Objectives: . FDXR encodes mitochondrial ferredoxin reductase, which is associated with auditory neuropathy spectrum disorder (ANSD) and optic atrophy. To date, only two studies have described FDXR-related hearing loss. The auditory rehabilitation outcomes of this disease entity have not been investigated, and the pathophysiological mechanisms remain incompletely understood. Here we report a hearing-impaired individual with co-segregation of the FDXR variant and post-synaptic type ANSD, who underwent cochlear implantation (CI) with favorable outcomes. We suggest a possible pathophysiological mechanism of adult-onset ANSD involving mitochondrial dysfunction. Methods: . A 35-year-old woman was ascertained to have ANSD. Exome sequencing identified the genetic cause of hearing loss, and a functional study measuring mitochondrial activity was performed to provide molecular evidence of pathophysiology. Expression of FDXR in the mouse cochlea was evaluated by immunohistochemistry. Intraoperatively, electrically evoked compound action potential (ECAP) responses were measured, and the mapping parameters were adjusted accordingly. Audiological outcomes were monitored for over 1 year. Results: . In lymphoblastoid cell lines (LCLs) carrying a novel FDXR variant, decreased ATP levels, reduced mitochondrial membrane potential, and increased reactive oxygen species levels were observed compared to control LCLs. These dysfunctions were restored by administering mitochondria isolated from umbilical cord mesenchymal stem cells, confirming the pathogenic potential of this variant via mitochondrial dysfunction. Partial ECAP responses during CI and FDXR expression in the mouse cochlea indicate that FDXR-related ANSD is post-synaptic. As a result of increasing the pulse width during mapping, the patient’s CI outcomes showed significant improvement over 1-year post-CI. Conclusion . A novel FDXR variant associated with mitochondrial dysfunction and post-synaptic ANSD was first identified in a Korean individual. Additionally, 1-year post-CI outcomes were reported for the first time in the literature. Excellent audiologic results were obtained, and our results reiterate the correlation between genotype and CI outcomes in ANSD.

Objectives: . The recent expansion of eligibility for cochlear implantation (CI) by the U.S. Food and Drug Administration (FDA) to include infants as young as 9 months has reignited debates concerning the clinically appropriate cut-off age for pediatric CI. Our study compared the early postoperative trajectories of receptive and expressive language development in children who received CI before 9 months of age with those who received it between 9 and 12 months. This study involved a unique pediatric cohort with documented etiology, where the timing of CI was based on objective criteria and efforts were made to minimize the influence of parental socioeconomic status. Methods: . A retrospective review of 98 pediatric implantees recruited at a tertiary referral center was conducted. The timing of CI was based on auditory and language criteria focused on the extent of delay corresponding to the bottom 1st percentile of language development among age-matched controls, with patients categorized into very early (CI at <9 months), early (CI at 9–12 months) and delayed (CI at 12–18 months) CI groups. Postoperative receptive/expressive language development was assessed using the Sequenced Language Scale for Infants receptive and expressive standardized scores and percentiles. Results: . Only the very early CI group showed significant improvements in receptive language starting at 3 months post-CI, aligning with normal-hearing peers by 9 months and maintaining this level until age 2 years. During this period (<2 years), all improvements were more pronounced in receptive language than in expressive language. Conclusion . CI before 9 months of age significantly improved receptive language development compared to later CI, with improvements sustained at least up to the age of 2. This study supports the consideration of earlier CI, beyond pediatric Food and Drug Administration labeling criteria (>9 months), in children with profound deafness who have a clear deafness etiology and language development delays (<1st percentile).

Background: To determine the effects of Jerusalem Artichoke extract (JAE) and inulin on blood glucose levels and insulin secretion in streptozotocin (STZ)-induced diabetic mice.
Methods: Thirty four mice were divided into a normal control group and three experimental groups: diabetic control, JAE, and inulin. STZ (50 mg/kg) was injected intraperitoneally to induce diabetes in the three experimental groups. The JAE and inulin groups were fed 10 g/kg JAE or fed 1 g/kg inulin, respectively, for 6 weeks. Fasting glucose was checked weekly. After 6 weeks, the oral glucose tolerance test (OGTT) was performed, and the insulin level was checked.
Results: Four mice from the JAE group (n = 9) died and autopsies revealed inflammation and ulceration of skin lesions on the chest areas. Fasting glucose levels were not decreased in the inulin or JAE group relative to diabetic control group. In the OGTT at 60 minutes and 120 minutes, the serum glucose levels were significantly higher in the inulin group (572.6 ± 52.0 mg/dL and 555.8 ± 72.9 mg/dL, respectively) than in diabetic control group (484.3 ± 81.6 mg/dL and 467.3 ± 111.1 mg/dL, respectively). Insulin levels were not increased in the inulin group relative to the diabetic control group.
Conclusion These results indicate that JAE and inulin might not be useful therapeutic strategies for diabetes mellitus and indiscreet intake of Jerusalem Artichoke could exacerbate to diabetes.

BACKGROUND AND OBJECTIVES: The Musical Background Questionnaire (MBQ) has been developed to assess formal musical training and listening enjoyment. The aims of this study were to translate MBQ into Korean with subsequent linguistic validation and to evaluate the effectiveness of the Korean version of MBQ (K-MBQ).SUBJECTS AND METHOD: Between 2013 and 2014, a panel affiliated with the questionnaire committee of the Korean Audiological Society reconciled the first draft K-MBQ translated by a bilingual person. A separate bilingual translator, who had never seen the original MBQ, translated the draft K-MBQ back into English, and subsequently, the panel reviewed its equivalence to the original one. K-MBQ was administered to 29 adults (M:F=15:14; aged 21 to 76 years) for cognitive debriefing. Pure tone and speech audiometry were performed in all participants. RESULTS: The translation of K-MBQ was completed through a multi-step process of forward translation, reconciliation, reverse translation, cognitive debriefing and proofreading. Thirteen (45%) of 29 subjects reported formal musical training, and 16 participants (55%) judged themselves as having no musical education and background. No significant correlation was found between musical background and hearing level, whereas self-perceived quality of music and self-perception of music elements quantified by K-MBQ were associated with hearing ability in terms of pure-tone and speech audiometry. CONCLUSION K-MBQ was translated and linguistically validated. The use of this questionnaire can provide further evaluation of musical background in patients with hearing loss or cochlear implant users.

BACKGROUND/AIMS: This study aimed to elucidate the prevalence of hepatitis B virus (HBV) serologic markers in Korean patients newly diagnosed with, but not yet treated for inflammatory bowel disease (IBD). METHODS: We prospectively enrolled 210 patients newly diagnosed with IBD (109 with ulcerative colitis and 101 with Crohn's disease). Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (anti-HBs), and hepatitis B core antibody (anti-HBc) levels were measured and compared with those of 1,100 sex- and age-matched controls. RESULTS: The prevalence of chronic HBV infection (positive HBsAg, positive anti-HBc, and negative anti-HBs results) and past infection (negative HBsAg, positive anti-HBc, and positive or negative anti-HBs results) were not significantly different between the patients and controls (chronic HBV infection: IBD, 3.8% vs. control, 4.9%, P=0.596; past infection: IBD, 26.2% vs. control, 28.8%, P=0.625). The patients with IBD aged < 20 years were at a higher susceptibility risk (nonimmune) for HBV infection than the controls (IBD, 41.5% vs. control, 22.4%; P=0.018). In the multivariate analysis, an age of < 20 years (P=0.024) and symptom duration of ≥12 months before diagnosis (P=0.027) were identified as independent risk factors for nonimmunity against HBV infection. CONCLUSIONS: The patients newly diagnosed with IBD were susceptible to HBV infection. The frequency of nonimmunity was high, especially in the patients aged < 20 years and those with a longer duration of symptoms before diagnosis. Therefore, it is necessary to screen for HBV serologic markers and generate a detailed vaccination plan for patients newly diagnosed with IBD.
Colitis, Ulcerative ; Crohn Disease ; Diagnosis ; Hepatitis B Surface Antigens ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Inflammatory Bowel Diseases* ; Multivariate Analysis ; Prevalence ; Prospective Studies ; Risk Factors ; Vaccination

BACKGROUND AND OBJECTIVES: The Speech, Spatial and Qualities of Hearing Scale (SSQ) was designed to measure self-reported auditory disability in a wide variety of listening situations. Its 49 items cover many aspects of speech perception, spatial hearing, and qualities of hearing, which constitute the three parts of SSQ. However, there has been no reliable and valid Korean version of SSQ (K-SSQ), which made the measurement of auditory disability difficult. The aim of this study is to develop a K-SSQ and to determine its reliability and validity for clinical or academic use. SUBJECTS AND METHOD: An expert panel translated the original SSQ into Korean. A bilingual translator back-translated the translated version into English, which was then compared with the original version. After cognitive debriefing, K-SSQ was administered to 400 patients with hearing disability in 14 referral hospitals. Reliability was assessed using Cronbach's alpha coefficient and correlation study. Validity was evaluated by factor analysis and criterion validity based on the results of pure tone audiometry. RESULTS: K-SSQ showed good reliability with high internal consistency (Cronbach's α=0.99), and strong positive correlations across all three parts of SSQ. Construct validity was confirmed by the results of factor analysis and criterion validity demonstrated positive correlations between each part of SSQ and the results of pure tone audiometry. CONCLUSION: The K-SSQ is a reliable and valid tool for use as a behavioral measure of hearing ability in Korean-speaking patients, and it will provide a very useful evaluation tool for both clinicians and researchers.
Audiometry ; Hearing* ; Humans ; Methods ; Referral and Consultation ; Reproducibility of Results* ; Speech Perception ; Statistics as Topic

BACKGROUND AND OBJECTIVES: The quantification of hearing aid benefit is important in hearing aid fitting. The abbreviated profile of hearing aid benefit (APHAB) is a useful tool for measuring the benefit of using hearing aids. In this study, we developed a Korean version of APHAB (K-APHAB) and determined its validity and reliability. SUBJECTS AND METHOD: An expert panel translated the original version of APHAB into Korean language. Two bilingual translators back-translated the first translated version into English and it was compared with the original version. After cognitive debriefing, the translation of APHAB into the Korean version was completed. One hundred ninety-seven subjects using hearing aids participated in this study and completed K-APHAB. Reliability was assessed using Cronbach's alpha coefficient, and validity was evaluated by factor analysis and criterion validity. RESULTS: K-APHAB had a good internal consistency (α=0.80). Confirmatory factor analysis showed that K-APHAB is comprised of four subsections, namely ‘communication in daily life’, ‘aversiveness’, communication in ‘background noise’ or ‘reverberation’. CONCLUSION: The K-APHAB is a useful tool for evaluating the benefit of hearing aids in Korean hearing-impaired individuals.
Hearing Aids* ; Hearing* ; Methods ; Reproducibility of Results

BACKGROUND AND OBJECTIVES: Client Oriented Scale of Improvement (COSI) has been designed to identify client needs, changes in listening ability, and final listening ability in situations important to each client. The aim of this study was to translate COSI into Korean with subsequent linguistic validation and to determine the reliability of the Korean version of the COSI (K-COSI). SUBJECTS AND METHOD: An expert panel translated the original version of COSI into the Korean language. A bilingual translator back-translated the translated version into English, which was subsequently compared with the original English version. K-COSI was administered at 14 referral hospitals, to 128 patients with hearing disability after cognitive debriefing. Reliability was assessed using correlation study. RESULTS: K-COSI showed an excellent test-retest correlation and high reliability in degree of change (Spearman correlation=0.89, interclass correlation coefficient=0.922). It also showed a fair test-retest correlation and high reliability in final hearing ability (Spearman correlation=0.49, interclass correlation coefficient=0.353) CONCLUSION: K-COSI proved to be highly reliable. The results suggest that the adapted Korean version of COSI is a reliable and valid measure for Korean-speaking patients with hearing loss.
Hearing ; Hearing Aids ; Hearing Loss ; Humans ; Linguistics ; Methods ; Referral and Consultation ; Statistics as Topic