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A Novel Heterozygous Missense Variant (c.667G>T;p.Gly223Cys) in USH1C That Interferes With Cadherin-Related 23 and Harmonin Interaction Causes Autosomal Dominant Nonsyndromic Hearing Loss

Song JS, Bahloul A, Petit C, Kim SJ, Moon IJ, Lee J, Ki CS

BACKGROUND: Pathogenic variants of USH1C, encoding a PDZ-domain-containing protein called harmonin, have been known to cause autosomal recessive syndromic or nonsyndromic hearing loss (NSHL). We identified a causative gene in...
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Asymptomatic Hearing Impairment Frequently Occurs in Early-Onset Parkinson's Disease

Shetty K, Krishnan S, Thulaseedharan JV, Mohan M, Kishore A

OBJECTIVE: Recent reports of hearing impairment in Parkinson's disease (PD) have suggested that auditory dysfunction could be a non-motor manifestation of PD. These reports were based on observations of elderly...
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Association between cadmium exposure and hearing impairment: a population-based study in Korean adults

Jung DJ

BACKGROUND: The present study aimed to evaluate the clinical association between cadmium exposure and hearing impairment among the Korean population. METHODS: This retrospective cross-sectional study used the data obtained from the...
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ROS Scavenger, Ebselen, Has No Preventive Effect in New Hearing Loss Model Using a Cholesterol-Chelating Agent

Lee MY, Kabara LL, Swiderski , Raphael Y, Duncan RK, Kim YH

BACKGROUND AND OBJECTIVES: The antioxidant ebselen will be able to limit or prevent the ototoxicity arising from 2-hydroxypropyl-β-cyclodextrin (HPβCD). Niemann-Pick Type C (NPC) disease is a disorder of lysosomal storage...
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Hazardous Alcohol Consumption and the Risk of Hearing Impairment in Adults Based on the Korean National Health and Nutrition Survey: A Retrospective Study

Park JA, Suh MJ

BACKGROUND AND OBJECTIVES: To investigate the relationship between hearing impairment and alcohol drinking patterns in South Korean adults. SUBJECTS AND METHODS: Data collection was performed by Korean National Health and Nutrition...
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Musical Hallucination Caused by Ceftazidime in a Woman with a Hearing Impairment

Song CI, Jung YE

Musical hallucinations remain a poorly understood clinical phenomenon, possibly because these types of hallucination have multiple causes and are rarely the focus of published reports. Here, the case of a...
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A Case of Middle Ear Neuroendocrine Adenoma in a Patient with Hearing Loss and Facial Palsy

Nam W, Kim TH, Kim MB

Middle ear adenoma is a very rare disease which is benign and originates from the middle ear mucosa. Patients of middle ear adenoma usually come to the clinic for unilateral...
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Comparison of Aided and Unaided Thresholds and Selection Processes of Contralateral Routing of Signal Hearing Aids and Implantable Bone Conduction Devices in Patients with Asymmetric Hearing Loss

Kim YH, Lee HJ, Bae MR, Ku JY, Shin CH, Park HJ

BACKGROUND AND OBJECTIVES: We reviewed the selection processes of contralateral routing of signal (CROS) hearing aids (HAs) and bone-conduction (BC) Has, and compared aided and unaided hearing thresholds. SUBJECTS AND METHOD:...
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Losartan Prevents Maladaptive Auditory-Somatosensory Plasticity After Hearing Loss via Transforming Growth Factor-β Signaling Suppression

Mun SK, Han KH, Baek JT, Ahn SW, Cho HS, Chang MY

OBJECTIVES: Hearing loss disrupts the balance of auditory-somatosensory inputs in the cochlear nucleus (CN) of the brainstem, which has been suggested to be a mechanism of tinnitus. This disruption results...
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Safety and efficacy of transcutaneous bone conduction implant surgery for hearing improvement in microtia patients with bilateral hearing impairment

Cheon JH, Lee HC, Im GJ, Park JY, Park C

BACKGROUND: In microtia patients with bilateral hearing impairment, hearing improvement is crucial for language development and performance. External auditory canal reconstruction (EACR) has been performed to improve hearing, but often...
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Genetics of Hearing Loss in North Iran Population: An Update of Spectrum and Frequency of GJB2 Mutations

Koohiyan M, Azadegan-Dehkordi F, Koohian F, Hashemzadeh-Chaleshtori M

Diagnosis of pre-lingual hearing loss (HL) is difficult owing to the high number of genes responsible. The most frequent cause of HL is DFNB1 due to mutations in the GJB2...
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Mutations in GJB2 as Major Causes of Autosomal Recessive Non-Syndromic Hearing Loss: First Report of c.299-300delAT Mutation in Kurdish Population of Iran

Azadegan-Dehkordi F, Bahrami T, Shirzad M, Karbasi G, Yazdanpanahi N, Farrokhi E, Koohiyan M, Tabatabaiefar MA, Hashemzadeh-Chaleshtori M

BACKGROUND AND OBJECTIVES: Autosomal recessive non-syndromic hearing loss (ARNSHL) with genetic origin is common (1/2000 births). ARNSHL can be associated with mutations in gap junction protein beta 2 (GJB2). To...
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Prognosis of Sudden Low Frequency Hearing Loss during Long-term Follow-up

Kim JH, Kwak SH, Bae SH, Kim SH, Nam GS

OBJECTIVES: The prognosis of sudden low frequency hearing loss (SLFHL) is relatively good, but recurrences of hearing loss and possible progression to Meniere's disease is still a clinically important concern....
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The Acceptable Noise Level Benefit From Directionality for Listeners With Severe Hearing Loss

Aghsoleimani M, Jalilvand , Mahdavi ME, Nazeri AR, Kamali M

OBJECTIVES: Directional microphone technology can enhance the speech intelligibility and listening comfort of listeners with hearing impairment. The main aim of this study is to investigate and compare the benefit...
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Clinical Safety and Efficacy of Autologous Bone Marrow-Derived Mesenchymal Stem Cell Transplantation in Sensorineural Hearing Loss Patients

Lee HS, Kim WJ, Gong JS, Park KH

Stem cell transplantation represents a promising therapy for several degenerating and necrotic diseases. In several animal studies, we could find hearing restoration after inoculation of the mesenchymal stem cells' as...
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The Protective Effect of Egb 761 Against 3-Nitropropionic Acid-Induced Hearing Loss: The Role of Sirtuin 1

Chang MY, Rhee J, Kim SH, Kim YH

OBJECTIVES: Local administration of 3-nitropropionic acid (3-NP) to the inner ear induces sensorineural hearing loss. Several studies have shown the otoprotective effects of ginkgo biloba extract EGb 761. Moreover, EGb...
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Genetic Hearing Loss and Gene Therapy

Carpena NT, Lee MY

Genetic hearing loss crosses almost all the categories of hearing loss which includes the following: conductive, sensory, and neural; syndromic and nonsyndromic; congenital, progressive, and adult onset; high-frequency, low-frequency, or...
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Comparison of the Marginal Utility and Disease Burden of Hearing Loss and Other Chronic Diseases

Kwon OD, Jung SY, Park HY, Peak SK, Cho SH, Cho SJ

BACKGROUND: Compared with other chronic diseases, hearing loss is generally overlooked from the perspective of disease burden. However, hearing loss is emerging as an important issue in the current society....
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Decreased Expression of TRPV4 Channels in HEI-OC1 Cells Induced by High Glucose Is Associated with Hearing Impairment

Xing Y, Ming J, Liu T, Zhang N, Zha D, Lin Y

PURPOSE: Previous reports have shown that hyperglycemia-induced inhibition of transient receptor potential vanilloid sub type 4 (TRPV4), a transient receptor potential ion channel, affects the severity of hearing impairment (HI)....
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Outcomes of Direct-to-Consumer Hearing Devices for People with Hearing Loss: A Review

Tran NR, Manchaiah

As the population of those with mild to moderate hearing loss increases the need remains for amplification solutions. There is a trend to offer alternative amplification options beyond traditional hearing...
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