• Journal of Genetic Medicine
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• v.6 no.1
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• pp.8-24
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• 2009

Understanding the genetic background of hearing loss is important since almost 50% of the cases of profound hearing loss are caused by genetic factors. Until now, more than 150 causative genes have been identified. In this review, classification of genetic hearing loss (syndromic versus non-syndromic, recessive versus dominant, X-linked and mitochondrial), pitfalls in elucidating causative genes, anatomy of the inner ear, introduction of the most common syndromic hearing loss, introduction of the most common non-syndromic hearing loss-causing genes, mitochondrial and multifactorial hearing losses were discussed. Moreover, clinical approaches to the patients with hereditary hearing loss and genetic counseling were also explained briefly. Finally, future directions of the hereditary hearing loss research in Korean population were presented.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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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.

• Progress in Medical Physics
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• v.9 no.4
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• pp.217-225
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• 1998

In this paper, the analysis of the relation between noise induced hearing loss and noise exposure is described for the A-weighted noise levels and exposure duration. The hearing loss and sensitivity threshold shift is investigated by changing the various parameters such as the effects of aging and noise exposure. Total hearing loss is proportional to a function of exposure level based upon the integral of pressure with time. If the noise exposure term is large so that the presbycusis may be neglected, the hearing loss due to aging and noise exposure becomes the noise induced hearing loss. It is shown that exposure to excessive noise can cause temporary loss of hearing that may become permanent if the exposure is prolonged or intense. An audiogram taken from a person suffering from noise induced hearing loss will usually show the greatest loss of hearing sensitivity in the 4kHz region, which is typically the region most sensitive to damage resulting from many types of industrial noise.

• Journal of Korean Neurosurgical Society
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• v.50 no.1
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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.

• Korean Journal of Audiology
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• v.24 no.4
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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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• v.61 no.12
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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.

• Journal of rehabilitation welfare engineering & assistive technology
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• v.11 no.3
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• pp.215-221
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• 2017

Audiological rehabilitation by itself can provide satisfactory services through acquisition of background information on hearing loss, audiological evaluation, analysis of electroacoustic characteristics of hearing aids, and determining acoustic gain. However, hearing can be exacerbated, and sound pressure level of hearing aids can be lowered. Moreover, breakdown of hearing aids can happen. Therefore, the early detection of hearing loss and regular follow-up observations are very important. The aim of this study concern the direction of effective audiological rehabilitation by checking the clinical cases where hearing aids fitting failures were found.

• Safety and Health at Work
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• v.11 no.2
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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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• v.24 no.3
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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.

• Korean Journal of Audiology
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• v.24 no.3
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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.