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A Novel Heterozygous Missense Variant (c.667G>T;p.Gly223Cys) in USH1C That Interferes With Cadherin-Related 23 and Harmonin Interaction Causes Autosomal Dominant Nonsyndromic Hearing Loss

Song JS, Bahloul A, Petit C, Kim SJ, Moon IJ, Lee J, Ki CS

BACKGROUND: Pathogenic variants of USH1C, encoding a PDZ-domain-containing protein called harmonin, have been known to cause autosomal recessive syndromic or nonsyndromic hearing loss (NSHL). We identified a causative gene in...
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Cortical Deafness and Tinnitus Following Sequential Bilateral Putaminal Hemorrhage

Yang S, Chen S, Zhu J, Han X

No abstract available.
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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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The Influence of Education Level on Tinnitus and Quality of Life in Korean Adults

Jung DJ, Yoo MH, Jung YJ, Lee HJ, Lee KY

BACKGROUND AND OBJECTIVES: We aimed to evaluate the effect of the level of education on the prevalence of tinnitus and quality of life (QoL) in the Korean population. SUBJECTS AND METHOD:...
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Two Cases of Pediatric Pneumolabyrinth with Traumatic Tympanic Membrane Perforation after Penetrating Injury

You YS, Koh JH, Kim BJ, Lee EJ

Pneumolabyrinth is an uncommon condition in which air is present in the vestibule or cochlear. It is rarely found, even in otic capsule violating fractures or in transverse fracture of...
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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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Correlation between Auditory Brainstem Response Characteristics and Hearing Recovery in Sudden Deafness

Lim EJ, Shin J, Kim TH, Park JH, Kim S

BACKGROUND AND OBJECTIVES: Auditory brainstem response (ABR) can be utilized to verify the hearing threshold and determine the existence of retrocochlear pathology in sudden deafness. However, little is known about...
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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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Bilateral Hearing Loss in Wernicke Encephalopathy

Bae H, Park JY, Cho HS, Lim SH, Ha SW

Wernicke encephalopathy is a syndrome caused by thiamine deficiency whose three typical symptoms are ophthalomoplegia, ataxia, and confusion. There are also rare reports of bilateral hearing loss, which can be...
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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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Educational Status in Bilateral Prelingual Deaf Children with Cochlear Implantation

Bae SH, Kwak SH, Nam GS, Choi JY

BACKGROUND AND OBJECTIVES: This study was undertaken to investigate the educational status in bilateral prelingual deaf children with a cochlear implant (CI), also known as early cochlear implantees (CIs). Type...
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Translation, Adaptation and Cross-Cultural Validation of Hearing Handicap Inventory for Adult in Malay Language

Zam Zam TZ, Dzulkarnain AAA, Rahmat S, Jusoh M

BACKGROUND AND OBJECTIVES: Sine a self-reported questionnaire for hearing-impaired listeners is not available by Malay language yet, it is important to develop or translate any available existing questionnaires. The aim of...
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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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Safety and Effectiveness of Endoscopic Ear Surgery: Systematic Review

Jeon MH, Chung S, Kim SH, Oh SH, Im GJ, Kim JR, Choi J, Lee BD

BACKGROUND AND OBJECTIVES: This study aims to evaluate that usefulness of the endoscopic ear surgery (EES) through the systematic review. SUBJECTS AND METHOD: We searched literatures in literature databases (MEDLINE, EMBASE,...
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The First Case of Otitis in Korea Caused by Brevibacterium otitidis

Mun SH, Lee AJ, Kim SG, Jeon CH, Park CG, Suh HS

Brevibacterium spp. are gram-positive rods that are considered to be strictly nonpathogenic, and a very few cases of their infection in humans have been reported. In this study, we report...
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Concurrent SHORT syndrome and 3q duplication syndrome

Boaz AM, Grasso SA, DeRogatis , Beesley EN

SHORT syndrome is an extremely rare congenital condition due to a chromosomal mutation of the PIK3R1 gene found at 5q13.1. SHORT is a mnemonic representing six manifestations of the syndrome:...
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Clinical Analysis of External Auditory Canal Osteoma

Kim YS, Kang MH, Choi YS, Shin SO, Jung HJ

BACKGROUND AND OBJECTIVES: External auditory canal osteoma is an uncommon benign lesion and represents as unilateral solitary pedunculated mass. The aim of this study is to evaluate the clinical symptoms...
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Predictors of Accidental Falls in the Community-dwelling Elderly by Age

Park NJ, Shin YS

PURPOSE: The purpose of this study is to identify risk factors of accidental falls by age in the Korean elderly. METHODS: The study subjects included 10,242 community-dwelling elders aged 65 or...
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Asymptomatic Hearing Impairment Frequently Occurs in Early-Onset Parkinson's Disease

Shetty K, Krishnan S, Thulaseedharan JV, Mohan M, Kishore A

OBJECTIVE: Recent reports of hearing impairment in Parkinson's disease (PD) have suggested that auditory dysfunction could be a non-motor manifestation of PD. These reports were based on observations of elderly...
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