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Speech Perception and Gap Detection Performance of Single-Sided Deafness under Noisy Conditions

Kwak C, Kim S, Lee J, Seo Y, Kong T, Han W

BACKGROUND AND OBJECTIVES: Many studies have reported no benefit of sound localization, but improved speech understanding in noise after treating patients with single-sided deafness (SSD). Furthermore, their performances provided a...
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Prediction of Cochlear Implant Outcomes in Patients With Prelingual Deafness

Kang DH, Lee MJ, Lee KY, Lee SH, Jang JH

OBJECTIVES: To evaluate the factors that limit post-cochlear implantation (CI) speech perception in prelingually deaf children. METHODS: Patients with CI were divided into two groups according to Category of Auditory Performance...
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MicroRNA-183 Family in Inner Ear: Hair Cell Development and Deafness

Mahmoodian sani MR, Hashemzadeh-Chaleshtori M, Saidijam M, Jami MS, Ghasemi-Dehkordi P, Saidijam M, Jami MS

miRNAs are essential factors of an extensively conserved post-transcriptional process controlling gene expression at mRNA level. Varoius biological processes such as growth and differentiation are regulated by miRNAs. Web of...
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Cordblood-Based High-Throughput Screening for Deafness Gene of 646 Newborns in Jinan Area of China

Li SX, Chen DL, Zhao SB, Guo LL, Feng HQ, Zhang XF, Ping LL, Yang ZM, Sun CX, Yao GD

OBJECTIVES: Infants with slight/mild or late-onset hearing impairment might be missed in universal newborn hearing screening (UNHS). We identified the mutation hot spot of common deaf gene in the newborns...
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Usefulness of Objective Hearing Tests for Screening Patients with Partial Deafness

Yu MJ, Mun HA, Lee JJ, Kang YS, Hong SA, Lim HJ, Park HY, Choung YH

BACKGROUND AND OBJECTIVES: 'Partial deafness' characterized by normal or slightly impaired hearing in the low frequency band and nearly total deafness in the high frequency range, is difficult to assess...
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Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome with a GATA3 mutation

Shim YS, Choi W, Hwang IT, Yang S

Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome is an autosomal dominant disease caused by mutations in the GATA3 gene on chromosome 10p15. We identified a patient diagnosed with hypoparathyroidism who...
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Preoperative Vestibular Function in Adults with Cochlear Implantation: Comparison between Prelingual and Poslingual Deafness

Han JJ, Hong S, Park H, Koo JW, Lee JH, Oh SH, Chang SO, Lee MY, Suh MW

BACKGROUND AND OBJECTIVES: Cochlear system and vestibular system have close relationship anatomically and developmentally. According to previous literatures, there are high incidences of vestibular dysfunction in subjects with severe hearing...
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Carrier frequency of SLC26A4 mutations causing inherited deafness in the Korean population

Kim H, Lim HS, Ryu JS, Kim HC, Lee S, Kim YT, Kim YJ, Lee KR, Park HJ, Han SH

PURPOSE: The mutation of the SLC26A4 gene is the second most common cause of congenital hearing loss after GJB2 mutations. It has been identified as a major cause of autosomal...
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Molecular Genetic Diagnosis of Deafness: Current and Future

Oh SH, Choi BY

Advances in molecular biology and molecular genetic technologies have revealed extreme etiologic heterogeneity of genetic hearing loss. Genes known to contribute to deafness have been reported to be involved with...
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Brainstem auditory evoked potential findings in a French bulldog with bilaterally congenital sensorineural deafness

An D, Jung DI, Kim HJ, Kang JH, Chang DW, Yang MP, Kang BT

  • KMID: 1790576
  • Korean J Vet Res.
  • 2013 Dec;53(4):265-267.
A 3-month-old, intact male French bulldog was suspected of deafness. The dog was irresponsive to environmental noises generated out of sight, but normal responses were noted for visual stimuli. No...
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A Case of MELAS with Maternally Transmitted Type II Diabetes Mellitus and Deafness

Yu HJ, Roh SY, Lee KE, Koh SH

  • KMID: 2343269
  • J Korean Neurol Assoc.
  • 2007 Aug;25(3):416-418.
MELAS syndrome is typically a multisystemic disorder. We report one case of MELAS showing both maternally transmitted type II diabetes mellitus (DM) and deafness. A 41-year-old woman was admitted because...
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A Case of Single Photon Emission Computerized Tomographic Finding in Patient with Acquired Deafness and Musical Hallucinosis after Viral Encephalitis

Cho GH, Chung YC, Jung HJ

  • KMID: 2286732
  • Korean J Psychopharmacol.
  • 2007 Jan;18(1):60-64.
OBJECTIVE: Musical hallucinosis is uncommon symptom in the province of neuropsychiatry. Musical hallucinosis is often accompanied with hearing impairment caused by physical illness and is characterized by no psychotic symptoms....
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Pure word deafness in a patient with systemic lupus erythematosus

Kim YJ, Kim HR, Park SN, Yoon CH, Lee SH, Park SH, Kim HY

  • KMID: 1652690
  • Korean J Med.
  • 2004 Apr;66(4):420-424.
Pure word deafness is characterized by an impairment of auditory comprehension and repetition of speech while a preservation of reading, writing, spontaneous speech and ability to comprehend non-verbal sounds. It...
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Biological Analysis of a New Spontaneous Mutant Mouse Showing Deafness and Circling Behavior

Cho DY, Kim MS, Chung WH, Ryoo ZY, Hong SH

  • KMID: 2276097
  • Korean J Otolaryngol-Head Neck Surg.
  • 2004 Feb;47(2):115-126.
BACKGROUND AND OBJECTIVES: Deafness is the most common sensory deficit and hereditary defect in human populations. The present study investigated the causative gene in circling mice using the complementation test....
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A Case of Pure Word Deafness in MELAS Syndrome

Shin DS, Kwon OS, Lee HO, Youn YC

  • KMID: 2137946
  • J Korean Neurol Assoc.
  • 2004 Oct;22(5):520-523.
An 18-year-woman was referred with seizure activity and global aphasia. Diagnosis of MELAS syndrome with left temporo-parieto-occipital infarction was confirmed by gene analysis. Global aphasia was improved completely. Right temporal...
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A Case of a Family with Knuckle Pads, Deafness and Palmoplantar Hyperkeratosis

Kim YT, Kim WS, Park YL, Cho MK, Whang KU

  • KMID: 2038624
  • Korean J Dermatol.
  • 2003 Aug;41(8):1057-1060.
The syndrome with knuckle pads, leukonychia, deafness and palmoplantar hyperkeratosis have been presented by several previous reports since the Bart and Pumphrey's report. This syndrome is that inheritance pattern is...
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Klippel-Feil Syndrome with Bilateral Congenital Aural Atresia and Bilateral Congenital Deafness

Park CW, Jang YH, Muhn DS, Tae K, Ahn KS

  • KMID: 2274396
  • Korean J Otolaryngol-Head Neck Surg.
  • 1998 Feb;41(2):261-265.
BACKGROUND AND OBJECTIVES: The Klippel-Feil syndrome is a congenital anomaly characterized by fusion of the cervical vertebrae. It is often associated with serious congenital anomalies of the nervous, cardivascular, respiratory,...
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A Case of Congenital Long QT Syndrome Associated with Deafness and Syncope

Lee SM, Choe CW, Kang HS, Kim KS, Song JS, Bae JH

Congenital long QT syndrome (LQTS) is an inherited disease characterized by prolonged QT intervals and polymorphic ventricular tachycardia. The clinical manifestations vary from sudden cardiac death by ventricular arrhythmia to...
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A case of pure word deafness

Sung SM, Kim SH, Park KH

  • KMID: 2442965
  • J Korean Neurol Assoc.
  • 1997 Apr;15(2):377-381.
Pure word deafness refers to an inability to understand spoken language with relatively normal reading, writing and speaking as well as comprehension of nonverbal sounds. We report a case of...
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Cognitive Ability in Total Deafness with K-WISC

Han KW, Ye MK, Chang HW, Huh MJ, Lee SH, Cho TH

  • KMID: 1922556
  • Korean J Otolaryngol-Head Neck Surg.
  • 1997 Mar;40(3):340-346.
The criteria of suitability for a cochlear implant has been extended postlingual deaf adult including prelingual deaf children in their difficulty with speech and vocabulary development. The cognitve ability of...
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