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Author Jinsei Jung

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COCH-related autosomal dominant nonsyndromic hearing loss: a phenotype-genotype study.

Kyung Seok Oh, Daniel Walls, Sun Young Joo, Jung Ah Kim, Jee Eun Yoo, Young Ik Koh, Da Hye Kim, John Hoon Rim, Hye Ji Choi, Hye-Youn Kim, Seyoung Yu, Richard J Smith, Jae Young Choi, Heon Yung Gee, Jinsei Jung,

This phenotype-genotype study aimed to investigate the extent of audioprofile variability related to cochlin major domains and to identify potential ethnic-specific differences associated with COCH-related hearing loss. Eight Korean families (26 cases) were diagnosed with COCH-related hearing loss by exome sequencing. Audiometric test results were combined with those from nine ... Read more >>

Hum Genet (Human genetics)
[2021, :]

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Differential genetic diagnoses of adult post-lingual hearing loss according to the audiogram pattern and novel candidate gene evaluation.

John Hoon Rim, Byunghwa Noh, Young Ik Koh, Sun Young Joo, Kyung Seok Oh, Kyumin Kim, Jung Ah Kim, Da Hye Kim, Hye-Youn Kim, Jee Eun Yoo, Seung-Tae Lee, Jin Woong Bok, Min Goo Lee, Jinsei Jung, Jae Young Choi, Heon Yung Gee,

Ski-slope hearing loss (HL), which refers to increased auditory threshold at high frequencies, is common in adults. However, genetic contributions to this post-lingual HL remain largely unknown. Here, we prospectively investigated deafness-associated and novel candidate genes causing ski-slope HL. We analyzed 192 families with post-lingual HL via gene panel and/or ... Read more >>

Hum Genet (Human genetics)
[2021, :]

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Supplementary Effect of Choline Alfoscerate on Speech Recognition in Patients With Age-Related Hearing Loss: A Prospective Study in 34 Patients (57 Ears).

Gina Na, Sang Hyun Kwak, Seung Hyun Jang, Hye Eun Noh, Jungghi Kim, SeungJoon Yang, Jinsei Jung,

To investigate the effect of choline alfoscerate (CA) on hearing amplification in patients with age related hearing loss, we performed a prospective case-control observational study from March 2016 to September 2020. We assessed patients with bilateral word recognition score (WRS) <50% using monosyllabic words. The patients were 65-85 years old, ... Read more >>

Front Aging Neurosci (Frontiers in aging neuroscience)
[2021, 13:684519]

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Corrigendum: Supplementary Effect of Choline Alfoscerate on Speech Recognition in Patients With Age-Related Hearing Loss: A Prospective Study in 34 Patients (57 Ears).

Gina Na, Sang Hyun Kwak, Seung Hyun Jang, Hye Eun Noh, Jungghi Kim, SeungJoon Yang, Jinsei Jung,

[This corrects the article DOI: 10.3389/fnagi.2021.684519.]. ... Read more >>

Front Aging Neurosci (Frontiers in aging neuroscience)
[2021, 13:730527]

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Increased incidence of unilateral sudden sensorineural hearing loss in patients with hematological malignancies requiring hematopoietic stem cell transplantation.

Seong Hoon Bae, Yujin Lee, Jinsei Jung,

Haematopoietic stem cell transplantation (HSCT) may dramatically alter the immunity of a recipient. Transient immunodeficiency that occurs before and after HSCT could be associated with the development of sudden sensorineural hearing loss (SSNHL), which is presumed to be often due to viral aetiology. We found an incidence of SSNHL of ... Read more >>

Clin Otolaryngol (Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery)
[2021, 46(6):1278-1281]

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Heterogeneity of MYO15A variants significantly determine the feasibility of acoustic stimulation with hearing aid and cochlear implant.

Gina Na, Hye Ji Choi, Sun Young Joo, John Hoon Rim, Jung Ah Kim, Hye-Youn Kim, Seyoung Yu, Yeonsu Jeong, Geun Cheol Shin, Hae Eun Noh, Ho Young Lee, Da Hye Kim, Heon Yung Gee, Jinsei Jung, Jae Young Choi,

Autosomal recessive nonsyndromic hearing loss 3 (DFNB3) mainly leads to congenital and severe-to-profound hearing impairment, which is caused by variants in MYO15A. However, audiological heterogeneity in patients with DFNB3 hinders precision medicine in hearing rehabilitation. Here, we aimed to elucidate the heterogeneity of the auditory phenotypes of MYO15A variants according ... Read more >>

Hear Res (Hearing research)
[2021, 404:108227]

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In vivo gene editing of outer hair cells prevents progressive hearing loss in a dominant-negative KCNQ4 murine model

Byunghwa Noh, John Hoon Rim, Ramu Gopalappa, Haiyue Lin, Kyu Min Kim, Heon Yung Gee, Jae Young Choi, Hyongbum Henry Kim, Jinsei Jung,

Adult-onset hearing loss (AHL)—including presbycusis—caused by outer hair cell (OHC) degeneration is the most common sensorial disorder. Despite the high prevalence of AHL and wide therapeutic window, no targeted therapy is currently available. Here, we generated a mouse model harboring Kcnq4W276S/+ to recapitulate DFNA2, a common genetic form of progressive ... Read more >>

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Relationship between Hearing Loss and Dementia Differs According to the Underlying Mechanism.

Jinsei Jung, Seong Hoon Bae, Ji Hyuk Han, Sang Hyun Kwak, Gi Sung Nam, Phil Hyu Lee, Young Ho Sohn, Mijin Yun, Byung Seok Ye,

<h4>Background and purpose</h4>The associations between hearing loss (HL) and the mechanisms underlying cognitive impairment (CI) remain unclear. We evaluated the effects of clinical factors, vascular magnetic resonance imaging (MRI) markers, and CI mechanisms on HL.<h4>Methods</h4>In total, 112 patients with CI (59% demented) and subjective HL prospectively underwent MRI, amyloid positron-emission ... Read more >>

J Clin Neurol (Journal of clinical neurology (Seoul, Korea))
[2021, 17(2):290-299]

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Effects of Contralateral Routing of Signal Hearing Aids on Audiological and Academic Performance in School-Age Children With Unilateral Hearing Loss.

Sang Hyun Kwak, Daeyoung Kim, Seong Hoon Bae, In Seok Moon, Sung Huhn Kim, Jae Young Choi, Jinsei Jung,

Clin Exp Otorhinolaryngol (Clinical and experimental otorhinolaryngology)
[2021, 14(3):355-358]

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Activation of KCNQ4 as a Therapeutic Strategy to Treat Hearing Loss.

John Hoon Rim, Jae Young Choi, Jinsei Jung, Heon Yung Gee,

Potassium voltage-gated channel subfamily q member 4 (KCNQ4) is a voltage-gated potassium channel that plays essential roles in maintaining ion homeostasis and regulating hair cell membrane potential. Reduction of the activity of the KCNQ4 channel owing to genetic mutations is responsible for nonsyndromic hearing loss, a typically late-onset, initially high-frequency ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2021, 22(5):]

Cited: 2 times

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Neutrophils infiltrate into the spiral ligament but not the stria vascularis in the cochlea during lipopolysaccharide-induced inflammation.

Seong Hoon Bae, Jee Eun Yoo, Young Ho Choe, Sang Hyun Kwak, Jae Young Choi, Jinsei Jung, Young-Min Hyun,

It has been challenging to apply intravital imaging for monitoring the inner ear, as the anatomical location and intricate structure hamper the access of imaging instruments to the inner ear of live mice. By employing intravital imaging of the cochlea in live mice with two-photon microscopy, we investigated neutrophil infiltration ... Read more >>

Theranostics (Theranostics)
[2021, 11(6):2522-2533]

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One-stage cochlear implantation in patients with chronic otitis media using canal wall up mastoidectomy.

Yeonsu Jeong, Young Kyun Hur, Jae Young Choi, Sung Huhn Kim, In Seok Moon, Jinsei Jung, Chan Il Song,

<h4>Background</h4>Cochlear implantation (CI) with subtotal petrosectomy was recommended to avoid the complications for patients with chronic otitis media (COM).<h4>Objectives</h4>To evaluate the surgical outcomes of CI in patients with COM using a one-stage operation with canal wall up mastoidectomy (CWUM).<h4>Methods</h4>Thirty-five patients with COM who underwent CI with CWUM as a one-stage ... Read more >>

Acta Otolaryngol (Acta oto-laryngologica)
[2021, 141(4):354-358]

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The Effects of Preoperative Audiovisual Speech Perception on the Audiologic Outcomes of Cochlear Implantation in Patients with Postlingual Deafness.

Hyun Jin Lee, Jeon Mi Lee, Jae Young Choi, Jinsei Jung,

<h4>Introduction</h4>Patients with postlingual deafness usually depend on visual information for communication, and their lipreading ability could influence cochlear implantation (CI) outcomes. However, it is unclear whether preoperative visual dependency in postlingual deafness positively or negatively affects auditory rehabilitation after CI. Herein, we investigated the influence of preoperative audiovisual per-ception on ... Read more >>

Audiol Neurootol (Audiology & neuro-otology)
[2021, 26(3):149-156]

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Tinnitus and Its Association With Mental Health and Health-Related Quality of Life in an Older Population: A Nationwide Cross-Sectional Study.

Hye-Min Park, Jinsei Jung, Jong-Koo Kim, Yong-Jae Lee,

This study investigated the relationship of tinnitus with mental health and health-related quality of life (QoL) in older people. Data source included 5,129 community-dwelling men and women ≥60 years old from the Korean National Health and Nutrition Examination Survey. Tinnitus was categorized into three groups: normal, tolerable tinnitus, and annoying ... Read more >>

J Appl Gerontol (Journal of applied gerontology : the official journal of the Southern Gerontological Society)
[2020, :733464820966512]

Cited: 2 times

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Synergistic effect of smoking on age-related hearing loss in patients with diabetes.

Seong Hoon Bae, Sang Hyun Kwak, Jae Young Choi, Jinsei Jung,

This study investigated the synergistic effects of risk factors on age-related hearing loss (ARHL) using nationwide cross-sectional data of 33,552 individuals from the 2010‒2013 Korea National Health and Nutrition Examination Survey. Patients with ARHL were selected based on their pure-tone audiometry results. Previously reported risk factors for ARHL were analyzed ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):18893]

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Feasibility of Asynchronous and Automated Telemedicine in Otolaryngology: Prospective Cross-Sectional Study.

Dongchul Cha, Seung Ho Shin, Jungghi Kim, Tae Seong Eo, Gina Na, Seonghoon Bae, Jinsei Jung, Sung Huhn Kim, In Seok Moon, Jaeyoung Choi, Yu Rang Park,

<h4>Background</h4>COVID-19 often causes respiratory symptoms, making otolaryngology offices one of the most susceptible places for community transmission of the virus. Thus, telemedicine may benefit both patients and physicians.<h4>Objective</h4>This study aims to explore the feasibility of telemedicine for the diagnosis of all otologic disease types.<h4>Methods</h4>A total of 177 patients were prospectively ... Read more >>

JMIR Med Inform (JMIR medical informatics)
[2020, 8(10):e23680]

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Cochlin-cleaved LCCL is a dual-armed regulator of the innate immune response in the cochlea during inflammation.

Hyeong-Jun Rhyu, Seong Hoon Bae, Jinsei Jung, Young-Min Hyun,

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 ... Read more >>

BMB Rep (BMB reports)
[2020, 53(9):449-452]

Cited: 1 time

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Effect of Cochlear Implantation on Hearing Fluctuation in Patients with Biallelic SLC26A4 Variants.

Gina Na, Jeon Mi Lee, Hyun Jin Lee, Yeonsu Jeong, Jinsei Jung, Jae Young Choi,

<h4>Introduction</h4>Fluctuating hearing loss is a distinctive feature caused by SLC26A4 variants. We investigated whether cochlear implantation had protective or deleterious effect on hearing fluctuation in patients with biallelic SLC26A4 variants.<h4>Methods</h4>Patients with biallelic SLC26A4 variants (N = 16; age = 10.24 ± 9.20 years) who had unilateral cochlear implantation and consecutive ... Read more >>

Audiol Neurootol (Audiology & neuro-otology)
[2021, 26(2):111-120]

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Three-Dimensional Distribution of Cochlear Macrophages in the Lateral Wall of Cleared Cochlea.

Seong Hoon Bae, Sang Hyun Kwak, Jee Eun Yoo, Kyu Min Kim, Young Min Hyun, Jae Young Choi, Jinsei Jung,

<h4>Objectives</h4>Resident macrophages are well known to be present in the cochlea, but the exact patterns thereof in spiral ligaments have not been discussed in previous studies. We sought to document the distribution of macrophages in intact cochleae using three-dimensional imaging.<h4>Methods</h4>Cochleae were obtained from C-X3-C motif chemokine receptor 1+/GFP mice, and ... Read more >>

Clin Exp Otorhinolaryngol (Clinical and experimental otorhinolaryngology)
[2021, 14(2):179-184]

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Investigation of intact mouse cochleae using two-photon laser scanning microscopy.

Seong Hoon Bae, Sang Hyun Kwak, Young Ho Choe, Young-Min Hyun, Jae Young Choi, Jinsei Jung,

<h4>Objectives</h4>The investigation of cochlear hair cells and lateral wall is a time-consuming and labor-intensive process. However, it is a mandatory experiment in audiology research. Here we suggest a novel method for investigating the inner ear microstructures from intact cochleae using two-photon laser scanning microscopy (TPLSM). This technique guarantees fewer artifacts ... Read more >>

Microsc Res Tech (Microscopy research and technique)
[2020, 83(10):1235-1240]

Cited: 2 times

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Bicarbonate permeation through anion channels: its role in health and disease.

Dong Hoon Shin, Minjae Kim, Yonjung Kim, Ikhyun Jun, Jinsei Jung, Joo Hyun Nam, Mary Hongying Cheng, Min Goo Lee,

Many anion channels, frequently referred as Cl<sup>-</sup> channels, are permeable to different anions in addition to Cl<sup>-</sup>. As the second-most abundant anion in the human body, HCO<sub>3</sub><sup>-</sup> permeation via anion channels has many important physiological roles. In addition to its classical role as an intracellular pH regulator, HCO<sub>3</sub><sup>-</sup> also controls ... Read more >>

Pflugers Arch (Pflugers Archiv : European journal of physiology)
[2020, 472(8):1003-1018]

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The Era of Precision Medicine: Reshaping Usher Syndrome.

Jinsei Jung,

Clin Exp Otorhinolaryngol (Clinical and experimental otorhinolaryngology)
[2020, 13(2):87-88]

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

Woongsu Han, Jeong-Oh Shin, Ji-Hyun Ma, Hyehyun Min, Jinsei Jung, Jinu Lee, Un-Kyung Kim, Jae Young Choi, Seok Jun Moon, Dae Won Moon, Jinwoong Bok, Chul Hoon Kim,

Outer hair cells (OHCs) play an essential role in hearing by acting as a nonlinear amplifier which helps the cochlea detect sounds with high sensitivity and accuracy. This nonlinear sound processing generates distortion products, which can be measured as distortion-product otoacoustic emissions (DPOAEs). The OHC stereocilia that respond to sound ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2020, 117(20):11109-11117]

Cited: 2 times

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Genetic Inheritance of Late-Onset, Down-Sloping Hearing Loss and Its Implications for Auditory Rehabilitation.

Mee Hyun Song, Jinsei Jung, John Hoon Rim, Hye Ji Choi, Hack June Lee, Byunghwa Noh, Jun Suk Lee, Heon Yung Gee, Jae Young Choi,

<h4>Objectives</h4>Late-onset, down-sloping sensorineural hearing loss has many genetic and nongenetic etiologies, but the proportion of this commonly encountered type of hearing loss attributable to genetic causes is not well known. In this study, the authors performed genetic analysis using next-generation sequencing techniques in patients showing late-onset, down-sloping sensorineural hearing loss ... Read more >>

Ear Hear (Ear and hearing)
[2020, 41(1):114-124]

Cited: 1 time

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Is Early Progression to Bilateral Involvement in Menière's Disease a Poor Prognostic Indicator?

Hyun Jin Lee, Jeon Mi Lee, Dae Bo Shim, Jinsei Jung, Sang Hyun Kwak, Sung Huhn Kim,

OBJECTIVE:To investigate differences in the clinical features of bilateral Menière's disease (BMD) with early and late bilateral involvement. STUDY DESIGN:A retrospective case-comparison study. SETTING:Tertiary referral center. PATIENTS:In total, 25 patients with definite BMD were enrolled. Patients with bilateral involvement with a transition interval shorter than 1 month were considered to ... Read more >>

Otol Neurotol (Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology)
[2019, 40(10):1333-1338]

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