• Molecules and Cells
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• 제38권9호
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• pp.781-788
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• 2015

Mutations of MYO15A are generally known to cause severe to profound hearing loss throughout all frequencies. Here, we found two novel MYO15A mutations, c.3871C>T (p.L1291F) and c.5835T>G (p.Y1945X) in an affected individual carrying congenital profound sensorineural hearing loss (SNHL) through targeted resequencing of 134 known deafness genes. The variant, p.L1291F and p.Y1945X, resided in the myosin motor and IQ2 domains, respectively. The p.L1291F variant was predicted to affect the structure of the actin-binding site from three-dimensional protein modeling, thereby interfering with the correct interaction between actin and myosin. From the literature analysis, mutations in the N-terminal domain were more frequently associated with residual hearing at low frequencies than mutations in the other regions of this gene. Therefore we suggest a hypothetical genotype-phenotype correlation whereby MYO15A mutations that affect domains other than the N-terminal domain, lead to profound SNHL throughout all frequencies and mutations that affect the N-terminal domain, result in residual hearing at low frequencies. This genotype-phenotype correlation suggests that preservation of residual hearing during auditory rehabilitation like cochlear implantation should be intended for those who carry mutations in the N-terminal domain and that individuals with mutations elsewhere in MYO15A require early cochlear implantation to timely initiate speech development.

• 한국음향학회지
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• 제31권5호
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• pp.298-308
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• 2012

This study was designed to examine the phoneme recognition errors of hearing-impaired (HI) listeners on a consonant-by-consonant basis, to show (1) how each HI ear perceives individual consonants differently and (2) how standard clinical measurements (i.e., using a tone and word) fail to predict these differences. Sixteen English consonant-vowel (CV) syllables of six signal-to-noise ratios in speech-weighted noise were presented at the most comfortable level for ears with mild-to-moderate sensorineural hearing loss. The findings were as follows: (1) individual HI listeners with a symmetrical pure-tone threshold showed different consonant-loss profiles (CLPs) (i.e., over a set of the 16 English consonants, the likelihood of misperceiving each consonant) in right and left ears. (2) A similar result was found across subjects. Paired ears of different HI individuals with identical pure-tone threshold presented different CLPs in one ear to the other. (3) Paired HI ears having the same averaged consonant score demonstrated completely different CLPs. We conclude that the standard clinical measurements are limited in their ability to predict the extent to which speech perception is degraded in HI ears, and thus they are a necessary, but not a sufficient measurement for HI speech perception. This suggests that the CV measurement would be a useful clinical tool.

• 한방안이비인후피부과학회지
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• 제16권1호
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• pp.206-213
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• 2003

The sudden deafness is generaly defined as sensorineural hearing loss without definite cause. The medical treatment of this disease is purposed on hemokinetic improvement and anti-inflammatory action. There are various prognoses such as perfect recovery or loss of hearing. The symptoms are easily redeveloped by fatigue or psychological stress. We treated two patients, who diagnosed as the flare of Liver fire and the flare based on insufficiency of Kidney Yin, of sudden deafness with herbal medication, acupuncture, moxibustion and herbal acupuncture. The symptoms were improved after three or four times of treatment.

• 한방안이비인후피부과학회지
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• 제33권2호
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• pp.23-42
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• 2020

Objectives : This study is designed to analyze the outpatients who received medical treatments for ear diseases in ophthalmology & otolaryngology & dermatology clinic of Daejeon Korean Medical Hospital from March, 2013 to February, 2019. Methods : We classified the otology outpatients who received medical treatments in ophthalmology & otolaryngology & dermatology clinic of Daejeon Korean Medical Hospital from March, 2012 to February, 2019 according to gender, year, age, season, anatomical parts, and main diagnosis. The statistical analysis was performed using SPSS 25.0. Results : 1. The number of outpatients with ear diseases were 880; 505 female and 375 male. The number of female patients were approximately 1.7 times higher. 2. The average age of the outpatients with ear diseases were 50.0. The number of middle-aged (40-50s) patients were 439, which is almost half the number(49.9%) of the whole patients. 3. By analyzing the number of patients classified by year, we found out that the number of the outpatients with ear diseases has increased gradually for the past 7 years. 4. In the analysis of the number of patients classified by the anatomical parts of the ear, the inner ear diseases group were the largest, amounting to 79.3% of the whole patients. 5. In the analysis of the number of patients classified by main diagnosis, Tinnitus turns out to be the largest group with 338 patients, followed by sudden sensorineural hearing loss, dizziness, otalgia, Meniere's syndrome, otitis, auditory tube dysfunction, BPPV. 6. No statistical-significant difference were shown in the analysis of the number of patients classified by season. Conclusions : It turns out that patients with ear diseases had increased by the years, and middle aged patients (40-50s) were the largest among the age groups. Most of the patients came for the inner-ear diseases, and tinnitus, sudden sensorineural hearing loss took up the largest proportions.

• 음성과학
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• 제7권1호
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• pp.77-87
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• 2000

Distortion Product Otoacoustic Emissions (DPOAEs) can be measured in the external ear canal two fold: amplitude and latency, but most DPOAE studies deal with amplitude aspects. The purpose of this study was to investigate the latency of the 2f1-f2 DPOAEs in ears with hearing losses and to see if it could be a clinically useful method to distinguish normal from abnormal ears. For this purpose, DPOAE latency were measured as a function of frequency from 1 to 8 kHz in 30 ears with conductive and sensorineural hearing losses (SNHLs). DPOAEs were recorded with Otodynamic Analyzer ILO92. Results showed that the latency decreased as the frequency increased up to 8 kHz. The mean values of DPOAE latency for ears of SNHLs were shorter at all frequencies when they were compared to the mean values of normal ears. The latency in ears of conductive hearing losses was shorter than normal ears at the selective frequencies, as well. The results support the hypothesis that latency values are shorter in pathological ears.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.10-13
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• 2016

Chromosome 11q13 deletion syndrome has been previously reported as either otodental syndrome or oculo-oto-dental syndrome. The otodental syndrome is characterized by dental abnormalities and high-frequency sensorineural hearing loss, and by ocular coloboma in some cases. The underlying genetic defect causing otodental syndrome is a hemizygous microdeletion involving the FGF3 gene on chromosome 11q13.3. Recently, a new form of severe deafness, microtia (small ear) and small teeth, without the appearance of eye abnormalities, was also reported. In this report, we describe a 1-year-old girl presenting with ptosis of the left upper eyelid, right auricular deformity, high-arched palate, delayed dentition, simian line on the right hand, microcephaly, and developmental delay. In this patient, we identified a deletion in the chromosome 11q13.2-q13.3 (2.75 Mb) region by using an array-comparative genomic hybridization analysis. The deletion in chromosome 11q13 results in a syndrome characterized by variable clinical manifestations. Some of these manifestations involve craniofacial dysmorphology and require a functional workup for hearing, ophthalmic examinations, and long-term dental care.

• 디지털콘텐츠학회 논문지
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• 제11권4호
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• pp.425-431
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• 2010

본 연구에서는 돌발성 난청을 보이는 환자를 대상으로 임상에서 자주 사용 하는 기법인 3D FSE과 새로운 기법인 3D FIESTA-C 을 정량적으로 비교 분석하여 3D FIESTA 기법의 효과와 유용성을 고찰해보고자 한다. 감각신경성 난청으로 진단받은 40명의 환자를 대상으로 3.0T MR scanner를 이용하여 3D FSE 영상과 3D FIESTA 영상인 축상면 T2 영상을 획득하였다. 획득한 3D FSE 영상과 3D FIESTA 영상은 신경의 주행방향에 수직으로 재구성을 시행하여 내이도의 오른쪽, 왼쪽의 시상면 영상을 획득 하였다. 분석 결과 3D FSE 기법을 이용한 그룹과 3D FIESTA기법을 이용한 두 그룹에서의 신호대 잡음비는 3D FIESTA 기법을 이용한 그룹에서 높은 신호대 잡음비를 보였으며 두 그룹에서의 대조도대 잡음비는 3D FIESTA 기법을 이용한 그룹에서 높은 대조도대 잡음비를 보였다.

• Journal of Audiology & Otology
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• 제25권4호
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• pp.171-177
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• 2021

Background and Objectives: The purpose of the present study was to validate the performance and diagnostic efficacy of the Korean digits-in-noise (K-DIN) test in comparison to the Korean speech perception-in-noise (K-SPIN) test, which is the representative speech-in-noise test in clinical practice. Subjects and Methods: Twenty-seven subjects (15 normal-hearing and 12 hearing-impaired listeners) participated. The recorded Korean 0-9 digits were used to form quasirandom digit triplets; 50 target digit triplets were presented at the most comfortable level of each subject while presenting speech-shaped background noise at various levels of signal-to-noise ratios (-12.5, -10, -5, or +5 dB). Subjects were then instructed to listen to both target and noise masker unilaterally and bilaterally through a headphone. K-SPIN test was also conducted using the same procedure as the K-DIN. After calculating their percent correct responses, K-DIN and K-SPIN results were compared using a Pearson-correlation test. Results: Results showed a statistically significant correlation between K-DIN and K-SPIN in all hearing conditions (left: r=0.814, p<0.001; right: r=0.788, p<0.001; bilateral: r=0.727, p<0.001). Moreover, the K-DIN test achieved better testing efficacy, shorter average listening time (5 min vs. 30 min), and easier performance of task according to participants' qualitative reports than the K-SPIN test. Conclusions: In this study, the Korean version of digit triplet test was validated in both normal-hearing and hearing-impaired listeners. The findings suggest that the K-DIN test can be used as a simple and time-efficient hearing-in-noise test in audiology clinics in Korea.

• 대한청각학회지
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• 제25권4호
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• pp.171-177
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• 2021

Background and Objectives: The purpose of the present study was to validate the performance and diagnostic efficacy of the Korean digits-in-noise (K-DIN) test in comparison to the Korean speech perception-in-noise (K-SPIN) test, which is the representative speech-in-noise test in clinical practice. Subjects and Methods: Twenty-seven subjects (15 normal-hearing and 12 hearing-impaired listeners) participated. The recorded Korean 0-9 digits were used to form quasirandom digit triplets; 50 target digit triplets were presented at the most comfortable level of each subject while presenting speech-shaped background noise at various levels of signal-to-noise ratios (-12.5, -10, -5, or +5 dB). Subjects were then instructed to listen to both target and noise masker unilaterally and bilaterally through a headphone. K-SPIN test was also conducted using the same procedure as the K-DIN. After calculating their percent correct responses, K-DIN and K-SPIN results were compared using a Pearson-correlation test. Results: Results showed a statistically significant correlation between K-DIN and K-SPIN in all hearing conditions (left: r=0.814, p<0.001; right: r=0.788, p<0.001; bilateral: r=0.727, p<0.001). Moreover, the K-DIN test achieved better testing efficacy, shorter average listening time (5 min vs. 30 min), and easier performance of task according to participants' qualitative reports than the K-SPIN test. Conclusions: In this study, the Korean version of digit triplet test was validated in both normal-hearing and hearing-impaired listeners. The findings suggest that the K-DIN test can be used as a simple and time-efficient hearing-in-noise test in audiology clinics in Korea.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 1981년도 제15차 학술대회연제순서 및 초록
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• pp.39.1-39
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• 1981

이명은 청기질환의 중요한 증후로 청기질환의 조기 혹은 단독증상으로 존재할 때도 있으나 대부분의 경우에 있어서 난청을 수반하게 된다. 이명의 성립기전은 불분명하나 청기내 혹은 그 중추경로에의 이상자극에 의해 발생한다고 한다. 1978년 1월에서 1980년 12월까지 이명을 주소로 이대부속병원 이비인후과에 내원한 환자중 순음청력검사를 시행한 111명을 임상적 분석 및 고찰을 하였다. 1) 성별분포는 남자가 52.2%, 여자가 47.8%로 남자가 약간 많았고 연령은 21세에서 30세까지가 가장 많아 28.8%였다. 2) 발병기간은 1달에서 1년까지가 가장 많아 31.5%였다. 3) 이명만을 호소하였던 환자는 11명 (10.0%)였으며 대부분인 72%에서는 수반증상으로 난청을 호소하였다 4) 38.7%의 환자에서는 양측, 32.4%는 좌측, 28.9%는 우측의 이명을 호소하였다. 5) 고막소견은 48.0%에서는 정상고막소견을 보였으나 33.1%에서는 내함, 8.4%에서는 고막천공이 있었다. 6) 청력소실정도는 29.2%에서 정상이었고 22.7%에서는 중등고도의 청력소실이 있었다. 7) 청력상을 보면 33.1%에서 고음장애형이었고 수평형은 33.0%, 저음장애형은 11.9%였으며 $C_{5}$ dip은 11.0%에서 있었다. 8) 난청의 종류는 감각신경성난청이 가장 많아 46.9%, 혼합성난청이 33.1% 전도성난청이 20.1%였다.