Association between Osteoporosis and Meniere's Disease: Two Longitudinal Follow-Up Cohort Studies.

A high rate of Meniere's disease (MD) in patients with osteoporosis has been suggested. This research intended to estimate the bidirectional association of MD with osteoporosis. The ≥40-year-old population in the Korean National Health Insurance Service-Health Screening Cohort 2002-2019 was examined. In study I, 9529 patients with MD and 38,116 control I participants were analyzed for a previous history of osteoporosis. In study II, 65,858 patients with osteoporosis and 65,858 control II participants were analyzed for a previous history of MD. Stratified Cox proportional hazard models were applied to calculate the hazard ratios (HRs) and 95% confidence intervals (CIs) of MD for osteoporosis in study I and of osteoporosis for MD in study II. The rate of a prior history of osteoporosis was 13.3% for the MD group and 11.3% for the control I group. The patients with MD had a 1.12 times higher HR for previous osteoporosis (95% CI = 1.04-1.20). In study II, the rate or a prior history of MD was 3.7% for patients with osteoporosis and 2.0% for the control II group. The patients with osteoporosis had a 1.50 times higher HR for previous MD (95% CI = 1.40-1.61). Most subgroups according to age, sex, and comorbid conditions demonstrated consistent bidirectional associations between MD and osteoporosis. Adult patients with MD had a greater risk of osteoporosis. In addition, adult patients with osteoporosis also showed a higher risk of MD.

The Reciprocal Relationship between Osteoporosis and Renal Stones.

Previous studies have proposed an association between osteoporosis and renal stones. The current analyses intended to investigate the bidirectional relationship between osteoporosis and renal stones. The ≥40-year-old population in the National Health Insurance Service-Health Screening cohort (2002-2015) was analyzed. In study I, 67,811 patients with osteoporosis and 67,811 control I participants were matched. The hazard ratio (HR) of osteoporosis for renal stones was calculated using stratified Cox proportional hazard models. In study II, 25,261 patients with renal stones and 101,044 control II participants were matched. The HR of renal stones for osteoporosis was estimated using stratified Cox proportional hazard models. In study I, 3.4% (2276/67,811) of osteoporosis patients and 2.5% (1696/67,811) of control I participants had renal stones. Osteoporosis patients had a 1.36 times higher HR for renal stones than control I participants (95% confidence intervals [CI] = 1.28-1.45). In study II, 9.2% (2319/25,261) of renal stone patients and 7.6% (7658/101,044) of control II participants had osteoporosis. Renal stone patients had a 1.26 times higher HR for osteoporosis than control II participants (95% CI = 1.21-1.32). Adults with osteoporosis had a higher risk of renal stones. Moreover, adults with renal stones had a higher risk of osteoporosis.

Thyroid Cancer and Psoriasis: A Nested Case-Control Study.

Previous researchers have suggested an elevated risk of thyroid cancer (TC) in patients with psoriasis with mixed results. The current study evaluated the relationship of psoriasis with the risk of TC in an adult population. The data from the Korean National Health Insurance Service­Health Screening Cohort ≥ 40 years old were analyzed. In total, 6822 patients with TC were equalized with 27,288 control participants using overlap weighting adjustment based on the propensity score. The history of psoriasis was compared between the TC and control groups using multivariable logistic regression analysis. Secondary analyses were conducted according to age, sex, income, region of residence, systolic blood pressure, diastolic blood pressure, fasting blood glucose, total cholesterol, obesity, smoking, alcohol consumption, the Charlson Comorbidity Index scores, hypothyroidism, goiter, thyrotoxicosis, and thyroiditis. The history of psoriasis was not different in patients with TC (overlap-weighted odds ratio (OR) = 1.02, 95% confidence intervals (CI) = 0.85−1.22). The <55-year-old group showed a high rate of TC associated with psoriasis (overlap-weighted OR = 1.69, 95% CI = 1.22−2.36, p = 0.002). The population without hypothyroidism demonstrated an increased rate of TC related to psoriasis (overlap-weighted OR = 1.29, 95% CI = 1.06−1.57, p = 0.012). The patients with hypothyroidism showed a low rate of TC for psoriasis (overlap-weighted OR = 0.59, 95% CI = 0.37−0.96, p = 0.034). None of the other subgroups showed an association between psoriasis and TC. Psoriasis was not related to the risk of TC in the overall adult population. Young adults and populations without hypothyroidism indicated an elevated rate of TC for psoriasis.

Changes in Otorhinolaryngologic Disease Incidences before and during the COVID-19 Pandemic in Korea.

This study aimed to investigate the change in the incidence and variance of otorhinolaryngologic diseases during the coronavirus disease 19 (COVID-19) pandemic. The entire Korean population (~50 million) was evaluated for the monthly incidence of 11 common otorhinolaryngologic diseases of upper respiratory infection (URI), influenza, acute tonsillitis, peritonsillar abscess, retropharyngeal and parapharyngeal abscess, acute laryngitis and bronchitis, stomatitis and related lesions, acute sinusitis, rhinitis, otitis media, and dizziness from January 2018 through March 2021 using the International Classification of Disease (ICD)-10 codes with the data of the Korea National Health Insurance Service. The differences in the mean incidence of 11 common otorhinolaryngologic diseases before and during COVID-19 were compared using the Mann-Whitney U test. The differences in the variance of incidence before and during COVID-19 were compared using Levene's test. The incidence of all 11 otorhinolaryngologic diseases was lower during COVID-19 than before COVID-19 (all p < 0.05). The variations in disease incidence by season were lower during COVID-19 than before COVID-19 for infectious diseases, including URI, influenza, acute tonsillitis, peritonsillar abscess, retropharyngeal and parapharyngeal abscess, acute laryngitis and bronchitis, acute sinusitis, and otitis media (all p < 0.05), while it was not in noninfectious diseases, including stomatitis, rhinitis, and dizziness. As expected, the incidences of all otorhinolalryngolgic diseases were decreased. Additionally, we found that seasonal variations in infectious diseases disappeared during the COVID-19 pandemic, while noninfectious diseases did not.

Smoking Is Positively Related and Alcohol Consumption Is Negatively Related to an Increased Risk of Meniere's Disease.

A few prior researchers presumed the impacts of smoking and alcohol on the risk of Meniere's disease (MD). This study investigated the relationship between smoking, alcohol consumption, and obesity with Meniere's disease in an adult population. The ≥40-year-old population in the Korean National Health Insurance Service-Health Screening Cohort 2002−2019 was analyzed. A total of 15,208 patients with MD were matched with 499,658 comparison participants. The current smoking, alcohol consumption, and past medical histories were collected. Body mass index (BMI) was grouped into underweight, normal, overweight, obese I, and obese II. The odds of histories of smoking and alcohol consumption and the BMI group for MD were analyzed using conditional logistic regression analysis. These associations were further analyzed in subgroups of age, sex, smoking, alcohol consumption, and BMI. In the overall adult population, smoking and alcohol consumption did not show an association with MD. Being underweight was linked with lower odds for MD (adjusted OR [aOR] = 0.80, 95% CI = 0.68−0.93, p = 0.004). In the male group, smoking was positively associated with MD (aOR = 1.08, 95% CI = 1.00−1.17, p = 0.043), while alcohol consumption was negatively related to MD (aOR = 0.87, 95% CI = 0.81−0.94, p < 0.001). Being underweight was related to a lower risk of MD. In adult men, smoking was predicted to increase, while alcohol consumption was predicted to decrease the risk of MD.

Effectiveness of Intratympanic Dexamethasone Injection for Tinnitus Treatment: A Systematic Review and Meta-Analysis.

OBJECTIVES: Intratympanic dexamethasone injection (ITDI) has been introduced as a treatment option for subjective tinnitus. However, the effects of ITDI on patients with tinnitus remain unclear. In the present systematic review and metaanalysis, we evaluated the effectiveness of ITDI for tinnitus treatment. METHODS: We searched Medline, the Cochrane Central Register of Controlled Trials, and Embase. Four double-blind randomized controlled trials that tested the efficacy of ITDI compared with a placebo were deemed eligible for a quantitative meta-analysis, while four prospective studies and seven retrospective studies reporting the effectiveness of ITDI on tinnitus treatment were included in a qualitative synthesis. RESULTS: In the four studies included in the quantitative meta-analysis, ITDI did not show evidence of tinnitus improvement compared with placebo (odds ratio [OR], 1.38; 95% confidence interval, 0.53-3.61). In the 11 studies included in the qualitative synthesis, seven retrospective studies without controls reported rates of tinnitus improvement after ITDI ranging from 35.9% to 91.3%. In the four prospective studies with controls, ITDI seemed to be effective when combined with other drugs for tinnitus treatment. CONCLUSION: ITDI alone did not show a significant effect for treating tinnitus compared with placebo. However, the potential of combination treatment of ITDI with other drugs for tinnitus therapy should be further studied in more systematic research.

The Risk of BPPV, Meniere's Disease, and Vestibular Neuronitis in Patients with Gout: A Longitudinal Follow-Up Study Using a National Health Screening Cohort.

This study evaluated the impact of pre-existing gout on the occurrence of benign paroxysmal positional vertigo (BPPV), Meniere's disease, and vestibular neuronitis, with the goal of identifying novel associations of gout with other comorbid diseases. The 2002−2019 Korean National Health Insurance Service Health Screening Cohort data were retrospectively analyzed. 23,827 patients with gout were matched to 95,268 controls without gout for age, sex, income, region of residence, and index date. The occurrence of BPPV, Meniere's disease, and vestibular neuronitis was evaluated in both groups. The hazard ratios (HRs) of gout for BPPV, Meniere's disease, and vestibular neuronitis were calculated using a stratified Cox proportional hazard model. Participants with gout demonstrated a 1.13-fold higher risk of BPPV (95% CI, 1.06−1.21, p < 0.001) and a 1.15-fold higher risk of Meniere's disease (95% CI, 1.15−1.37, p < 0.001) than the matched control group. However, the HR for vestibular neuronitis was not significantly higher in the gout group (adjusted HR = 1.06, 95% CI, 0.93−1.21, p = 0.391). A previous history of gout was related to a higher risk of BPPV and Meniere's disease. Additional studies are necessary to elucidate the mechanism underlying the relationship between gout and comorbid diseases such as BPPV and Meniere's disease.

Development and Characterization of a Biomimetic Totally Implantable Artificial Basilar Membrane System.

Cochlear implants (CIs) have become the standard treatment for severe-to-profound sensorineural hearing loss. Conventional CIs have some challenges, such as the use of extracorporeal devices, and high power consumption for frequency analysis. To overcome these, artificial basilar membranes (ABMs) made of piezoelectric materials have been studied. This study aimed to verify the conceptual idea of a totally implantable ABM system. A prototype of the totally implantable system composed of the ABM developed in previous research, an electronic module (EM) for the amplification of electrical output from the ABM, and electrode was developed. We investigated the feasibility of the ABM system and obtained meaningful auditory brainstem responses of deafened guinea pigs by implanting the electrode of the ABM system. Also, an optimal method of coupling the ABM system to the human ossicle for transducing sound waves into electrical signals using the middle ear vibration was studied and the electrical signal output according to the sound stimuli was measured successfully. Although the overall power output from the ABM system is still less than the conventional CIs and further improvements to the ABM system are needed, we found a possibility of the developed ABM system as a totally implantable CIs in the future.

Comparison of endoscopic and microscopic ear surgery in pediatric patients: A meta-analysis.

OBJECTIVES: Recently, the endoscope has been increasingly introduced for middle-ear surgery. To evaluate the postoperative outcomes of endoscopic ear surgery (EES) in pediatric patients, we did a qualitative analysis with a systematic review and quantitative analysis with meta-analysis of available literature. METHODS: Studies reporting the comparative surgical outcomes of EES in pediatric patients were systematically reviewed by searching the MEDLINE, PubMed, and Embase databases from database inception through 2017. The selected articles included clinical studies conducted with at least 30 subjects and at least one postoperative parameter, including residual or recurrent cholesteatoma and graft success in tympanoplasty. Two investigators independently reviewed all studies and extracted the data using a standardized form. A meta-analysis was performed using a random-effects model and qualitative review was performed on the smaller studies. RESULTS: Ten studies were identified as appropriate for quantitative meta-analysis and 19 studies for qualitative analysis. In the meta-analysis, residual or recurrence rate of cholesteatoma was significantly lower in the EES group than in the microscopic ear surgery (MES) group (odds ratio [OR]: 0.56, 95% confidence interval [CI]: 0.38-0.84, P = .005). The graft success rate of tympanoplasty was not statistically different between EES and MES groups (OR: 0.72, 95% CI: 0.41-1.26, P = .249). In the qualitative analysis, most of the studies reported similar audiological outcomes after tympanoplasty and success rate of cholesteatoma removal between the two groups. CONCLUSIONS: It appears that EES reduces the risk of residual cholesteatoma in children and that the success of perforation closure is equivalent to MES. Laryngoscope, 129:1444-1452, 2019.

Cochlear Damage Caused by the Striking Noise of Titanium Head Golf Driver.

OBJECTIVES: To investigate how mouse cochleae are affected by the striking noise of titanium head golf driver. METHODS: Thirty-two BALB/c mice (20-22 g) with normal hearing were used. The impact acoustic stimulus generated by the striking of titanium golf driver head was centered around 4.5 kHz with 120.5 dB sound pressure level. The recorded impact noise was provided to mice in two ways with the same exposure time of 288 seconds: 1,440 repetitions and an impact duration of 0.2 seconds for 2 hours (repetitive noise) or serially connected impact noise for 288 seconds (continuous noise). Auditory brainstem responses were measured at baseline, day 7, and day 14 after exposure. The mice were then sacrificed for histology. RESULTS: Both groups showed statistically significant threshold shifts immediately after noise exposure. Mice in the continuous exposure group, except for those exposed to 32 kHz noise, recovered from threshold shifts 1-2 weeks after noise exposure. However, in the repetitive exposure group, threshold shifts remained for 2 weeks after exposure. The repetitive exposure group had greater hair cell damage than did the continuous exposure group. Structural changes in the stria vascularis were observed in the repetitive exposure group. CONCLUSION: Overexposure to impact noise caused by hitting of titanium head golf driver may be hazardous to the cochlea, and repetitive exposure may induce greater damage than continuous exposure.

Does the Width of the Bony Cochlear Nerve Canal Predict the Outcomes of Cochlear Implantation?

A narrow bony cochlear nerve canal (BCNC) is associated with sensorineural hearing loss necessitating cochlear implantation (CI). This study evaluated the implications of BCNC width for post-CI outcomes. A total of 56 children who had received CIs were included. The patients were divided into three groups according to the width of the BCNC (Group 1: diameter < 1.4 mm, n = 17; Group 2: diameter 1.4-2.0 mm, n = 14; Group 3: diameter > 2.0 mm, n = 25). The post-CI speech performances were compared among the three groups according to BCNC width. The correlation between BCNC width and post-CI speech performance was evaluated. Logistic regression analysis was also performed to investigate factors that can impact post-CI speech performance. Cochlear nerve deficiency (CND) occurred more frequently in Group 1. Groups 1 and 2 had significantly worse post-CI outcomes. Patients with intact cochlear nerves had significantly better post-CI outcomes than those with CND. When the cochlear nerve was intact, patients with a narrower BCNC showed less favorable results. Therefore, patients with either a narrow BCNC or CND seemed to have poorer outcomes. A narrow BCNC is associated with higher CND rates and poor outcomes. Measurement of BCNC diameter may help predict CI outcomes.

Management of myringitis: Current concepts and review.

OBJECTIVES: This study reviews the proposed mechanisms underlying the development of myringitis and suggests optimal guidelines for the successful treatment and prevention of recurrence through a literature review. REVIEW METHODS: Studies reporting the treatment outcomes of myringitis were systematically reviewed by searching the MEDLINE, PubMed, and Embase databases for studies published from 2000 to 2017. The search for myringitis yielded 57 results, and 31 were found to be relevant. Thirteen articles related to bullous myringitis associated with acute otitis media were excluded; the remaining 18 were included in this study. The studies were reviewed with attention to clinical characteristics, treatment options, and results after treatment. RESULTS: Among 18 publications, 10 suggested therapeutic options for myringitis: 1) five studies suggested management using topical agents, 2) three studies suggested laser-assisted treatment, and 3) two studies suggested surgical treatment. Treatments using topical agents or a laser showed highly variable resolution rates (63.6%-100% and 20%-85.7%, respectively). Two studies introducing surgical therapy reported good treatment outcomes (95.8%-100%). Five articles discussed the characteristics or diagnosis of myringitis; however, there was a lack of evidence for the related mechanism. CONCLUSIONS: No well-designed randomized studies have assessed optimal treatments. Although surgery showed better treatment results than topical agents and laser-assisted treatment, the wide range of resolution rates indicates that proper patient selection is needed. Further studies are needed to improve the current understanding and management of myringitis. Laryngoscope, 128:2157-2162, 2018.

The Analysis of A Frequent TMPRSS3 Allele Containing P.V116M and P.V291L in A Cis Configuration among Deaf Koreans.

We performed targeted re-sequencing to identify the genetic etiology of early-onset postlingual deafness and encountered a frequent TMPRSS3 allele harboring two variants in a cis configuration. We aimed to evaluate the pathogenicity of the allele. Among 88 cochlear implantees with autosomal recessive non-syndromic hearing loss, subjects with GJB2 and SLC26A4 mutations were excluded. Thirty-one probands manifesting early-onset postlingual deafness were sorted. Through targeted re-sequencing, we detected two families with a TMPRSS3 mutant allele containing p.V116M and p.V291L in a cis configuration, p.[p.V116M; p.V291L]. A minor allele frequency was calculated and proteolytic activity was measured. A p.[p.V116M; p.V291L] allele demonstrated a significantly higher frequency compared to normal controls and merited attention due to its high frequency (4.84%, 3/62). The first family showed a novel deleterious splice site variant-c.783-1G>A-in a trans allele, while the other showed homozygosity. The progression to deafness was noted within the first decade, suggesting DFNB10. The proteolytic activity was significantly reduced, confirming the severe pathogenicity. This frequent mutant allele significantly contributes to early-onset postlingual deafness in Koreans. For clinical implication and proper auditory rehabilitation, it is important to pay attention to this allele with a severe pathogenic potential.

Corrigendum: Determinants of Conductive Hearing Loss in Tympanic Membrane Perforation: Correction of the Sixth Author Name.

[This corrects the article on p. 92 in vol. 8, PMID: 26045905.].

Determinants of conductive hearing loss in tympanic membrane perforation.

OBJECTIVES: Tympanic membrane perforations are common, but there have been few studies of the factors determining the extent of the resulting conductive hearing loss. The aims of this study were to determine whether the size of tympanic membrane perforation, pneumatization of middle ear & mastoid cavity, and location of perforation were correlated with air-bone gap (ABG) of patients. METHODS: Forty-two patients who underwent tympanoplasty type I or myringoplasty were included and preoperative audiometry were analyzed. Digital image processing was applied in computed tomography for the estimation of middle ear & mastoid pneumatization volume and tympanic membrane photograph for the evaluation of perforation size and location. RESULTS: Preoperative mean ABG increased with perforation size (P=0.018), and correlated inversely with the middle ear & mastoid volume (P=0.005). However, perforations in anterior versus posterior locations showed no significant differences in mean ABG (P=0.924). CONCLUSION: The degree of conductive hearing loss resulting from a tympanic membrane perforation would be expected with the size of perforation and pneumatization of middle ear and mastoid.

De novo large genomic deletions involving POU3F4 in incomplete partition type III inner ear anomaly in East Asian populations and implications for genetic counseling.

OBJECTIVE: The aim of this study was to understand the prevalence and molecular genetic etiology of incomplete partition type III (IP type III) anomaly in Koreans. We also attempted to verify the prevalence of genomic deletions in the DFNX2 locus and to look for association between inheritance patterns and mutation type in East Asian IP type III subjects. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Subjects with IP type III anomaly and their biological mothers. INTERVENTIONS: Sanger sequencing, array-comparative genomic hybridization (aCGH), and PCR were performed. We also analyzed the type and inheritance of the causative genetic abnormality in East Asian DFNX2 patients. MAIN OUTCOME MEASURE: Mutation type and occurrence. RESULTS: We identified IP type III in 10 (4.8%) of 206 patients with an inner ear abnormality. We confirmed an etiologic homogeneity, DFNX2, of the IP type III in this Korean population. Two (20%) of the 10 DFNX2 carried a large genomic deletion affecting POU3F4, as proved by aCGH. PCR confirmed that the 2 deletions occurred de novo. Genetic alteration occurred de novo in 29.4% (5/17) of all reported Korean IP type III cases. From this study and literature review, we observed a striking difference of de novo occurrence rate (75% versus 12.5%, p = 0.032) between large genomic deletions and point mutations in East Asian population. CONCLUSIONS: Our data suggest that different POU3F4 mutations might show different recurrence rate in siblings of the IP type III families, especially in East Asian population. Genetic counseling should be provided accordingly.

Whole-exome sequencing identifies a novel genotype-phenotype correlation in the entactin domain of the known deafness gene TECTA.

Postlingual progressive hearing loss, affecting primarily the high frequencies, is the clinical finding in most cases of autosomal dominant nonsyndromic hearing loss (ADNSHL). The molecular genetic etiology of ADNSHL is extremely heterogeneous. We applied whole-exome sequencing to reveal the genetic etiology of high-frequency hearing loss in a mid-sized Korean family without any prior linkage data. Whole-exome sequencing of four family members (two affected and two unaffected), together with our filtering strategy based on comprehensive bioinformatics analyses, identified 21 potential pathogenic candidates. Sanger validation of an additional five family members excluded 20 variants, leaving only one novel variant, TECTA c.710C>T (p.T237I), as the strongest candidate. This variant resides in the entactin (ENT) domain and co-segregated perfectly with non-progressive high-frequency hearing loss in the family. It was absent among 700 ethnically matched control chromosomes, and the T237 residue is conserved among species, which supports its pathogenicity. Interestingly, this finding contrasted with a previously proposed genotype-phenotype correlation in which variants of the ENT domain of TECTA were associated with mid-frequency hearing loss. Based upon what we observed, we propose a novel "genotype to phenotype" correlation in the ENT domain of TECTA. Our results shed light on another important application of whole-exome sequencing: the establishment of a novel genotype-phenotype in the molecular genetic diagnosis of autosomal dominant hearing loss.

A Case of Post-Traumatic Meniere's Disease.

There are only a few reports of post-traumatic Meniere's disease and there is few literature that contains detailed data associated with the disease. We report a case of post-traumatic Meniere's disease. He suffered from tinnitus, fluctuating sensorineural hearing loss, and recurrent vertigo. Symptomatic medical treatment was not helpful and neither was soft tissue plugging around the oval and round windows during exploratory tympanotomy. Three months after soft tissue plugging, endolymphatic sac decompression surgery was performed. The patient's symptoms improved markedly thereafter. The clinical significance of post-traumatic Meniere's disease is described and we present a brief review of the literature.

A novel mutation of TMPRSS3 related to milder auditory phenotype in Korean postlingual deafness: a possible future implication for a personalized auditory rehabilitation.

UNLABELLED: Appropriate customized auditory rehabilitation for hearing impaired subjects requires prediction of residual hearing and progression of hearing loss. Mutations in TMPRSS3 encoding a transmembrane serine protease were reported to be associated with two different autosomal recessive nonsyndromic hearing loss (arNSHL) phenotypes, DFNB8 and DFNB10, in terms of residual hearing that may mandate different rehabilitation. We aimed to reveal the genetic contribution of TMPRSS3 mutations among Korean populations and to correlate the clinical phenotype with TMPRSS3 genotypes. Fifty families that segregated arNSHL and have visited our clinic recently for 2 years were recruited for TMPRSS3 screening. Novel TMPRSS3 variants detected in our cohort were modeled using a predicted three-dimensional (3D) structure of the serine protease domain. The prevalence reached up to 11.2 % (3/27) among subjects with either prelingual hearing loss but retaining some degree of language development or with postlingual ski-slope hearing loss. We also found that a p.A306T allele is a founder allele in this population. Based upon the 3D modeling, we were able to correlate significant retention of residual low-frequency hearing and slower progression of its loss to this novel variant p.T248M that was predicted to have milder pathogenicity. A yeast-based protease assay confirmed a mild pathogenic potential of the p.T248M variant and a tight correlation between the protease activity and the residual hearing. Preservation of this low-frequency hearing should be of utmost importance when considering auditory rehabilitation. Our results significantly narrow down the candidate population for TMPRSS3 sequencing for more efficient genetic diagnosis. More importantly, genotype-phenotype correlation of this gene observed in our cohort suggests that TMPRSS3 can be an appropriate candidate for personalized and customized auditory rehabilitation. KEY MESSAGE: The prevalence of TMPRSS3 mutations among Korean postlingual hearing loss is 8.3 %. The p.A306T variant of TMPRSS3 is the common founder allele in Koreans. A novel variant, p.T248M of TMPRSS3, was predicted to have milder pathogenicity. There was a genotype-phenotype correlation of this gene in Koreans. Our data support implication of this gene for personalized rehabilitation.