. Otoacoustic emissions should be used for early detection of subtle cochlear dysfunction in operated cardiac children.
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Alqudah, Safa, Mark Chertoff, Dianne Durham, Jackob Moskovitz, Hinrich Staecker, and Marcello Peppi. "Methionine Sulfoxide Reductase A Knockout Mice Show Progressive Hearing Loss and Sensitivity to Acoustic Trauma." Audiology and Neurotology 23, no. 1 (2018): 20–31. http://dx.doi.org/10.1159/000488276.
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Methionine sulfoxide reductases (MsrA and MsrB) protect the biological activity of proteins from oxidative modifications to methionine residues and are important for protecting against the pathological effects of neurodegenerative diseases. In the current study, we characterized the auditory phenotype of the MsrA knockout mouse. Young MsrA knockout mice showed small high-frequency threshold elevations for auditory brainstem response and distortion product otoacoustic emission compared to those of wild-type mice, which progressively worsened in older MsrA knockout mice. MsrA knockout mice showed an increased sensitivity to noise at young and older ages, suggesting that MsrA is part of a mechanism that protects the cochlea from acoustic damage. MsrA mRNA in the cochlea was increased following acoustic stimulation. Finally, expression of mRNA MsrB1 was compromised at 6 months old, but not in younger MsrA knockout mice (compared to controls). The identification of MsrA in the cochlea as a protective mediator from both early onset hearing loss and acoustic trauma expands our understanding of the pathways that may induce protection from acoustic trauma and foster further studies on how to prevent the damaging effect of noise exposure through Msr-based therapy.
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Fishman, Andrew J., Rau Christoph, Fan Lixin, and Claus-Peter Richter. "Cochlear Soft Tissue Imaging Using In-Line Phase Contrast." Otolaryngology–Head and Neck Surgery 139, no. 2_suppl (August 2008): P100. http://dx.doi.org/10.1016/j.otohns.2008.05.520.
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Problem Most contemporary imaging methods require invasive specimen preparation and/or are time consuming, or lack sufficient spatial resolution. Methods Experiments were made at the Advanced Photon Source (APS), Argonne National Laboratory. The APS is a synchrotron radiation source of the third generation, for which the particular characteristic is the highly coherent X-ray radiation. X-rays are generated with an undulator, inserted in a straight section of the storage ring. Images taken with hard X-rays and the grating interferometer were compared with images from the same specimen acquired with light microscopy. Results The results show that imaging of cochlear soft tissue structures is possible with hard X-rays and a grating interferometer. Conclusion Imaging of cochlear soft tissue structures is possible with hard X-rays and a grating interferometer. Significance It is important to examine cochlear morphology from many points of view, including comparative anatomy, cochlear developmental changes, malformation caused by genetic defects, changes related to diseases, sensory physiology, and cochlear modeling. Best imaging results were obtained from in situ experiments in the absence of tissue distortion. However, most contemporary imaging methods require invasive specimen preparation and/or are time consuming, or lack sufficient spatial resolution. A method that potentially overcomes these shortcomings is presented in this paper and it uses hard X-rays and a grating interferometer. Furthermore, hard X-rays would allow imaging cochlear soft tissue structures without opening the cochlea itself. Support The UNICAT facility at the Advanced Photon Source is supported by the U.S. DOE under Award No. DEFG02-91ER45439, through the Frederick Seitz Materials Research Laboratory at the University of Illinois at Urbana-Champaign, the ORNL (U.S. DOE contract DE-AC.
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Oh, Se-Joon, Il-Woo Lee, Soo-Geun Wang, Soo-Keun Kong, Hong-Ki Kim, and Eui-Kyung Goh. "Extratympanic Observation of Middle and Inner Ear Structures in Rodents Using Optical Coherence Tomography." Clinical and Experimental Otorhinolaryngology 13, no. 2 (May 1, 2020): 106–12. http://dx.doi.org/10.21053/ceo.2019.00766.
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Objectives. This study aimed to investigate whether optical coherence tomography (OCT) provides useful information about the microstructures of the middle and inner ear via extratympanic approach and thereby could be utilized as an alternative diagnostic technology in ear imaging.Methods. Five rats and mice were included, and the swept-source OCT system was applied to confirm the extent of visibility of the middle and inner ear and measure the length or thickness of the microstructures in the ear. The cochlea was subsequently dissected following OCT and histologically evaluated to compare with the OCT images.Results. The middle ear microstructures such as ossicles, stapedial artery and oval window through the tympanic membrane with the OCT could be confirmed in both rats and mice. It was also possible to obtain the inner ear images such as each compartment of the cochlea in the mice, but the bone covering bulla needed to be removed to visualize the inner ear structures in the rats which had thicker bulla. The bony thickness covering the cochlea could be measured, which showed no significant differences between OCT and histologic image at all turns of cochlea.Conclusion. OCT has been shown a promising technology to assess real-time middle and inner ear microstructures noninvasively with a high-resolution in the animal model. Therefore, OCT could be utilized to provide additional diagnostic information about the diseases of the middle and inner ear.
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Sugiura, M., S. Naganawa, E. Sato, and T. Nakashima. "Visualization of a high protein concentration in the cochlea of a patient with a large endolymphatic duct and sac, using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging." Journal of Laryngology & Otology 120, no. 12 (September 25, 2006): 1084–86. http://dx.doi.org/10.1017/s0022215106003331.
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Objective: To evaluate the inner ear of a patient with an enlarged vestibular aqueduct and acute hearing deterioration, using three-dimensional fluid-attenuated inversion recovery (3D FLAIR) magnetic resonance imaging (MRI).Background: Three-dimensional fluid-attenuated inversion recovery MRI has been recently developed to detect haemorrhage and high concentrations of protein. Application of this method to inner-ear diseases has not been fully described.Method: We used 3D FLAIR MRI to evaluate a patient with an enlarged vestibular aqueduct and acute hearing deterioration.Results: On 3D FLAIR MRI, this patient had high signals in the endolymphatic duct and sac, the vestibule and the cochlea and was diagnosed as having a high protein concentration in the inner ear. These high signal areas were not detected by T1- and T2-weighted MRI.Conclusion: This is the first published case in which a high protein concentration in the cochlea of a patient with an enlarged vestibular aqueduct was detected by 3D FLAIR MRI. These findings strongly supported the theory of the pathophysiology of hearing deterioration in patients with an enlarged vestibular aqueduct, which states that hyperosmolar fluid in the enlarged endolymphatic sac refluxes into the cochlea, resulting in damage to the hair cells. Thus, these finding may help clarify the cause of hearing deterioration in the presence of a large endolymphatic duct and sac.
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Selvanayagam, Dyana Lekha, and Amuthaganesh Mathialagan. "A review on hearing loss caused by occupational hazard and trauma." International Journal Of Community Medicine And Public Health 8, no. 8 (July 27, 2021): 4116. http://dx.doi.org/10.18203/2394-6040.ijcmph20213051.
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Occupational hearing loss (OHL) is known to be one of the most common occupational diseases. Common causes are being exposed to workplace hazardous noise levels, ototoxic chemicals, head injuries and barotrauma. Occupational noise-induced hearing loss is mostly due to the death or damage of hair cells in our ear, which do not grow back. On the other hand, ototoxic chemicals affect the structures in cochlear and auditory neurological pathways leading to hearing loss. In occupational head trauma, the parts of the ear that are damaged are the tympanic membrane, middle ear and cochlea which leads to hearing disability. Huge changes in pressure on the outside and the inside of the ear leads to barotrauma causing potential hearing loss. Many of the research available are regarding noise-induced OHL or chemicals causing OHL followed by barotrauma. More studies on OHL due to workplace head trauma should be conducted. Preventative measures should be taken by the worker’s supervisors or the government to implement safety measures to prevent and reduce OHL. This can be done via providing equipment and facilities that prevent hearing loss and education on OHL.
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Huang, Qiuhong, Hao Xiong, Haidi Yang, Yongkang Ou, Zhigang Zhang, Suijun Chen, Yongyi Ye, and Yiqing Zheng. "Differential Expression of Bcl-2 in the Cochlea and Auditory Cortex of a Mouse Model of Age-Related Hearing Loss." Audiology and Neurotology 21, no. 5 (2016): 326–32. http://dx.doi.org/10.1159/000450937.
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Bcl-2, the first gene shown to be involved in apoptosis, is a potent regulator of cell survival and known to have protective effects against a variety of age-related diseases. However, the possible relationship between hearing and Bcl-2 expression in the cochlea or auditory cortex of C57BL/6 mice, a mouse model of age-related hearing loss, is still unknown. Using RT-PCR, immunohistochemistry, and Western blot analysis, our results show that Bcl-2 is strongly expressed in the inner hair cells and spiral ganglion neurons of young mice. In addition, moderate Bcl-2 expression is also detected in the outer hair cells and in the neurons of the auditory cortex. A significant reduction of Bcl-2 expression in the cochlea or auditory cortex is also associated with elevated hearing thresholds and hair cell loss during aging. The expression pattern of Bcl-2 in the peripheral and central auditory systems suggests that Bcl-2 may play an important role in auditory function serving as a protective molecule against age-related hearing loss.
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Ahn, K. S., S. J. Jeon, J. Y. Jung, T. Choi, S. J. Choi, and H. Shim. "277 ESTABLISHMENT OF EMBRYONIC STEM CELL LINES DERIVED FROM A EGFP-TRANSGENIC MOUSE AND THEIR SURVIVAL IN THE COCHLEA OF C57BL/6 MICE." Reproduction, Fertility and Development 20, no. 1 (2008): 218. http://dx.doi.org/10.1071/rdv20n1ab277.
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Embryonic stem (ES) cells isolated from inner cell mass cells of blastocyst-stage embryos are capable of differentiating into various cell lineages. Transplantation of these cells may potentially be a treatment for many degenerative diseases. Such cell therapy has often been tested using allografts of ES cells in mice. However, it has been difficult to locate transplanted ES cells and to avoid the rejection of allogeneic ES cells by the host. The aims of this study were to establish ES cell lines ubiquitously expressing enhanced green fluorescent protein (EGFP) and to test survival of ES cells in allografts into the cochlea of inbred C57BL/6 mice. Nine hatched blastocysts collected from a C57BL/6-green mouse that ubiquitously expresses transgene EGFP were plated onto an inactivated STO feeder layer. Two putative ES-like colonies were obtained from the plated blastocysts, and repeated subculture of these colonies produced two cell lines expressing EGFP. The cell lines possessed typical characteristics of ES cells, including densely packed colonies of the cells with prominent nucleoli, a high nuclear-cytoplasmic ratio, and high alkaline phosphatase activity. In suspension culture, these cells formed simple and cystic embryoid bodies. Undifferentiated EGFP-transgenic ES cells (106 cells per mouse) were injected into the cochlea of five C57BL/6 mice deafened by gentamycin treatment. Although no behavioral changes were noticed until four weeks after the transplantation, histological study revealed that grafted cells survived in the scala media of all injected mice. Incorporation of the cells expressing EGFP into the host was found along the auditory nerve fibers close to the organ of Corti. Such incorporation was also discovered in the area of the spiral ganglion neurons, cochlear sensory epithelia, and stria vascularis. Morphology and size of the cells varied depending on their sites of incorporation. The results from the present study demonstrate that, due to their survival in transplantation without allogeneic rejection as well as ubiquitous and stable expression of EGFP, ES cells from an EGFP-transgenic mouse may be a useful means of studying cell therapy.
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Sheue, Er Wang. "Physiological and Histological Evaluations of the Cochlea between 3xTg-AD Mouse Model of Alzheimer's Diseases and R6/2 Mouse Model of Huntington's Diseases." Chinese Journal of Physiology 58, no. 6 (December 31, 2015): 359–66. http://dx.doi.org/10.4077/cjp.2015.bad334.
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Lin, Yi-Chun, Yuan-Yung Lin, Hsin-Chien Chen, Chao-Yin Kuo, Ai-Ho Liao, Ying-Liang Chou, Chia-Lien Hung, Cheng-Ping Shih, and Chih-Hung Wang. "Ultrasound Microbubbles Enhance the Efficacy of Insulin-Like Growth Factor-1 Therapy for the Treatment of Noise-Induced Hearing Loss." Molecules 26, no. 12 (June 13, 2021): 3626. http://dx.doi.org/10.3390/molecules26123626.
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The application of insulin-like growth factor 1 (IGF-1) to the round window membrane (RWM) is an emerging treatment for inner ear diseases. RWM permeability is the key factor for efficient IGF-1 delivery. Ultrasound microbubbles (USMBs) can increase drug permeation through the RWM. In the present study, the enhancing effect of USMBs on the efficacy of IGF-1 application and the treatment effect of USMB-mediated IGF-1 delivery for noise-induced hearing loss (NIHL) were investigated. Forty-seven guinea pigs were assigned to three groups: the USM group, which received local application of recombinant human IGF-1 (rhIGF-1, 10 µg/µL) following application of USMBs to the RWM; the RWS group, which received IGF-1 application alone; and the saline-treated group. The perilymphatic concentration of rhIGF-1 in the USM group was 1.95- and 1.67- fold of that in the RWS group, 2 and 24 h after treatment, respectively. After 5 h of 118 dB SPL noise exposure, the USM group had the lowest threshold shift in auditory brainstem response, least loss of cochlear outer hair cells, and least reduction in the number of synaptic ribbons on postexposure day 28 among the three groups. The combination of USMB and IGF-1 led to a better therapeutic response to NIHL. Two hours after treatment, the USM group had significantly higher levels of Akt1 and Mapk3 gene expression than the other two groups. The most intense immunostaining for phosphor-AKT and phospho-ERK1/2 was detected in the cochlea in the USM group. These results suggested that USMB can be applied to enhance the efficacy of IGF-1 therapy in the treatment of inner ear diseases.
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Watanabe, Ken-ichi, Ken Jinnouchi, Alexander Hess, Olaf Michel, Shunkichi Baba, and Toshiaki Yagi. "Carboplatin Induces Less Apoptosis in the Cochlea of Guinea Pigs than Cisplatin." Chemotherapy 48, no. 2 (2002): 82–87. http://dx.doi.org/10.1159/000057667.
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Zhang, Yanzhuo, Zhe Lv, Yudong Liu, Huan Cao, Jianwang Yang, and Baoshan Wang. "PIN1 Protects Hair Cells and Auditory HEI-OC1 Cells against Senescence by Inhibiting the PI3K/Akt/mTOR Pathway." Oxidative Medicine and Cellular Longevity 2021 (June 2, 2021): 1–17. http://dx.doi.org/10.1155/2021/9980444.
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A growing amount of evidence has confirmed the crucial role of the prolyl isomerase PIN1 in aging and age-related diseases. However, the mechanism of PIN1 in age-related hearing loss (ARHL) remains unclear. Pathologically, ARHL is primarily due to the loss and dysfunction of hair cells (HCs) and spiral ganglion cells (SGCs) in the cochlea. Therefore, in this study, we aimed to investigate the role of PIN1 in protecting hair cells and auditory HEI-OC1 cells from senescence. Enzyme-linked immunosorbent assays, immunohistochemistry, and immunofluorescence were used to detect the PIN1 protein level in the serum of ARHL patients and C57BL/6 mice in different groups, and in the SGCs and HCs of young and aged C57BL/6 mice. In addition, a model of HEI-OC1 cell senescence induced by H2O2 was used. Adult C57BL/6 mice were treated with juglone, or juglone and NAC, for 4 weeks. Interestingly, we found that the PIN1 protein expression decreased in the serum of patients with ARHL, in senescent HEI-OC1 cells, and in the cochlea of aged mice. Moreover, under H2O2 and juglone treatment, a large amount of ROS was produced, and phosphorylation of p53 was induced. Importantly, PIN1 expression was significantly increased by treatment with the p53 inhibitor pifithrin-α. Overexpression of PIN1 reversed the increased level of p-p53 and rescued HEI-OC1 cells from senescence. Furthermore, PIN1 mediated cellular senescence by the PI3K/Akt/mTOR signaling pathway. In vivo data from C57BL/6 mice showed that treatment with juglone led to hearing loss. Taken together, these findings demonstrated that PIN1 may act as a vital modulator in hair cell and HEI-OC1 cell senescence.
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Yu, Lanying, Tiantian Liu, Shuang Fu, Li Li, Xiaoping Meng, Xin Su, Zhanfeng Xie, et al. "Physiological functions of urea transporter B." Pflügers Archiv - European Journal of Physiology 471, no. 11-12 (November 22, 2019): 1359–68. http://dx.doi.org/10.1007/s00424-019-02323-x.
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AbstractUrea transporters (UTs) are membrane proteins in the urea transporter protein A (UT-A) and urea transporter protein B (UT-B) families. UT-B is mainly expressed in endothelial cell membrane of the renal medulla and in other tissues, including the brain, heart, pancreas, colon, bladder, bone marrow, and cochlea. UT-B is responsible for the maintenance of urea concentration, male reproductive function, blood pressure, bone metabolism, and brain astrocyte and cardiac functions. Its deficiency and dysfunction contribute to the pathogenesis of many diseases. Actually, UT-B deficiency increases the sensitivity of bladder epithelial cells to apoptosis triggers in mice and UT-B-null mice develop II-III atrioventricular block and depression. The expression of UT-B in the rumen of cow and sheep may participate in digestive function. However, there is no systemic review to discuss the UT-B functions. Here, we update research approaches to understanding the functions of UT-B.
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Cortada, Maurizio, Soledad Levano, and Daniel Bodmer. "mTOR Signaling in the Inner Ear as Potential Target to Treat Hearing Loss." International Journal of Molecular Sciences 22, no. 12 (June 14, 2021): 6368. http://dx.doi.org/10.3390/ijms22126368.
Full textAbstract:
Hearing loss affects many people worldwide and occurs often as a result of age, ototoxic drugs and/or excessive noise exposure. With a growing number of elderly people, the number of people suffering from hearing loss will also increase in the future. Despite the high number of affected people, for most patients there is no curative therapy for hearing loss and hearing aids or cochlea implants remain the only option. Important treatment approaches for hearing loss include the development of regenerative therapies or the inhibition of cell death/promotion of cell survival pathways. The mammalian target of rapamycin (mTOR) pathway is a central regulator of cell growth, is involved in cell survival, and has been shown to be implicated in many age-related diseases. In the inner ear, mTOR signaling has also started to gain attention recently. In this review, we will emphasize recent discoveries of mTOR signaling in the inner ear and discuss implications for possible treatments for hearing restoration.
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Chan, S. Y., M. Medhi, A. Ekbote, S. Moses, N. Sibtain, T. Andrews, A. F. O'connor, and R. Kulasegaram. "Syphilis causing hearing loss." International Journal of STD & AIDS 19, no. 10 (October 2008): 721–22. http://dx.doi.org/10.1258/ijsa.2008.008124.
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An HIV-positive man with hepatitis B co-infection, naïve to highly active antiretroviral therapy, with a CD4 of 594 copies/mL and HIV-1 viral load of 140,070 copies, presented with right-sided facial weakness and hearing loss. He had been treated for secondary syphilis three months earlier when his rapid plasma reagin (RPR) result was 1:16, this had fallen to neat. At presentation, his RPR had risen to 1:16 again. A magnetic resonance imaging scan showed enhancement of the internal auditory canal and right cochlea. His cerebrospinal fluid examination was normal. He was treated with acyclovir and prednisolone before the syphilis serology was known. He was then treated for syphilis with doxycycline. He made an excellent recovery.
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Handi, Prakash S., Mallikarjun N. Patil, and Nisha P. "High resolution computed tomography of temporal bone in the evaluation of otologic diseases." International Journal of Otorhinolaryngology and Head and Neck Surgery 4, no. 1 (December 22, 2017): 87. http://dx.doi.org/10.18203/issn.2454-5929.ijohns20174546.
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<p class="abstract"><strong>Background:</strong> <span lang="EN-IN">Conventional radiological methods like X-ray mastoid have limited ability to delineate the details of the complex temporal bone and cochlea-vestibular anatomy and disease pathology. This can be overcome by the use of High resolution computed tomography (HRCT) evaluation of the temporal bone. HRCT is helpful in evaluating the anatomy and pathology type and extent of disease, thereby helps in preoperative planning. </span></p><p class="abstract"><strong>Methods:</strong> <span lang="EN-IN">The CT scan of forty five patients attending the outpatient department of otorhinolaryngology in a tertiary care centre between August 2014 to August 2017 was evaluated in this study. Each CT scan was interpreted by an otolaryngologist and a radiologist and interpretation was by consensus. The data collected was evaluated and results are reported as rates and proportions (%). </span></p><p class="abstract"><strong>Results:</strong> <span lang="EN-IN">The study evaluated CT scan of 45 patients of which 35 were male patients and 10 were female patients with age ranging from 5 years to 75 years. Most of these patients belonged to the 21-30, 31-40 age groups [9 (20%) in each group]. Infections of middle ear cleft (all with cholesteatoma), fractures of the temporal bone, anomalies and tumors of the external auditory canal and middle ear were the pathologies observed in these CT scans with infection (otomastoiditis) [23 (51.1%)] being the most common pathology observed. </span></p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-IN">Pre-operative CT scan may not only help in identifying the nature, extent of infectious disease including ossicular, bony erosions; it may also identify anomalies in the temporal bone and surrounding structures. Thus, HRCT temporal bone will help the surgeon in planning effective surgical strategy, reducing morbidity during the surgery.</span></p>
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Yassuda, Camila C., Ana Elisa M. Righetti, Maria Cristina L. Cury, Miguel Ângelo Hyppolito, José Antonio A. de Oliveira, and Omar Féres. "The role of hyperbaric oxygen therapy (HOT) as an otoprotection agent against cisplatin ototoxicity." Acta Cirurgica Brasileira 23, suppl 1 (2008): 72–76. http://dx.doi.org/10.1590/s0102-86502008000700013.
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PURPOSE: Hyperbaric oxygen therapy (HOT) consists of intermittent inhalations of 100% oxygen at a pressure higher than 1 atm. It is an important adjuvant therapy in pathological processes like soft tissue infections, radiation injury, gas gangrene, osteomyelitis and decompressive diseases. Cisplatin, a potent antineoplastic drug, widely used in cancer therapy is highly ototoxic causing bilateral, irreversible damage to the hearing of high frequency sounds (4-8 KHz). OBJECTIVE:This experimental study conducted at the Faculty of Medicine of Ribeirao Preto, University of Sao Paulo aims to evaluate Hyperbaric Oxygen Therapy as an otoprotection agent against drug toxicity. METHODS: Albino guinea pigs were divided into two groups: in Group A, 5 animals (10 cochlea) received cisplatin, i. p., 8.0 mg/kg/day during three days and afterwards were submitted to HOT; in Group B, 3 animals (6cochlea) received cisplatin, i. p. 8.0mg/kg/day during three days. Guinea pigs were evaluated by acoustic otoemissions (AOE) and scanning electron microscopy (SEM). RESULTS: Group B animals showed loss of auditory functions as measured by AOE and distorted outer hair cells by SEM. In Group A, outer hair cells shown by SEM images were mostly preserved. CONCLUSION: It is presumed that Hyperbaric Oxygen Therapy has a protector effect against cisplatin ototoxicity.
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Møller, Aage R. "Sensorineural Tinnitus: Its Pathology and Probable Therapies." International Journal of Otolaryngology 2016 (2016): 1–13. http://dx.doi.org/10.1155/2016/2830157.
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Tinnitus is not a single disease but a group of different diseases with different pathologies and therefore different treatments. Regarding tinnitus as a single disease is hampering progress in understanding of the pathophysiology of tinnitus and perhaps, more importantly, it is a serious obstacle in development of effective treatments for tinnitus. Subjective tinnitus is a phantom sound that takes many different forms and has similarities with chronic neuropathic pain. The pathology may be in the cochlea, in the auditory nerve, or, most commonly, in the brain. Like chronic neuropathic pain tinnitus is not life threatening but influences many normal functions such as sleep and the ability to concentrate on work. Some forms of chronic tinnitus have two components, a (phantom) sound and a component that may best be described as suffering or distress. The pathology of these two components may be different and the treatment that is most effective may be different for these two components. The most common form of treatment of tinnitus is pharmacological agents and behavioral treatment combined with sound therapy. Less common treatments are hypnosis and acupuncture. Various forms of neuromodulation are becoming in use in an attempt to reverse maladaptive plastic changes in the brain.
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Tower-Gilchrist, Cristy, Stephanie A. Zlatic, Dehong Yu, Qing Chang, Hao Wu, Xi Lin, Victor Faundez, and Ping Chen. "Adaptor protein-3 complex is required for Vangl2 trafficking and planar cell polarity of the inner ear." Molecular Biology of the Cell 30, no. 18 (August 15, 2019): 2422–34. http://dx.doi.org/10.1091/mbc.e16-08-0592.
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Planar cell polarity (PCP) regulates coordinated cellular polarity among neighboring cells to establish a polarity axis parallel to the plane of the tissue. Disruption in PCP results in a range of developmental anomalies and diseases. A key feature of PCP is the polarized and asymmetric localization of several membrane PCP proteins, which is essential to establish the polarity axis to orient cells coordinately. However, the machinery that regulates the asymmetric partition of PCP proteins remains largely unknown. In the present study, we show Van gogh-like 2 (Vangl2) in early and recycling endosomes as made evident by colocalization with diverse endosomal Rab proteins. Vangl2 biochemically interacts with adaptor protein-3 complex (AP-3). Using short hairpin RNA knockdown, we found that Vangl2 subcellular localization was modified in AP-3–depleted cells. Moreover, Vangl2 membrane localization within the cochlea is greatly reduced in AP-3–deficient mocha mice, which exhibit profound hearing loss. In inner ears from AP-3–deficient mocha mice, we observed PCP-dependent phenotypes, such as misorientation and deformation of hair cell stereociliary bundles and disorganization of hair cells characteristic of defects in convergent extension that is driven by PCP. These findings demonstrate a novel role of AP-3–mediated sorting mechanisms in regulating PCP proteins.
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Anuwatworn, Amornpol, Prince Sethi, Kelly Steffen, Orvar Jonsson, and Marian Petrasko. "Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome." Case Reports in Cardiology 2017 (2017): 1–3. http://dx.doi.org/10.1155/2017/1705927.
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Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.
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Xia, Liang, Jingjing Liu, Yuanyuan Sun, Haibo Shi, Guang Yang, Yanmei Feng, and Shankai Yin. "Rosiglitazone Improves Glucocorticoid Resistance in a Sudden Sensorineural Hearing Loss by Promoting MAP Kinase Phosphatase-1 Expression." Mediators of Inflammation 2019 (May 14, 2019): 1–10. http://dx.doi.org/10.1155/2019/7915730.
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In this study, we investigated the role of MAP kinase phosphatase-1 (MKP-1) and rosiglitazone (RSG) in glucocorticoid resistance and glucocorticoid sensitivity, respectively, using a guinea pig model of lipopolysaccharide- (LPS-) induced sudden sensorineural hearing loss (SSHL). The pigs were divided into control, LPS, LPS+dexamethasone (DEX), LPS+RSG, and LPS+DEX+RSG groups. Their hearing was screened by auditory brainstem response measurement. Immunofluorescence staining was used to identify the location of MKP-1 in the inner ear. The expression levels of MKP-1 and the related proteins in the inner ear were detected using western blotting. The morphological changes in the cochlea were observed via hematoxylin-eosin staining. Severe hearing loss was observed in the LPS group, as opposed to the protection from hearing loss observed in the LPS+DEX+RSG group. A positive correlation was observed between MKP-1 expression levels and protection from hearing loss. RSG and DEX synergistically influenced inner ear inflammation. In conclusion, resistance of LPS-induced SSHL guinea pig models to glucocorticoids may result from impaired MKP-1 function in inner ear tissues, induced by glucocorticoids, impairing the inhibition of inflammation. Our findings present novel targets to develop potential therapeutics to treat inflammatory diseases of the inner ear.
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Chester, Jacqueline, Edan Johnston, Daniel Walker, Melissa Jones, Corina Mihaela Ionescu, Susbin Raj Wagle, Božica Kovacevic, et al. "A Review on Recent Advancement on Age-Related Hearing Loss: The Applications of Nanotechnology, Drug Pharmacology, and Biotechnology." Pharmaceutics 13, no. 7 (July 8, 2021): 1041. http://dx.doi.org/10.3390/pharmaceutics13071041.
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Aging is considered a contributing factor to many diseases such as cardiovascular disease, Alzheimer’s disease, and hearing loss. Age-related hearing loss, also termed presbycusis, is one of the most common sensory impairments worldwide, affecting one in five people over 50 years of age, and this prevalence is growing annually. Associations have emerged between presbycusis and detrimental health outcomes, including social isolation and mental health. It remains largely untreatable apart from hearing aids, and with no globally established prevention strategies in the clinical setting. Hence, this review aims to explore the pathophysiology of presbycusis and potential therapies, based on a recent advancement in bile acid-based bio-nanotechnologies. A comprehensive online search was carried out using the following keywords: presbycusis, drugs, hearing loss, bile acids, nanotechnology, and more than 150 publications were considered directly relevant. Evidence of the multifaceted oxidative stress and chronic inflammation involvement in cellular damage and apoptosis that is associated with a loss of hair cells, damaged and inflamed stria vascularis, and neuronal signalling loss and apoptosis continues to emerge. New robust and effective therapies require drug delivery deeper into the various layers of the cochlea. Bile acid-based nanotechnology has gained wide interest in its permeation-enhancing ability and potential for numerous applications in treating presbycusis.
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Tahera, Yeasmin, Inna Meltser, Peter Johansson, Anita C. Hansson, and Barbara Canlon. "Glucocorticoid Receptor and Nuclear Factor-κB Interactions in Restraint Stress-Mediated Protection against Acoustic Trauma." Endocrinology 147, no. 9 (September 1, 2006): 4430–37. http://dx.doi.org/10.1210/en.2006-0260.
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The role of glucocorticoid receptors (GRs) in the protective effect of restraint stress (RS) before acoustic trauma was studied in spiral ganglion neurons of CBA mice. RS increased corticosterone and protected against elevated auditory brain stem thresholds caused by acoustic trauma. This protection was inhibited by the pretreatment with a corticosterone synthesis inhibitor, metyrapone (MET), and a GR antagonist (RU486). RS followed by acoustic trauma caused an immediate increase in corticosterone that triggered nuclear translocation of GR, without a change in the expression of GR protein. RU486 + MET before RS and acoustic trauma caused an immediate increase in GR mRNA followed by increased GR protein expression (24 h after trauma). GR signaling was further characterized by analyzing nuclear factor-κB (NFκB) nuclear translocation and protein expression. NFκB nuclear translocation was reduced after acoustic trauma or pretreatment with RU486 + MET before RS and acoustic trauma. On the contrary, RS protected against the trauma-induced NFκB reduction of its nuclear translocation in inhibitory-κB (IκB)-dependent manner. RU486 + MET caused a simultaneous decreased IκB expression and NFκB nuclear translocation, demonstrating an interference with the IκB-mediated activation of NFκB. In summary, RS protects the cochlea from acoustic trauma by increasing corticosterone and activating GRs. These results emphasis how GR activity modulates hearing sensitivity and its importance for the rationale use of glucocorticoids in inner ear diseases.
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Merchant, Saumil N., Michael E. Ravicz, Susan E. Voss, William T. Peake, and John J. Rosowski. "Middle ear mechanics in normal, diseased and reconstructed ears." Journal of Laryngology & Otology 112, no. 8 (August 1998): 715–31. http://dx.doi.org/10.1017/s0022215100141568.
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AbstractA review of the structure-function relationships in normal, diseased and reconstructed middle ears is presented. Variables used to describe the system are sound pressure, volume velocity and acoustic impedance. We discuss the following(1) Sound can be transmitted from the ear canal to the cochlea via two mechanisms: the tympanoossicular system (ossicular coupling) and direct acoustic stimulation of the oval and round windows (acoustic coupling). In the normal ear, middle-ear pressure gain, which is the result of ossicular coupling, is frequency-dependent and smaller than generally believed. Acoustic coupling is negligibly small in normal ears, but can play a significant role in some diseased and reconstructed ears.(2) The severity of conductive hearing loss due to middle-ear disease or after tympanoplasty surgery can be predicted by the degree to which ossicular coupling, acoustic coupling, and stapes-cochlear input impedance are compromised. Such analyses are used to explain the air-bone gaps associated with lesions such as ossicular interruption, ossicular fixation and tympanic membrane perforation.(3) With type IV and V tympanoplasty, hearing is determined solely by acoustic coupling. A quantitative analysis of structure-function relationships can both explain the wide range of observed postoperative hearing results and suggest surgical guidelines in order to optimize the post-operative results.(4) In tympanoplasty types I, II and III, the hearing result depends on the efficacy of the reconstructed tympanic membrane, the efficacy of the reconstructed ossicular chain and adequacy of middle-ear aeration. Currently, our knowledge of the mechanics of these three factors is incomplete. The mechanics of mastoidectomy and stapedectomy are also discussed.
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Rasouli Fard, Parisa, Farnoush Jarollahi, Seyyed Jalal Sameni, and Mohammad Kamali. "Effect of rehabilitation training on an elderly population with mild to moderate hearing loss: study protocol for a randomised clinical trial." F1000Research 9 (June 9, 2020): 582. http://dx.doi.org/10.12688/f1000research.23332.1.
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Background: Age-related hearing loss (presbycusis) is a form of hearing loss in over 60-year-olds and has a negative impact on quality of life. The pathophysiology of presbycusis is multifactorial and is predominately characterised with a loss of speech perception in noise. In the cochlea, auditory filters decompose broadband sound into a series of narrowband output signals, which contains two kinds of temporal information: slow changes in overall amplitude envelope (ENV) and faster variations in temporal fine structure (TFS). TFS is important for recognition of target speech in noise. The main aim of the study is to evaluate the effect of TFS rehabilitation training in participants over the age of 60 years with mild to moderate hearing loss. Methods: A randomised clinical trial will be conducted on 30 participants with mild (loss of 20-39dB) to moderate (40-69dB) hearing loss, aged between 60 and 75 years old. Participants with conductive hearing loss, abnormal middle ear pathology and central nerve system disease will be excluded. Participants will be randomly selected to an intervention and control group with a 1:1 ratio. Rehabilitation for the intervention group will be 30-minute sessions three times a week for a total five weeks of vowel consonant vowel words that are used to eliminate ENV and keep only TFS. Word in noise test, binaural TFS test, and Speech, Spatial and Qualities of Hearing Scale scores will be performed at the beginning and end of study to evaluate the effect of rehabilitation training. Conclusion: Life expectancy in the elderly has improved, leading to an increased prevalence of age-related diseases including presbycusis. A literature review highlighted that TFS damage is permanent; however, in this study we will attempt to prove that TFS training may lead to speech in noise perception restored. Trial registration: Registry of Clinical Trials, IRCT2019625044006N1 (7th August 2019).
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Rasouli Fard, Parisa, Farnoush Jarollahi, Seyyed Jalal Sameni, and Mohammad Kamali. "Effect of rehabilitation training on an elderly population with mild to moderate hearing loss: study protocol for a randomised clinical trial." F1000Research 9 (August 11, 2020): 582. http://dx.doi.org/10.12688/f1000research.23332.2.
Full textAbstract:
Background: Age-related hearing loss (presbycusis) is a form of hearing loss in over 60-year-olds and has a negative impact on quality of life. The pathophysiology of presbycusis is multifactorial and is predominately characterised with a loss of speech perception in noise. In the cochlea, auditory filters decompose broadband sound into a series of narrowband output signals, which contains two kinds of temporal information: slow changes in overall amplitude envelope (ENV) and faster variations in temporal fine structure (TFS). TFS is important for recognition of target speech in noise. The main aim of the study is to evaluate the effect of TFS rehabilitation training in participants over the age of 60 years with mild to moderate hearing loss. Methods: A randomised clinical trial conduct on 30 participants with mild (loss of 20-39dB) to moderate (40-69dB) hearing loss, aged between 60 and 75 years old. Participants with conductive hearing loss, abnormal middle ear pathology and central nerve system disease were excluded. Participants were randomly selected to an intervention and control group with a 1:1 ratio. Rehabilitation for the intervention Group are 30-minute sessions three times a week for a total five weeks of vowel consonant vowel words that are used to eliminate ENV and keep only TFS. Word in noise test, binaural TFS test, and Speech, Spatial and Qualities of Hearing Scale scores are performed at the beginning and end of study to evaluate the effect of rehabilitation training. Conclusion: Life expectancy in the elderly has improved, leading to an increased prevalence of age-related diseases including presbycusis. A literature review highlighted that TFS damage is permanent; however, in this study we will attempt to prove that TFS training may lead to speech in noise perception restored. Trial registration: Registry of Clinical Trials, IRCT2019625044006N1 (7th August 2019).
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Rasouli Fard, Parisa, Farnoush Jarollahi, Seyyed Jalal Sameni, and Mohammad Kamali. "Effects of rehabilitation training on an elderly population with mild to moderate hearing loss: study protocol for a randomised clinical trial." F1000Research 9 (September 15, 2020): 582. http://dx.doi.org/10.12688/f1000research.23332.3.
Full textAbstract:
Background: Age-related hearing loss (presbycusis) is a form of hearing loss in over 60-years-olds and has a negative impact on quality of life. Presbycusis is multifactorial and is predominately characterised with a loss of speech perception in noise. In the cochlea, auditory filters decompose broadband sound into a series of narrowband output signals, which contains two kinds of temporal information: slow changes in overall amplitude envelope (ENV) and faster variations in temporal fine structure (TFS). TFS is important for recognition of target speech in noise. The main aim of the study is to evaluate the effect of TFS rehabilitation training in participants over the age of 60 years with mild to moderate hearing loss. Methods: A randomised clinical trial conducted on 30 participants with mild (loss of 20-39dB HL) to moderate (40-69dB HL) hearing loss, aged between 60 and 75 years old. Participants with conductive hearing loss, abnormal middle ear pathology and central nervous system disease were excluded. Participants were selected randomly to an intervention and control group with a 1:1 ratio. Rehabilitation for the intervention Group are 30-minute sessions three times a week for a total five weeks of vowel consonant vowel words that are used to eliminate ENV and keep only TFS. Word in noise test, binaural TFS test, and Speech, Spatial and Qualities of Hearing Scale scores are performed at the beginning and end of study to evaluate the effect of rehabilitation training. Conclusion: Life expectancy in the elderly has improved, leading to an increased prevalence of age-related diseases including presbycusis. A literature review highlighted that TFS damage is permanent; however, in this study we will attempt to prove that TFS training may lead to speech in noise perception improvement. Trial registration: Registry of Clinical Trials, IRCT2019625044006N1 (7th August 2019).
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Segana, Hasan Abdul Cader, Reghunandanan Nair, and Fahim Ahmed Shah. "Vitamin D Deficiency in South Sharqiya in Oman and its Impact in ENT Patients- A Retrospective Study." Bengal Journal of Otolaryngology and Head Neck Surgery 24, no. 3 (December 18, 2016): 122–28. http://dx.doi.org/10.47210/bjohns.2016.v24i3.89.
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Introduction
Vitamin D deficiency has multitude of causes and can present with varying clinical manifestations. Studies show that it can lead on to recurrent respiratory infections, ear infections and deafness. Vitamin D also has immunomodulant action. Here we discuss the varying features concerning an Otolaryngologist in general as far as Vitamin D metabolism is concerned.
Materials and Methods
This retrospective study was performed on 800 patients 152 males and 648 females of different socioeconomic background at secondary level regional referral hospital under Ministry of Health in Sultanate of Oman. The patients attending the outpatient clinic with various complaints and not responding to conventional treatment were advised for assessment of vitamin D [25 (OH) D] level in blood. The patients were evaluated with general history, blood samples of serum calcium, phosphate, alkaline phosphatase and serum vitamin D level were measured by the most standardized laboratory of the country.
Results
Out of 800 patients, 275 cases had Vitamin D levels below 20 ng/ml and 167 patients had values greater than 30 ng/ml in serum.81% patients with vitamin D deficiency were females.56.25% patients were between third and sixth decade. Otolaryngologic manifestations were acute and recurrent URTI (n=352) 44%. 7% of the patients presented with recurrent ear infection (otitis externa). The rest of them presented to a lesser extent with deafness, otosclerosis.
Discussion
Vitamin D deficiency has been reported worldwide as one of the commonest deficiency diseases. It can lead to autoimmune dysfunctions, Beta cell dysfunction in pancreas, Multiple sclerosis, recurrent chest infections and congestive cardiac failure. Studies have shown the involvement of cochlea with sensorineural hearing loss and otosclerosis.
Conclusion
Vitamin D deficiency has multi system implications as patients presenting with different signs and symptoms. Mass level screening and vitamin D supplementation should be planned to decrease its varied and multidimensional ill effects on health. Adequate vitamin D supplementation and sensible sunlight exposure to reach optimal vitamin D status are among the front line factors of prophylaxis for spectrum of disorders.
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El-Kashlan, Hussam K., and Steven A. Telian. "Cochlear implantation in the chronically diseased ear." Current Opinion in Otolaryngology & Head and Neck Surgery 12, no. 5 (October 2004): 384–86. http://dx.doi.org/10.1097/01.moo.0000134439.99357.c9.
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Im, G. J., and H. H. Jung. "Side selection for cochlear implantation in a case of Cogan's syndrome." Journal of Laryngology & Otology 122, no. 3 (May 25, 2007): 310–13. http://dx.doi.org/10.1017/s0022215107008742.
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AbstractCogan's syndrome is a rare clinical entity that is characterised by non-syphilitic interstitial keratitis and audiovestibular symptoms. The cause of Cogan's syndrome is considered to be autoimmune disease, which is supported by the resolution of hearing loss after steroid treatment, and the association with other autoimmune diseases. The sensorineural hearing loss of Cogan's syndrome is progressive over a few months, and sudden, bilateral deafness often occurs, which may be an indication for cochlear implantation. This paper presents the case of a young woman suffering from Cogan's syndrome and sudden, bilateral deafness. With reference to this case, we describe problems regarding cochlear implantation for Cogan's syndrome and radiological findings aiding selection of cochlear implantation side.
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Harada, Tamotsu, Mitsuhito Sano, Satoshi Ogino, Masafumi Sakagami, and Toru Matsunaga. "Mechanism of Immune Complex—Mediated Inner Ear Diseases." Annals of Otology, Rhinology & Laryngology 101, no. 10_suppl (October 1992): 72–77. http://dx.doi.org/10.1177/0003489492101s1015.
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Tissues of the stria vascularis of normal rabbits were collected as cochlear antigen and injected into the foot pad of guinea pigs. The mechanism of development of endolymphatic hydrops was studied by the tracer method. Damage to the capillary endothelium and an increase in vascular permeability were found in the stria vascularis. Therefore, we studied patients with a raised level of immunoglobulin (Ig) G—class circulating immune complexes (CICs). Hearing loss was found in 3 of 22 patients with significantly elevated IgG CIC levels (13.6%). The relationship between inner ear disease and the presence of CIC was considered to be very significant. The possible mechanism of CIC-mediated inner ear diseases is discussed.
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Mulwafu, W., D. R. Strachan, R. Bartlett, and C. Caron. "Cochlear implantation in Malawi: report of the first four cases." Journal of Laryngology & Otology 131, no. 10 (August 15, 2017): 914–18. http://dx.doi.org/10.1017/s0022215117001724.
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AbstractObjective:This paper reports on the first four cochlear implant cases in Malawi.Case reports:Three patients were deafened from infectious diseases and one from an unknown cause. They all had post-lingual deafness. Six months after the last implant, they are all progressing well.Conclusion:Despite significant practical difficulties, it has proved possible, with the right support, to carry out cochlear implantation in one of the world's poorest countries. The project has also raised awareness of deafness in Malawi and highlighted significant public health issues relating to the aetiology of deafness in developing countries.
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Yamashita, Hiroshi, and Kazuma Sugahara. "Mechanism of Cochlear Protection and Therapy for Inner Ear Diseases." Practica Oto-Rhino-Laryngologica 99, no. 12 (2006): 981–87. http://dx.doi.org/10.5631/jibirin.99.981.
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Seidullayeva, A., D. Bayesheva, B. Turdalina, A. Altynbekova, R. Utegenova, G. Nurtazina, G. Volkova, et al. "Sensorineural hearing loss after bacterial meningitis in children residing in Nur-Sultan." Infekcionnye bolezni 18, no. 4 (2020): 189–94. http://dx.doi.org/10.20953/1729-9225-2020-4-189-194.
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Bacterial meningitis (BM) is a widespread health problem characterized by severe clinical manifestations and high incidence of neurological complications. BM remains one of the main causes of disability and mortality among young children all over the world. Hearing loss is one of neurological complications associated with BM. It accounts for up to 60%–90% of all cases of acquired sensorineural hearing loss (SNHL). Between 2015 and 2018, we performed screening for SNHL among children who had had BM. Seven out of 62 patients examined (11.3%) were found to have SNHL. BM was primarily caused by Streptococcus pneumoniae (n = 4) and Neisseria meningitidis (n = 3). Three out of 4 patients had grade 3–4 SNHL. Computed tomography revealed cochlear ossification in two children and cochlear fibrosis in one child (who had successful cochlear implantation later). For the rest of the patients, we recommended hearing aids. We also found that hearing loss usually develops after BM and does not affect patients with viral meningitis (caused by enteroviruses). We recommend that children with BM undergo regular screening for SNHL (every 3 days during treatment) using otoacoustic emission. These patients should be also examined by an audiologist after discharge from hospital and then every three months for a year. Key words: children, cochlear implantation, meningitis, sensorineural hearing loss, acquired deafness
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Colletti, Vittorio, Marco Carner, Veronica Miorelli, Maurizio Guida, Liliana Colletti, and Francesco Fiorino. "Auditory Brainstem Implant (ABI): New Frontiers in Adults and Children." Otolaryngology–Head and Neck Surgery 133, no. 1 (July 2005): 126–38. http://dx.doi.org/10.1016/j.otohns.2005.03.022.
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Previous studies have considered only patients with neurofibromatosis type 2 (NF2) older than 12 years as candidates for an auditory brainstem implant (ABI). Our study expands the potential criteria to include both children and adult subjects with other cochlear or cochlear nerve malfunctions who either would not benefit at all from a cochlear implant (eg, cochlear nerve aplasia or avulsion) or whose benefit was or would be severely compromised (eg, cochlear ossification, cochlear fracture). STUDY DESIGN: In our department, over the period from April 1997 to September 2002, 29 patients, 20 adults and 9 children, were fitted with ABIs. Their ages ranged from 14 months to 70 years. Thirteen subjects had tumors, 10 NF2 and 3 solitary vestibular schwannoma, and 16 patients had a variety of nontumor (NT) cochlear or cochlear nerve diseases. A retrosigmoid-transmeatal approach was used in T and a retrosigmoid approach in NT patients. The electrode array was inserted into the lateral recess of the fourth ventricle and correct electrode positioning was monitored with the aid of electrically evoked auditory brainstem responses (EABRs). RESULTS: Correct implantation was achieved in all patients. No complications were observed due to implantation surgery or related to ABI activation or long-term use. Auditory sensations were induced in all patients with various numbers of electrodes (from 5 to 15). Different pitch sensations were identifiable with different electrode stimulation. Closed-set word recognition, open-set sentence recognition, and speech tracking scores achieved by the patients are reported in detail. The auditory performance of the patients showed significantly better outcomes than controls (Multicentric European clinical investigations on ABI with NF2). CONCLUSION: We have shown that the indications for the ABI can be extended to include NT patients with severe cochlear and/or cochlear nerve abnormalities. The degree of auditory benefit varies as a function of the underlying pathological conditions, with NT subjects exhibiting significantly better outcomes than the T patients.
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Harcourt, J. P., P. Lennox, P. D. Phelps, and G. B. Brookes. "CT screening for temporal bone abnormalities in idiopathic bilateral sensorineural hearing loss." Journal of Laryngology & Otology 111, no. 2 (February 1997): 117–21. http://dx.doi.org/10.1017/s002221510013662x.
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AbstractBilateral sensorineural hearing loss can be caused by a variety of temporal bone abnormalities including primary cochlear otoscierosis, local and systemic bony diseases and some metabolic conditions. These may be identified using computerized tomography (CT), with attenuation recordings taken across the cochlear capsule (CT densitometry). Eighty patients with bilateral sensorineural hearing loss were screened over a period of six and a half years using this technique, and only three cases (3.8 per cent) of treatable disease were detected. Positive yields may be increased by screening selected cases with other clinical or biochemical stigmata of temporal bone disease.
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Tono, Tetsuya, Yuki Nagano, and Takumi Okuda. "Electrical Stimulation Test for Acquired Retrocochlear Diseases in Cochlear Implant Candidates." Practica oto-rhino-laryngologica. Suppl. 132 (2012): 104–8. http://dx.doi.org/10.5631/jibirinsuppl.132.104.
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Bance, Manohar, and Jason Erb. "A Reliable Radiologic Landmark for the Facial Nerve in Axial Temporal Bone Computed Tomography Scans." Otolaryngology–Head and Neck Surgery 128, no. 2 (February 2003): 251–56. http://dx.doi.org/10.1067/mhn.2003.64.
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OBJECTIVE: We sought to test the reliability of a radiologic marker in identifying the vertical portion of the facial nerve in axial computed tomography (CT) temporal bone scans. STUDY DESIGN AND SETTING: At a tertiary care academic center, we used, with a random sample of 25 CT scans, a marker (the “B-line”) to identify the facial nerve. The variations in distance from this marker to the facial nerve were measured. RESULTS: This marker, which consists of a tangent line extrapolated from the posterior border of the basal turn of the cochlea, fell within 1 mm of the facial nerve on average. The average distance from the midpoint of the posterior border of the basal turn of the cochlea to the facial nerve was 11 ± 1 mm. CONCLUSION: This is a very reliable marker for the vertical portion of the facial nerve. SIGNIFICANCE: This marker can be used to rapidly find the facial nerve, even in diseased or postsurgical temporal bones.
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Joglekar, Shruti Siddharth, Armin Farajzadeh Deroee, Norimasa Morita, Sebahattin Cureoglu, Schachern Patricia, Michael M. Paparella, and Steven K. Juhn. "S239 – Labyrinthine Changes in Otitis Media." Otolaryngology–Head and Neck Surgery 139, no. 2_suppl (August 2008): P155. http://dx.doi.org/10.1016/j.otohns.2008.05.414.
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Objectives Otitis media causes labyrinthine changes and subsequent sensorineural hearing loss. The aim of this histopathologic study was to evaluate the extension of inflammation to the inner ear and its effects. Methods Out of 614 temporal bones with otitis media, 47 (30 cases) with chronic and 35 (21 cases) with purulent were selected for histopathologic study. Subjects with a history of acoustic trauma, head trauma, ototoxic drugs and other otologic and systemic diseases affecting the inner ear were excluded. The pattern of labyrinthine inflammation was classified as localized purulent, localized serous, generalized seropurulent and generalized serous. Inner ear findings were compared to age-matched controls. Results 19% of temporal bones with chronic and 9% of temporal bones with purulent otitis media showed labyrinthine inflammatory changes. In chronic otitis media, inflammatory changes were: 56% localized purulent; 22% localized serous; 11% generalized seropurulent; and 11% generalized serous. Inflammatory changes in temporal bones with purulent otitis media included: 67% localized purulent; and 33% generalized seropurulent. Pathological findings included: serofibrinous precipitates and inflammatory cells in the scala tympani of basal turn and cochlear aqueduct; significant decrease in area of stria vascularis (p = 0.033); and loss of hair cells in the organs of Corti. No significant difference was found in area of spiral ligament area or number of fibrocytes in diseased and control bones. Conclusions Middle ear/inner ear interaction in otitis media can result in labyrinthine inflammation and cochlear damage. Early diagnosis and treatment of otitis media is important in preventing inner ear damage.
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Shidlovskaya, Tetiana A., and Nadiya Ya Navalkivska. "Distortion product otoacoustic emissions among the patients suffering diabetes mellitus type II with hearing impairment." OTORHINOLARYNGOLOGY, no. 4-5(2) 2019 (March 12, 2020): 47–52. http://dx.doi.org/10.37219/2528-8253-2019-4-47.
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Topicality: Objective research methods of the auditory analyzer are of great importance in diagnosis sensorineural disorders of auditory function, treatment of such diseases and solving a number of expert questions. While diagnosis the damage of auditory system, especially the receptor part of the auditory analyzer the method of the caused OAE on the frequency distortion product (DPOAE) plays a significant role. Quite often at sensorineural hearing loss of different genesis is observed an affection of cochlea structures of the inner ear. Diabetes mellitus (DM) is one of the most acute medical and social problems. According to the latest data, there are 463 million adults with diabetes mellitus in the world; accounting for 9.3% of the world's total population, meaning that 1 among 11 people suffers from diabetes (IDF Diabetes Atlas 9th edition, 2019). Among patients with diabetes, according to various authors, there are disorders in the auditory analyzer. Purpose: to determine changes in the condition of the receptor part of auditory analyzer according to the OAE registration of the frequency of distortion products among patients with diabetes mellitus type II with impaired auditory function. Materials and methods: 17 patients with diabetes mellitus type II were examined, who, according to subjective audiometry, revealed impairments of auditory function of the I-II degree. The control group consisted of 15 healthy normal-hearing persons who did not report hearing impairment, had no elevated blood sugar levels. 32 persons were examined in total. The registration of the caused OAE at frequency of distortion product (DPOAE) was conducted on the analyzing system "Eclipse" "Interacoustics" (Denmark) at frequencies 1-6 kHz. Statistical processing of the obtained results was carried out according to the conventional methods of mathematical variation statistics using the STATISTICA software package. The average value of indicators was calculated - magnitude (M) and its error (+- m). The probability of changes and differences between the comparative values was estimated by the criterion for the reliability of the difference (t) according to the Student's table. Results and discussion: We have selected for the analysis patients with diabetes mellitus type II, who according to subjective audiometry revealed impaired auditory function. Patients also reported otologic complaints of hearing loss, periodic or persistent subjective ear noise. As a result of the OAE researches at frequency distortion products, the following data were obtained. A full adequate response of the otoacoustic emission across all frequency spectrums was reported in only 3 patients (9.4% of cases) on the one hand. The majority of the observed patients in whom the OAE was registered received a partially positive test (68.7%). In 7 patients (21.8%) the OAE response was not registered. The absence of OAE response or a partially positive registration in 90.6% of the observed patients indicated violation function of the cochlea in such patients. The DPOAE (response intensity) emission amplitude at all tested frequencies (1-6 kHz) in patients with diabetes mellitus type II was significantly (P<0.01) lower than the norm at all frequencies. Particularly pronounced such a decrease is in the area of 4 kHz, where the amplitude of the OAE response was 5.9±0.2 dB at a norm of 9.4±0.5 dB. Therefore, in patients with diabetes mellitus type II with hearing impairment, there are changes in the receptor part of the auditory analyzer, which is confirmed by the data of objective examination methods. The changes we have identified in the OAE indicators may express the affection of the receptor structures of the auditory analyzer among patients with diabetes mellitus type II with impaired auditory function. The information obtained on the OAE indicators provides the detection of early violations and the objective ascertainment of changes in the receptor part of the hearing analyzer in diabetes mellitus type II. Conclusions: 1. OAE data on the frequency of distortion products in patients with diabetes mellitus type II objectively confirm the presence of violations in the auditory receptor. 2. In patients with diabetes mellitus type II, the OAE amplitude at the frequency of distortion products is significantly lower than normal at all tested frequencies (1-6 kHz). Particularly pronounced such a decrease is in the area of 4 kHz, where the amplitude of the OAE response was 5.9±0.2 dB at a norm of 9.4±0.5 dB. 3. The conducted researches prove the expediency of examination by the method of registration of OAE of patients with diabetes mellitus type II, since it promotes the early detection of violation in the receptor structures of the auditory analyzer in such patients. 4. The use of the method of recording otoacoustic emissions in the examination of patients with type II diabetes is important for the objectification of early signs of sensorineural hearing impairment in this contingent.
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Lin, Yung-Song. "Management of otitis media-related diseases in children with a cochlear implant." Acta Oto-Laryngologica 129, no. 3 (January 2009): 254–60. http://dx.doi.org/10.1080/00016480801901741.
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