• Biomedical Science Letters
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• v.29 no.3
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• pp.95-102
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• 2023

The inner ear constitutes a complex organ responsible for auditory perception and equilibrium. It comprises diverse cellular entities operating collaboratively to perceive and transmit sensory information to the brain. Inner ear disease is a sophisticated and multifactorial scenario substantially impacting the quality of life of affected individuals. Gaining insights into the developmental process of the inner ear is crucial for diagnosing and treating inner ear diseases, which can lead to hearing loss and impaired balance. Recent research in inner ear development and associated pathophysiology has focused on several pivotal domains, including identifying new genes and signaling pathways involved in inner ear development, using stem cells for inner ear regeneration, and developing novel therapies for inner ear diseases. Recent advances in genetics research have shed new light on the fundamental etiologies of inner ear diseases, with a growing body of evidence suggesting that genetic mutations might exert a pivotal influence on the development and progression of this condition. In this review, we have delved into certain common genetic mutations linked to inner ear disorders. We also discussed ongoing research endeavors and future directions for understanding the genetic mechanisms underlying this condition and potential therapeutic avenues.

• BMB Reports
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• v.53 no.9
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• pp.449-452
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• 2020

The inner ear is a complex and delicate structure composed of the cochlea and the vestibular system. To maintain normal auditory function, strict homeostasis of the inner ear is needed. A proper immune response against infection, thus, is crucial. Also, since excessive immune reaction can easily damage the normal architecture within the inner ear, the immune response should be fine regulated. The exact mechanism how the inner ear's immune response, specifically the innate immunity, is regulated was unknown. Recently, we reported a protein selectively localized in the inner ear during bacterial infection, named cochlin, as a possible mediator of such regulation. In this review, the immunological function of cochlin and the mechanism behind its role within inner ear immunity is summarized. Cochlin regulates innate immunity by physically entrapping pathogens within scala tympani and recruiting innate immune cells. Such mechanism enables efficient removal of pathogen while preserving the normal inner ear structure from inflammatory damage.

• Journal of Biomedical Engineering Research
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• v.43 no.4
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• pp.193-198
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• 2022

Contrast enhanced magnetic resonance imaging using gadolinium-based contrast agent (GBCA) is a very useful in vivo technique to visualize the inner ear pathology including endolymphatic hydrops. Although systemic intravenous (IV) administration can visualize the perilymph space, the visualization was possible by indirect passage of contrast agent through blood-perilymph barrier. All animal experimental procedures were performed under anesthesia with 5% isoflurane. Lipopolysaccharide (LPS) was instilled into the left tympanic cavity through the tympanic membrane using a sterile 27gauge needle to induce hydrops model. Tucker-Davis Technologies system was used to measure Auditory Brainstem Responses (ABRs). For intracerebroven-tricular (ICV) administration, 25 µmol of GADOVIST (Bayer, Berlin, Germany) was used and diluted GADOVIST injection was 10 µl. MR imaging was acquired with a 9.4 Tesla MRI scanner. Transmit-receive volume coil with 40 mm inner diameter and 75 mm out diameter was used. ICV administration well demonstrated the strong enhancement along the cerebrospinal fluid (CSF) microcirculation pathway including CSF fluid in the subarachnoid space and CSF space of the inner ear structures. On the other hand, IV administration showed no contrast enhancement along the CSF microcirculation pathway and showed weak enhancement in the inner ear structures. In case of rat hydrops model, ICV administration showed that the reduced contrast enhancement in the perilymph space of the hydrops induced inner ear compared to the contrast enhancement in the perilymph space of the normal inner ear. New systemic ICV administration method provide contrast enhancement of GBCA in the inner ear through CSF microcirculation pathway.

• Molecules and Cells
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• v.41 no.7
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• pp.695-702
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• 2018

The inner ear is a complex sensory organ responsible for hearing and balance. Formation of the inner ear is dependent on tight regulation of spatial and temporal expression of genes that direct a series of developmental processes. Recently, epigenetic regulation has emerged as a crucial regulator of the development of various organs. However, what roles higher-order chromatin organization and its regulator molecules play in inner ear development are unclear. CCCTC-binding factor (CTCF) is a highly conserved 11-zinc finger protein that regulates the three-dimensional architecture of chromatin, and is involved in various gene regulation processes. To delineate the role of CTCF in inner ear development, the present study investigated inner ear-specific Ctcf knockout mouse embryos (Pax2-Cre; $Ctcf^{fl/fl}$). The loss of Ctcf resulted in multiple defects of inner ear development and severely compromised otic neurogenesis, which was partly due to a loss of Neurog1 expression. Furthermore, reduced Neurog1 gene expression by CTCF knockdown was found to be associated with changes in histone modification at the gene's promoter, as well as its upstream enhancer. The results of the present study demonstrate that CTCF plays an essential role in otic neurogenesis by modulating histone modification in the Neurog1 locus.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.22 no.1
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• pp.133-140
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• 2009

Objective : To evaluate autonomic nerve balance and sympathetic nerve activity in patients with inner ear dysfunction as examined by heart rate variability(HRV) Research Methods and Procedures : One hundred and twenty three patients(between 15 to 59 years old) who visited Dept. of Oriental medical Opthalmology & Otolaryngology, Kyunghee university and had an examination of HRV test, were selected as subjects of our study. We checked items of HRV test(such as SDNN, RMSSD, LF, HF, TP, norm-LF, norm-HF, LF/HF ratio), and analyzed time and frequency domain differences between three groups classified as tinnitus, hearing loss and vertigo. And we compared HRV items(such as SDNN,RMSSD, TP and LF/HF ratio) with standard levels. Results and Conclusion : Our results showed no significant differences between three groups in HRV items except for mean heart rate. Patients with inner ear dysfunction showed significant lower TP than 2000$(ms^{2})$(p <0.001) and significant higher LF/HF ratio than 1.5(p=0.003). So we suggest that patients with inner ear dysfunctions such as tinnitus, vertigo and hearing loss, have sympathetic hypertonus and inactivity of autonomic nerve regulation.

• Journal of Biomedical Engineering Research
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• v.17 no.4
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• pp.535-544
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• 1996

In this paper, we analyzed the coil-magnet type vibrating transducer for the implantable middle ear hearing aid which is appropriate for patient's hearing level, and an experimental transducer system is designed For the objective and quantitative analysis of the transducer, a theoretical equivalent model containing coil, magneto and inner ear is developed To perform effective evaluation of the transducer, a transforming ratio Tr is introduced and its range that is suitable for practical implantable middle ear hearing device is foun4 The result of applying physical parameters of ear system to the proposed analytical model shows that frequency response of the coil magrlet type vibrator is predominantly governed by resistive impedance of the coil rather than inertia effect of the magnet and the inner parameters. In addition, we realized an experimental middle ear hearing aid system to show the theoretical validity of designed system and this will provide the basis of the development for actually implantable system.

• Korean Journal of Computational Design and Engineering
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• v.14 no.2
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• pp.87-95
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• 2009

This paper describes the design of the ossicular replacement prosthesis for the people who have problem of hearing because of middle ear disease. Also the simulation results of the designed product for verification by finite element analysis software (ABAQUS) are presented. New model is applied to middle ear FE analysis which was generated in previous study. The full replacements of ossicular prosthesis for ossicles (malleus, incus and stapes) are made of Hydroxyapatite and Titanium. Although other existing prosthesis models consider only sound amplification effect, current type has damper system which is operating on the audible frequencies. High frequency sound transmitted to inner ear can be reduced and the prosthesis and inner ear can be prevented from damage.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• v.56 no.12
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• pp.749-753
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• 2013

Basically stem cells have characteristics of multi-potency, differentiation into multiple tissue types, and self-renew through proliferation. Recent advances in stem cell biology can make identifying the stem-cell like cells in various mammalian tissues. Stem cells in various tissues can restore damaged tissue. Stem cells from the adult nervous system proliferate to form clonal floating colonies called spheres in vitro, and recent studies have demonstrated sphere formation by cells in the tympanic membrane, vestibular system, spiral ganglion, and partly in the organ of Corti. The presence of stem cells in the ear raises the possibilities for the regeneration of the tympanic membrane & inner ear hair cells & neurons. But the gradual loss of stem cells postnatally in the organ of Corti may correlate with the loss of regenerative capacity and limited hearing restoration. Future strategies using endogenous stem cells in the ear can be the another treatment modality for the patients with intractable inner ear diseases.

• Speech Sciences
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• v.5 no.1
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• pp.167-179
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• 1999

The hearing mechanism is a complicated system. Sound is generated by a source that sends out air pressure or power. The pressure or power makes the sound waves. These waves reach the eardrum, or tympanic membrane, which vibrates at a rate and magnitude proportional to the nature of the sound waves. The tympanic membrane transforms this vibration into the mechanical energy in the middle ear, which in turn converts it to the hydraulic energy in the fluid of the inner ear. The hydraulic energy stimulates the sensory cells of the inner ear which send neuroelectrical impulses to the central auditory nervous system. The passive perception of auditory information starts just here. The listener gives attention to the speech sound, differentiates the sound from background noise, and integrates his experience with similar sounds. The listener then puts all of these aspects of audition into the context of the moment to identify the nature of sound. This has a major role in human communication. This paper provides an overview of the nature and characteristics of sound, the structure and function of the auditory system, and the way in which sound is processed by the auditory system.

• Health Policy and Management
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• v.7 no.2
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• pp.46-64
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• 1997

Background & Objectives : Korea is face with the social need for health care technology assessment so that it is urgently needed to found principles and methodology in technology assessment in health care. As a groundwork for health care technology assessment, we tried to prioritize medical technology for assessment. Among medical technologies, procedure is somewhat difficult to assess, compared to drug or equipment. In this study, we aimed at the prioritisation of medical procedure to be assessed, in terms of efficay, safety, and adequacy. Method : For the standardized classification of medical procedure, ICD-9-CM(International Classification of Diseases 9th edition - Clinical Modification) was used. Among the list the procedures coming under otorhinolaringjology and thoracic surgery were selected by three family physicians. The list of procedure was mailed to the board certified surgeons of both disciplines, with the question asking about the necessity for assessment in terms of efficay, safety, and adequacy. Replied questionnaires were analyzed in each procedure. Results : Of 560 otorhinolaryngologist and 480 thoracic surgeon, 114 surgeons replied. Of otorhinolaryngological procedure, incision, excision, and destruction of inner ear : fenestration of inner ear : stapedectomy and its revision were the most urgent technology to assess in the aspect of safety. For adequacy, operations on Eustachian tube: fenestration of inner ear: incision, excision, and destruction of inner ear were highly ranked in necessity, and for efficary, operations on Eustachian tube; external maxillary antrotomy; fenestration of inner ear. Thoracic surgeons replied thoracic procedures, lung transplantation; heart transplantation; implantation of heart assist system [pump] are most important for evaluation in terms of safety; and heart transplantation; Lung transplantation; Implantation of heart assist system [pump] in terms of adequacy, and surgical collapse of lung [Artificia니 pnemothorax or pnuexoperitoeum]; lung transplantation; periarterial sympathectomy in terms of efficacy. As a whole, surgeons regard safety evaluation is more urgent than adequacy or efficary. In addition, otorhinolaryngological surgeons regard evaluation of their procedures more urgent than thoracic surgeons regard theirs. Conclusion : By the questionnaire to board certified physicians, we get some preliminary data for prioritisation of technologies to assess. Through the questionnaire like this, much information would be gathered for technology assessment, especially for medical procedure, if not enough. In the near future, well structured expert opinion gathering research, such as modified Delphi or nominal group technique, should be done succeedingly.