• Journal of Genetic Medicine
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• 제6권1호
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• pp.8-24
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• 2009

난청의 약 50%는 유전적 요소가 원인이기 때문에 난청의 유전적 배경을 이해하는 것은 중요하다. 현재까지 150개 이상의 원인유전자들이 밝혀져 있다. 이번 종설에서는 유전성 난청의 분류, 유전성 난청 원인규명의 난점, 유전성 난청과 관련된 내이의 구조와 기능, 증후군성난청, 비증후군성난청, 미토콘드리아 유전성 난청, 그리고 다인자성 난청에 관해 논하고자 한다. 그리고 유전성 난청을 가진 환자의 치료적 접근과 유전적 상담을 간략하게 설명하고, 마지막으로 유전성 난청에 대한 앞으로의 연구 방향을 제시하고자 한다.

• Clinical and Experimental Pediatrics
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• 제50권1호
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• pp.7-13
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• 2007

Hearing loss in newborns is the most frequently occurring birth defect. If hearing impaired children are not identified and managed early, it is difficult for many of them to acquire the fundamental language, social and cognitive skills that provide the foundation for later schooling and success in society. All newborns, both high and low risk, should be screened for hearing loss in the birth hospital prior discharge (Universal Newborn Heaing Screening, UNHS). Objective physiologic measures must be used to detect newborns and very young infants with hearing loss. Recent technological developments have produced screening methods and both evoked otoacoustic emission (EOAE) and auditory brainstem response (ABR) have been successfully implemented for UNHS. Audiologic evaluation should be carried out before 3 months of age and infants with confirmed hearing loss should receive intervention before 6 months of age. All infants who pass newborn hearing screening but who have risk indicators for other auditory disorders and/or speech and language delay receive ongoing audiologic surveillance and monitoring for communication development. Infants with sensorineural hearing loss are managed with hearing aids and receive auditory and speech-language rehabilitation therapies. Cochlear implants can be an outstanding option for certain children aged 12 months and older with severe to profound hearing loss who show limited benefit from conventional amplifications.

• 한국의학물리학회지:의학물리
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• 제9권4호
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• pp.217-225
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• 1998

본 연구에서는 A청감보정 소음레벨과 폭로기간에 대한 소음유발 청력손실과 소음폭로 사이의 관계를 해석하였다. 연령과 소음폭로등 다양한 변수에 대하여 청력손실과 청감민감도 변화를 조사하였다. 연구결과 전체 청력손실은 음압의 시간적분에 의한 소음폭로 레벨에 비례하였다. 만약 소음폭로가 노인성 난청보다 크면 연령과 소음에 의해 발생하는 청력손실은 주된 원인이 소음에 의한 것임을 확인하였다. 과도한 소음은 일시적 청력손실의 원인이며, 폭로가 길어지거나 강력하면 영구적 청력손실의 원인이 될 수 있다. 소음유발 청력손실을 겪는 사람의 청력도는 4kHz 영역에서 청감민감도의 급격한 손실을 보여주며, 이 영역은 여러 형태의 산업소음으로 인해 가장 손상받기 쉬운 전형적인 주파수영역임을 확인하였다.

• Journal of Korean Neurosurgical Society
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• 제50권1호
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• pp.51-53
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• 2011

We present a rare case of medulloblastoma which presented with unilateral sudden sensorineural hearing loss as an initial symptom. A 19-year-old man was admitted to our hospital with a chief complaint of dizziness and facial numbness on the right side. His illness had begun two years previously with sudden hearing loss on the right side, for which he had been treated as an idiopathic sudden hearing loss. Magnetic resonance imaging demonstrated abnormal signals located mainly in the right middle cerebellar peduncle. We performed partial resection of the tumor by suboccipital craniotomy. The histopathological diagnosis was medulloblastoma. Intrinsic brain tumor is an extremely rare cause of sudden sensorineural hearing loss and is therefore easily overlooked as was in the present case. The present case highlights not only the need to evaluate patients with sudden sensorineural hearing loss by magnetic resonance imaging but also the importance of paying attention to intrinsic lesions involving the brainstem. Although this condition like the presented case might be rare, intrinsic brain tumor should be considered as a potential cause of sudden sensorineural hearing loss, as it may be easily missed leading to a delay in appropriate treatment.

• 대한청각학회지
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• 제24권4호
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• pp.174-179
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• 2020

Background and Objectives: The integration of auditory-visual speech information improves speech perception; however, if the auditory system input is disrupted due to hearing loss, auditory and visual inputs cannot be fully integrated. Additionally, temporal coincidence of auditory and visual input is a significantly important factor in integrating the input of these two senses. Time delayed acoustic pathway caused by the signal passing through digital signal processing. Therefore, this study aimed to investigate the effects of hearing loss and hearing aid digital delay circuit on sound-induced flash illusion. Subjects and Methods: A total of 13 adults with normal hearing, 13 with mild to moderate hearing loss, and 13 with moderate to severe hearing loss were enrolled in this study. Subsequently, the sound-induced flash illusion test was conducted, and the results were analyzed. Results: The results showed that hearing aid digital delay and hearing loss had no detrimental effect on sound-induced flash illusion. Conclusions: Transmission velocity and neural transduction rate of the auditory inputs decreased in patients with hearing loss. Hence, the integrating auditory and visual sensory cannot be combined completely. Although the transmission rate of the auditory sense input was approximately normal when the hearing aid was prescribed. Thus, it can be concluded that the processing delay in the hearing aid circuit is insufficient to disrupt the integration of auditory and visual information.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• 제61권12호
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• pp.705-709
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• 2018

It is challenging to achieve sufficient hearing gain in patients with mixed hearing loss. In chronic middle ear diseases, conventional passive reconstructive surgeries often result in suboptimal hearing gain and additional hearing aids may have limitations due to insufficient sound amplification, occlusion effect, acoustic feedback, and skin irritation. Middle ear implantation (MEI) using Vibrant Soundbridge (VSB) is another option for auditory rehabilitation in mixed hearing loss as well as sensorineural hearing loss. The floating mass transducer of VSB can be placed on various middle ear structures either directly or using different types of couplers in order to deliver vibratory mechanical energy to the cochlea. We report a patient who presented with bilateral mixed hearing loss due to chronic otitis media and had limitations using conventional hearing aids in the worse hearing ear. The patient was successfully treated with MEI using the Bell coupler together with middle ear surgery in a single step.

• 재활복지공학회논문지
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• 제11권3호
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• pp.215-221
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• 2017

청각학적 재활은 청력손실 배경 정보 획득, 청각학적 평가, 보청기 전기음향특성 분석, 음향 이득 결정만으로도 충분히 만족스러운 서비스를 제공할 수 있다. 그러나 청력은 나빠질 수 있고, 보청기는 출력음압이 낮아지거나 고장이 발생할 수도 있다. 따라서 청력손실의 조기 발견과 정기적인 추적 관찰은 매우 중요하다. 이 연구는 정상적인 추적에도 불구하고 보청기 조절 실수가 확인된 증례를 통해 효과적인 청각언어재활 방향을 고민하는데 목적이 있다.

• Safety and Health at Work
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• 제11권2호
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• pp.199-206
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• 2020

Background: In a wide range of industries, noise-induced hearing loss remains one of the most prevalent occupational problems. This study aimed to assess the noise exposure level and associated factors of hearing loss among textile workers in Yangon Region, Myanmar. Methods: A cross-sectional study was conducted at a Textile mill (Thamine), Yangon Region, from April to December 2018. In total, 226 workers who were randomly selected from 3 weaving sections participated in face-to-face interviews using a structured questionnaire. A digital sound level meter and pure-tone audiometer were used for the assessment of noise exposure level and hearing loss, respectively. Logistic regression analysis was performed to assess the associated factors of hearing loss. Results: In total workers, 66.4% were exposed to ≥85 dB(A) of noise exposure, and the prevalence of hearing loss was 25.7%. Age ≥35 years, below high school education, hearing difficulty, tinnitus, hypertension, > 9 years of service duration in a textile mill were positively associated with hearing loss. After adjusting confounding factors, age ≥35 years (adjusted odds ratio = 6.90, 95% confidence interval = 3.45-13.82) and tinnitus (adjusted odds ratio = 2.88, 95% confidence interval = 1.13-7.37) were persistently associated with hearing loss. Conclusion: Providing occupational hazard education and enforcement of occupational safety regulations should be taken to decrease the noise exposure level. The regular audiometry test should be conducted for assessment of hearing threshold shift. The employer needs to implement a hearing conservation program in workplace when noise exposure reaches or exceeds 85 dB(A) for 8 hours.

• Journal of Audiology & Otology
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• 제24권3호
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• pp.127-132
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• 2020

Background and Objectives: In this study, we compared the outcomes of patients with idiopathic sudden sensorineural hearing loss who underwent steroid treatment with or without hyperbaric oxygen (HBO) therapy and were followed-up in our clinic. Subjects and Methods: Patients were divided into two groups according to their treatment regimen. Steroid group received intravenous 1 mg/kg methylprednisolone which was due to be completed in 2-3 weeks with decreasing doses, and five doses of 0.5 mL intratympanic dexamethasone. Steroid+HBO group received the same steroid treatment with the addition of HBO therapy. The audiologic results of both treatment groups were compared after considering the patients' risk factors. Results: There was no significant difference between the steroid and Steroid+HBO groups in terms of hearing gain and degree of recovery, both at all degrees of hearing loss, and in severe and profound hearing loss. Hearing gain was similar when evaluated by audiogram type and admission time in both treatment groups. Conclusions: We found that the addition of HBO therapy to systemic plus intratympanic steroid treatment did not affect hearing gain at all degrees of hearing loss in this study. Furthermore, audiogram type and admission time did not affect hearing gain between the two groups.

• 대한청각학회지
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• 제24권3호
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• pp.127-132
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• 2020

Background and Objectives: In this study, we compared the outcomes of patients with idiopathic sudden sensorineural hearing loss who underwent steroid treatment with or without hyperbaric oxygen (HBO) therapy and were followed-up in our clinic. Subjects and Methods: Patients were divided into two groups according to their treatment regimen. Steroid group received intravenous 1 mg/kg methylprednisolone which was due to be completed in 2-3 weeks with decreasing doses, and five doses of 0.5 mL intratympanic dexamethasone. Steroid+HBO group received the same steroid treatment with the addition of HBO therapy. The audiologic results of both treatment groups were compared after considering the patients' risk factors. Results: There was no significant difference between the steroid and Steroid+HBO groups in terms of hearing gain and degree of recovery, both at all degrees of hearing loss, and in severe and profound hearing loss. Hearing gain was similar when evaluated by audiogram type and admission time in both treatment groups. Conclusions: We found that the addition of HBO therapy to systemic plus intratympanic steroid treatment did not affect hearing gain at all degrees of hearing loss in this study. Furthermore, audiogram type and admission time did not affect hearing gain between the two groups.