• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.706-710
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• 2005

Purpose : Early diagnosis of congenital hearing loss through the neonatal hearing screening test minimizes language defect. This research intends to identify frequency of congenital hearing loss in infants through neonatal hearing screening test with the aim of communicating the importance of hearing test for infants. Methods : From May 20, 2003 to May 19, 2004, infants were subjected to Automated Auditory Brainstem Response test during one month of birth to conduct the test with 35 dB sound. Infants who passed the 1st round of hearing test, were classified into 'pass' group whereas those who did not were classified into 'refer' group. Infants who did not 'pass' in the hearing test conducted within one month of birth were subjected to re-test one month later, and if classified as 'refer' during the re-test, they were subjected to the diagnosis for validation of hearing loss by requesting test to the hearing loss clinic. Results : There was no difference among the 'pass' and 'refer' group in terms of form of childbirth, weight at birth and gestational age. In the 1st test, total of 45 infants were classified into 'refer' group. Six among 35 who were subjected to re-test(17%) did not pass the re-test, and all were diagnosed with congenital hearing loss. This corresponds to 0.35%(3.5 per 1,000) among total number of 1,718 subjects. Conclusion : In our study the congenital hearing loss tends to be considerably more frequently than congenital metabolic disorder. Accordingly, newly born infants are strongly recommended to undergo neonatal hearing screening test.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.210-214
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• 2010

Purpose : Cisplatin is highly effective for the treatment of solid tumors in children. However, the clinical use of cisplatin is limited by its ototoxicity. The aim of this study was to evaluate the ototoxicity in children treated with cisplatin. Method : We performed a single institution retrospective analysis of pediatric oncology patients who received cisplatin therapy between January 2001 and January 2008. Thirty-seven patients with sufficient medical and audiologic data were included in this study. Results : The median age at the time of diagnosis was 10.7 (range 3.8-6.7) years. There were 16 males and 21 females. The underlying diseases were osteosarcoma (15 cases), medulloblastoma (14 cases), germ cell tumors (7 cases), and hepatoblastoma (1 case). The median individual dose was $100mg/m^2$/cycle (56-200). The median cumulative dose was $480mg/m^2$ (200-1,490). Sixteen patients (43%) received cranial radiotherapy. Of the 37 patients, 17 developed hearing loss, leading to an overall incidence of 46%. Logistic regression showed that age at treatment (P =0.04) and cumulative dose of cisplatin (P =0.005) were the significant risk factors in predicting hearing loss in children treated with cisplatin. In all the patients who had hearing loss, there was neither improvement nor aggravation during the follow-up (3-8 months). Conclusion : The cumulative dose of cisplatin (>$500mg/m^2$) and younger age at treatment (<12 years) were 2 most important risk factors for ototoxicity in patients treated with cisplatin. Serial audiometric evaluations are needed in the patients with risk factors during and after cisplatin treatment.

• 한국노년학
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• v.30 no.3
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• pp.973-992
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• 2010

The hearing impairment is the representative disorder that affects the quality of the routine life of the aged period. This study was aimed to develop the Korean evaluation scale for hearing handicap(KESHH) with which we can evaluate social and psychological effects of the hearing impairment. Applying this scale clinically, we can analyze the geriatric hearing loss specifically and improve the quality of the aural rehabilitation that can help the hardness of the hearing impairment. Data were collected from 288 participants(176 hearing aid users and 112 non-hearing aid users) and the average age of the participants was 67.4 years old ( 60.15 for the hearing aids users and 78.9 for the non hearing users). The composition ratio of the male and female participants were 58.0% and 42.0% and extrovert and introvert personality were 49.3% and 50.7% showing balanced formation. The tentative draft of KESHH measurements were produced with 30 items and following 5 subscales. Using factor analysis, 6 items were erased and 4 subscales - social effect, psycho/emotional effect, interpersonal effect, and perception of hearing aids - were identified. As each subscale consisted of 6 items, 24 items were corrected and remained totally. Conclusively, the KESHH was developed with 24 items and 4 subscales including 6 items on each subscale. In addition, the KESHH was divided into type-1 and 2 depending on hearing aid users and non hearing aid users. The results of this study can be summarized as the following 5 parts. Firstly, the reliabilities of the KESHH were proved to be high because the subscales' Cronbach alpha values were from 0.723 through 0.895. Secondly, the KESHH showed systematically increasing score as the hearing impairment increased. The lowest score was 24 and the highest score was 117 and the average scores of the hearing impaired and non-hearing impaired are 72.06(SD=15.67) and 66.98(SD=20.94) showing 5.08 increased score for the hearing impaired. Depending on the degree of the hearing loss, the scores recorded 52.63 at the below of the mild hearing loss, 67.29 for the moderate hearing loss, 71.89 for the moderately severe hearing loss, and 75.57 for the severe hearing loss The comparison of the scores by hearing levels indicated that the higher the hearing levels were, the higher the scores of the KESHH with statistical significance(p<0.001). Thirdly, the correlation among 4 subscales was 0.384~0.880(p<0.001). Also, the pure tone average, personality, and the four subscales correlations showed statistical significance with 0.148~0.880 except for the pure tone average and personality and the pure tone average and perception of hearing aids. Fourthly, the total variances explained for the independent subscles were analyzed with multiple regression. The social effect was explained 17.4% with pure tone average, personality, and the status of hearing aid use variances. The psycho/emotional effect was explained 14.4% with puretone average, personality, and age variances. The interpersonal effect was explained 11.2% with pure tone average, personality, and the status of hearing aid use variances. The perception of hearing aids effect was explained 2.2% with only personality. Finally, test-retest reliability was proved to be high with 0.791(p<0.001). Conclusively, the KESHH that was developed considering Korean culture can be a useful instrument for expressing the hearing handicaps of the Korean aged hearing impaired in scores for both hearing aid users and non-users. Also, it is thought that the KESHH is useful clinically for identifying the changes of the hearing handicap scores before and after wearing hearing aids and aural rehabilitation at diverse situations.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1987.05a
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• pp.9.1-9
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• 1987

뇌간유발청력검사는 최근 들어 신생아 및 영유아에 있어 청력 및 청신경로의 성숙(auditory maturation)정도를 파악할 수 있는 객관적인 진단 방법으로 평가되고 있다. 그러나 상기 검사결과는 측정계기의 종류 및 측정방법에 따라 다소 차이를 보일 수 있다. 우리나라에서는 아직 정상 신생아를 대상으로 뇌간유발청력검사에 대한 보고가 없는 실정이다. 이에 본 교실에서는 87년 3~4월에 Severance병원에서 출생한 정상 신생아 20명을 대상으로, 출생 후 16내지 69 시간내 두 차례에 걸쳐 뇌간유발 전달시간 및 뇌간유발 역치를 측정, 다음과 같은 결과를 얻었기에 보고하는 바이다. 1) 72.5 ㏈, 4KHz click에 대한 평균 절대적 latency는 각각 I(1.68), II(2.74), III(4.33), IV(5.71), V(6.77), Ⅵ(8.16), Ⅶ(9.85)였고 tone pip은 각각 I(2.04), II(3.04), III(4.60), IV(5.75), V(7.14), Ⅵ(8.54), Ⅶ(10.60) 이었다. 2) 평균 뇌간전위 유발역치는 29.5㏈ SPL 이었다. 3) 파형은 A형이 1.7%, 3형이 30.2%, C형이 45.7%, D형이 5.2%, E형이 1.7%, F형이 15.5%이었고, click과 tone pip, 자극음의 강도, ISI에 따른 차이가 없었다. 4) 제1소파에서 제5소파까지의 절대적 latency와 I- III, III-V,및 I-V의 상대적 latency에 있어서, 반복율, 자극음 종류 및 첫째날과 둘째날의 비교치에 있어서만이 선택적으로 유의한 차이가 발생하였으며, 자극음의 크기에는 차이가 나타나지 않았다.

• Journal of rehabilitation welfare engineering & assistive technology
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• v.3 no.1
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• pp.27-34
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• 2009

Hearing impaired person's hearing loss has personally various shape, so existing symmetrical auditory filter of frequency band method wasn't properly simulated the hearing impaired person's various hearing loss shape. The shapes of auditory filter are asymmetrical different with each center frequency and each input level. Hearing impaired person which has hearing loss was differently changed with that of normal hearing people and it has different value for speech of quality through auditory filter. In this study, the asymmetrical auditory filter was simulated and then some tests to estimate the filter's performance objectively were performed. The experiment as simulated auditory filter's performance evaluation method used perceptual evaluation of speech quality (PESQ) and log likelihood ratio (LLR) for speech through auditory filter. In the test, processed speech was evaluated objective speech quality and distortion using PESQ and LLR value. When hearing loss processed, PESQ and LLR value have big difference between symmetrical and asymmetrical auditory filter. It means that the difference of the shape auditory filter may affect to speech quality. Especially, when hearing loss existed, auditory filter changing according to asymmetrical shape for each center frequency affected to perceive speech quality of the hearing impaired.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.158-162
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• 2010

Purpose : Turner syndrome (TS) is a disorder in which various anomalies can be accompanied, especially cardiovascular, renal, thyroid and auditory problems. The aim of this study is to identify the incidence of these disorders in patients with TS according to karyotype. Methods : We reviewed medical records of 90 patients with TS diagnosed by chromosomal analysis in 4 hospitals from Jan 1998 to Dec 2007. We evaluated these cases by prepared protocol of 4 medical problems.Results : The distribution of karyotype was 45,X (47.8%), mosaic pattern (34.4%) and structural aberration group (17.8%). Renal anomalies, cardiovascular anomalies, thyroid disorders and auditory problems are accompanied in 4.4%, 10.0 %, 11.1% and 5.6%, respectively. 45,X group had renal anomalies (7.0%), cardiovascular anomalies (18.6%), thyroid disorders (9.3%) and auditory problems (11.6%). Mosaic group had renal anomalies (3.2%), thyroid disorders (12.9%), no cardiovascular anomalies and auditory problems. Structural aberration group had cardiovascular anomalies (6.3%), thyroid disorders (12.5%) and no other 2 problems. Patients with 45,X group had a significant higher incidence of cardiovascular anomalies (P =0.025). Conclusion : Our results indicate that there are differences clinically according to karyotype of TS, especially in incidence of cardiovascular anomalies.

• Journal of Digital Convergence
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• v.14 no.4
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• pp.363-370
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• 2016

Noise is unwanted sound that is the reason of the stress and hearing loss. The current study attempted to estimate whether the noise of dental laboratory affected stress and pure tone audiometry (PTA) of dental laboratory technicians (DLTs) using heart rate variability, air and bone conduction audiometry. The age, heights, and weights of DLTs were resembled control. Standard deviation of normal to normal interval such as stress resistance and normalized HF of DLTs were significantly decreased, but heart rates, normalized LF, and LF/HF ratio of DLTs were significantly increased compared with control. In air conduction audiometry of DLTs, significant increments of thresholds encountered in 125, 250, 500, 1000, 2000, 3000, 4000, and 6000 Hz in the right ears and 125, 250, 500, 1000, and 2000 Hz in the left ears. Thresholds of bone conduction audiometry in both ears were significantly increased in 250, 500, 1000, 2000, and 4000 Hz. The findings in this study provide that stress and hearing loss observed in noise-exposed DLTs at dental laboratory. Therefore, proper safety precautions should be carried out at dental laboratory.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1981.05a
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• pp.39.1-39
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• 1981

The author presents clinical study for 111 cases of tinnitus with pure tone audiometry from Jan. 1, 1978 to Dec. 31, 1980 in department of otolaryngology, Ewha Womans University Hospital. The results were as follows; 1) Male to Female ratio was as 1 : 1, and peak age incidence was in the age group of 21 to 30. 2) Most frequent duration was 1 month to 1 year in the cases of the tinnitus. 3) The patient who complained tinnitus only was 10.0% and the patient who complained tinnitus with hearing impairment was 72.0% in the cases of tinnitus. 4) The affected site in the tinnitus, in order of frequency were; Both ear 38.7% Left ear 32.4% Right ear 28.9% 5) The drum finding, in order frequency were; Normal 48.0% Retraction 33.1% Perforation 8.4% 6) The degree of hearing loss in the audiometry were; Normal 29.2% Moderate severe 22.7% 7) The shape of hearing impairments were; High tone loss 46.9% Flat 33.0% Low tone loss 11.9% 8) The occurrence of $C_{5}$ dip was 11.0% in cases of the tinnitus. 9) The classification of hearing loss were; Sensorineural hearing loss 46.9% Mixed hearing loss 33.1 % Conductive hearing loss 20.1%

• Neonatal Medicine
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• v.18 no.2
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• pp.328-336
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• 2011

Purpose: An association between very low birth weight infants(VLBWI) and hearing loss has long been recognized. Early identification and intervention for hearing loss benefits language and speech/cognitive development. We investigated the risk factors and clinical outcomes of hearing loss among VLBWI. Methods: We analyzed the results of auditory brainstem response (ABR) testing of VLBWI. These infants were admitted to the neonatal intensive care unit (NICU) of Pusan National University Yangsan Hospital between December 2008 and February 2011. A follow-up was conducted subsequently. Results: ABR evaluations were performed on 65 infants, and 31 showed abnormal results (47.7%). Among the 31 infants, 10 were classified with moderate/severe/profound hearing loss (15.4%). The infants with abnormal ABR had a higher incidence of low birth weight, prolonged ventilator care, cumulative dose of furosemide, and the lowest $PaO_2$ (P<0.05). Those with moderate/severe/profound hearing loss had a higher incidence of low Apgar scores at 5 minutes (odds ratio[OR],0.34; 95% confidence interval[CI],0.13-0.89), prolonged ventilator care (OR,1.06; 95% CI,1.01-1.12), and mild hearing loss compared to those without profound hearing loss. Follow-up evaluations on eight infants with ABR reveled improvements 5.6${\pm}$3.9 months later. One infant, who had profound hearing loss in both ears, used a hearing aid. Conclusion: Factors influencing hearing loss at the first VLBWI hearing screening test included lower Apgar scores at 5 min and prolonged use of a ventilator. Most VLBWI with hearing losses were expected to recover after several months of follow-up.

• Journal of Life Science
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• v.31 no.5
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• pp.453-463
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• 2021

Hearing loss is a group of clinically and genetically heterogeneous disorders characterized by congenital- to adult-onset deafness with frequent additional symptoms such as myopathy, nephropathy, and optic disorders. It is commonly divided into two types: syndromic, with no other symptoms, and nonsyndromic, with other symptoms. Autosomal recessive hearing loss is relatively frequent in Pakistan, which may be due in part to frequent consanguineous marriages. This study was performed by whole exome sequencing to determine the genetic causes in two Pakistani consanguineous families with autosomal recessive hearing loss. We identified a pathogenic homozygous variant (p.Leu326Gln in MYO7A) in a family with prelingual-onset hearing loss and two variants of uncertain significance (p.Val3094Ile in GPR98 and p.Asp56Gly in PLA2G6) in a family with early-onset hearing loss concurrent with muscular atrophy. The missense mutations in MYO7A and PLA2G6 were located in the highly conserved sites, and in silico analyses predicted pathogenicity, while the GPR98 mutation was located in the less conserved site, and most in silico analysis programs predicted its nonpathogenic effect. Homozygosity mapping showed that both alleles of the homozygous mutations identified in each family originated from a single founder; spread from this single source might be due to consanguineous marriages. This study will help provide exact molecular diagnosis and treatment for autosomal recessive hearing loss patients in Pakistan.