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Clinical and Genetic Spectrum of ATP1A3-Related Disorders in a Korean Pediatric Population

Kim WJ, Shim YK, Choi SA, Kim SY, Kim H, Hwang H, Choi J, Kim KJ, Chae JH, Lim BC

BACKGROUND AND PURPOSE: The aim of this study was to expand the understanding of the genotype-phenotype spectrum of ATP1A3-related disorders and to evaluate the therapeutic effect of a ketogenic diet...
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Applications of photobiomodulation in hearing research: from bench to clinic

Lee JH, Kim S, Jung JY, Lee MY

Hearing loss is very common and economically burdensome. No accepted therapeutic modality for sensorineural hearing loss is yet available; most clinicians emphasize rehabilitation, placing hearing aids and cochlear implants. Photobiomodulation...
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A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent

Lee YW, Lee J, Kim MB, Chang SO

Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular...
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Decreased Aquaporin 4 and 6 mRNAs in Patients With Chronic Otitis Media With Otorrhea

Kim JH, Kim SS, Kim YI, Jung SY, Kim SH, Yeo SG

OBJECTIVES: Aquaporins (AQPs) are integral membrane proteins engaged in the modulation of water homeostasis, but the roles they play in chronic otitis media (COM) have not been well investigated. Accordingly,...
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Effects of Residual Hearing on the Auditory Steady State Response for Cochlear Implantation in Children

Kim YS, Han SA, Woo H, Suh MW, Lee JH, Oh SH, Park MK

BACKGROUND AND OBJECTIVES: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). SUBJECTS AND METHODS:...
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Comparison of Intratympanic, Systemic, and Combined Steroid Therapies for Sudden Sensorineural Hearing Loss in Patients with Diabetes Mellitus

Kim HJ, Lee YJ, Kang HT, Lee SA, Lee JD, Kim BG

BACKGROUND AND OBJECTIVES: We investigated whether the intratympanic steroid injections might be an effective initial treatment for sudden sensorineural hearing loss (SSNHL) in patients with diabetes mellitus (DM). SUBJECTS AND METHOD:...
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Characteristic of Sudden Sensorineural Hearing Loss with Objective Vestibular Involvement

Choi KY, Kim MT, Choi JE, Jung JY, Lee MY

BACKGROUND AND OBJECTIVES: To analyze the difference in audiologic-vestibular and clinical characteristics between acute cochlea-vestibulopathy (ACV) and idiopathic sudden sensorineural hearing loss (ISSNHL). SUBJECTS AND METHOD: We retrospectively analyzed the clinical...
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Duplicated Internal Auditory Canal: High-Resolution CT and MRI Findings

Wang L, Zhang L, Li X, Guo X

OBJECTIVE: To summarize the high-resolution computed tomography (HRCT) and magnetic resonance imaging (HRMRI) features of duplicated internal auditory canals (DIACs). MATERIALS AND METHODS: Ear HRCT data of 64813 patients with sensorineural...
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Hearing Outcome of Patients with Acute Noise-Induced Hearing Loss

Choi BG, Hong SK, Kim HJ, Lee HJ

BACKGROUND AND OBJECTIVES: Although poor hearing outcomes have been associated with acute noise-induced hearing loss (ANIHL), only limited studies exist on this issue. This study evaluated the prognosis of ANIHL...
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A Case of Bilateral Semicircular Canal Dysplasia with Normal Cochlear Found in Bilateral Vestibulopathy

Lee S, Choi SW

A 25-year-old woman presented with atypical dizziness with oscillopsia. The neuro-otological evaluations showed bilateral vestibulopathy with mild sensorineural hearing loss. Computed tomography and magnetic resonance imaging demonstrated bilateral isolated lateral...
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Comparison of Treatment Outcomes between Intratympanic Steroid Injection and Oral Diuretics in Patients with Acute Low Frequency Sensorineural Hearing Loss with Vertigo

An YH, Shim HJ

OBJECTIVES: Sudden sensorineural hearing loss and Meniere disease can be managed with intratympanic steroid injection (ITSI) as a primary treatment. The aim of this study was to compare the effectiveness...
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The Value of Posterior Semicircular Canal Function in Predicting Hearing Recovery of Sudden Sensorineural Hearing Loss

Shin JW, Kim SW, Kim YW, Jang W, Kim BH, Lim YS, Park SW, Cho CG, Park JH

OBJECTIVES: Postulated etiologies for idiopathic sudden sensorineural hearing loss (SSNHL) include viral cochleitis, microvascular events. If SSNHL is caused by vascular compromise of common cochlear artery that supplies cochlea and...
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Clinical and genetic characteristics of Korean patients with IARS2-related disorders

Lee JS, Kim MJ, Kim SY, Lim BC, Kim KJ, Choi M, Seong MW, Chae JH

PURPOSE: Genetic defects in the nuclear-encoded mitochondrial aminoacyl-tRNA synthetases were first identified as causes of various disorders in 2007. Variants in IARS2, which encodes a mitochondrial isoleucyl-tRNA synthetase, were first...
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Comparison of the Results between the First Steroid Treatment Group and the Retreatment Group with Idiopathic Sudden Sensorineural Hearing Loss

Ko JB, Kim CH, Jo KH, Lee HH, Kwon JH

BACKGROUND AND OBJECTIVES: Sudden sensoryneural hearing loss (SSNHL) is an otological emergency disease of a non-specific cause. If there is no improvement or if the degree of hearing loss is...
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Two Cases of Localized Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Manifested with Intractable Otitis Media

Lee E, Chung WH

Localized form of myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis (MPO-AAV) in the ear is a rare disease. It manifests as a sudden otalgia with hearing loss accompanied by dizziness and facial nerve...
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Response of Glucocorticoid Receptor Alpha and Histone Deacetylase 2 to Glucocorticoid Treatment Predicts the Prognosis of Sudden Sensorineural Hearing Loss

Zhang X, Chen J, Gao Z, Qi H, Dai Y, She W

OBJECTIVES: To investigate glucocorticoid receptor (GR) and histone deacetylase 2 (HDAC2) gene expression and protein levels in peripheral blood mononuclear cells (PBMCs) of patients with severe or profound sudden sensorineural...
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Prognosis of Sudden Low Frequency Hearing Loss during Long-term Follow-up

Kim JH, Kwak SH, Bae SH, Kim SH, Nam GS

OBJECTIVES: The prognosis of sudden low frequency hearing loss (SLFHL) is relatively good, but recurrences of hearing loss and possible progression to Meniere's disease is still a clinically important concern....
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Incidence of Progression into Ménière Disease from Idiopathic Sudden Sensorineural Hearing Loss: Midterm Follow-up Study

Lee BM, Seo JH, Park HW, Lee HJ, Hur DG, Ahn SK

OBJECTIVES: Ménière disease is a clinical syndrome characterized by the four major symptoms of episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. Sensorineural hearing loss, especially low frequency, is...
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Vestibular Function and Prognosis of Sudden Sensorineural Hearing Loss with Vertigo

Lee SA, Kim HJ, Kim BG, Lee JD

OBJECTIVES: Vertigo combined with sudden sensorineural hearing loss (SSNHL) is known as a poor prognostic factor. We investigated clinical findings and vestibular function tests in patients of SSNHL with vertigo...
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The Significance of Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio as Markers for Prognosis in Idiopathic Sudden Sensorineural Hearing Loss

Yi KI, Oh SJ, Choi SW, Kim SL, Kong SK, Goh EK

BACKGROUND AND OBJECTIVES: The cause of idiopathic sudden sensorineural hearing loss (ISSNHL) is still unclear, but recently, chronic inflammation and thrombosis have received attention. The neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte...
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