Suppression of the TGF-ß signaling exacerbates degeneration of auditory neurons in kanamycin-induced ototoxicity in mice.

Transforming growth factor-ß (TGF-ß) signaling plays a significant role in multiple biological processes, including inflammation, immunity, and cell death. However, its specific impact on the cochlea remains unclear. In this study, we aimed to investigate the effects of TGF-ß signaling suppression on auditory function and cochlear pathology in mice with kanamycin-induced ototoxicity. Kanamycin and furosemide (KM-FS) were systemically administered to 8-week-old C57/BL6 mice, followed by immediate topical application of a TGF-ß receptor inhibitor (TGF-ßRI) onto the round window membrane. Results showed significant TGF-ß receptor upregulation in spiral ganglion neurons (SGNs) after KM-FA ototoxicity, whereas expression levels in the TGF-ßRI treated group remained unchanged. Interestingly, despite no significant change in cochlear TGF-ß expression after KM-FS ototoxicity, TGF-ßRI treatment resulted in a significant decrease in TGF-ß signaling. Regarding auditory function, TGF-ßRI treatment offered no therapeutic effects on hearing thresholds and hair cell survival following KM-FS ototoxicity. However, SGN loss and macrophage infiltration were significantly increased with TGF-ßRI treatment. These results imply that inhibition of TGF-ß signaling after KM-FS ototoxicity promotes cochlear inflammation and SGN degeneration.

Effect of statins on hearing outcome in patients with idiopathic sudden sensorineural hearing loss.

Objective: Statins have been reported to improve vascular endothelial function and microcirculation, reduce oxidative stress, and exert anti-inflammatory and protective effects against inner ear damage. Therefore, this study aimed to investigate the effect of statins on hearing prognosis in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Methods: We reviewed the medical records of 149 patients diagnosed with ISSNHL. Clinical characteristics, hearing thresholds, statin medications, and hematological findings were investigated. First, patients with ISSNHL were assigned to the good and poor outcome groups, and factors influencing their prognosis were analyzed. Furthermore, patients with dyslipidemia were investigated to determine whether statins have therapeutic effects on ISSNHL. Results: Significant differences in age (p = .011), days from the onset of ISSNHL to the initiation of treatment (p = .04), and hematological total cholesterol (TC; p = .015) between the good and poor outcome groups were observed. Furthermore, when hearing outcomes were investigated in patients with dyslipidemia, TC was significantly lower in the good outcome group (p = .03). Although no significant therapeutic effects of statins were observed in participants with dyslipidemia, patients in the statin-treated group were significantly older and experienced more diabetic complications than those in the non-statin-treated group. Conclusion: Although our study showed that dyslipidemia is a poor prognostic factor for ISSNHL, statins had no significant therapeutic effects on hearing recovery in ISSNHL patients with dyslipidemia. The patients that received statin medications were significantly older and experienced more diabetic complications, which may have affected their hearing prognosis. Level of Evidence: Level 4.

Is repetitive systemic corticosteroid therapy effective for idiopathic sudden sensorineural hearing loss? a retrospective study.

Introduction: Some idiopathic sudden sensorineural hearing loss (ISSHL) cases experience repetitive systemic corticosteroid treatment, but studies focusing on repetitive systemic corticosteroid administration have not been reported. Thus, we investigated the clinical characteristics and usefulness of repetitive systemic corticosteroid treatment in ISSHL cases. Methods: We reviewed the medical records of 103 patients who received corticosteroids only in our hospital (single-treatment group), and 46 patients who presented at our hospital after receiving corticosteroids in a nearby clinic and were subsequently treated with corticosteroids again in our hospital (repetitive-treatment group). Clinical backgrounds, hearing thresholds, and hearing prognosis were assessed. Results: The final hearing outcomes were not different between the two groups. Further, in the repetitive-treatment group, statistical differences were found between the good and poor prognosis groups in the number of days to start corticosteroid administration (p = 0.03), the dose of corticosteroid (p = 0.02), and the duration of corticosteroid administration (p = 0.02) at the previous facility. Multivariate analysis revealed a significant difference in the dose of corticosteroids administered by the previous clinic (p = 0.004). Conclusion: The repetitive systemic corticosteroid administration might play a supplementary role in hearing improvement, and initial sufficient corticosteroid administration would lead to good hearing outcomes in an early phase of ISSHL.

Investigation of hearing aid fitting according to the national acoustic laboratories' prescription for non-linear hearing aids and the desired sensation level methods in Japanese speakers: a crossover-controlled trial.

OBJECTIVE: Some studies have directly compared the National Acoustic Laboratories' prescription for non-linear hearing aids (HAs) version 2 (NAL-NL2) and Desired Sensation Level for non-linear HAs version 5 (DSLv5), although none were performed in Japan. As the Japanese language is a tonal language that has different linguistic characteristics than those of the studied languages, we compared the outcomes of the NAL-NL2 and DSLv5 in hearing-impaired Japanese participants. METHODS: A crossover-controlled trial was conducted on 18 first-time HA users with bilateral moderate sensorineural hearing loss. Participants wore HAs adjusted with each prescriptive method for four weeks. The prescriptions were assessed using speech discrimination testing and the abbreviated profile of hearing aid benefit (APHAB). Consequently, participants were asked to select their preferred prescription and determine which was better for "listening to a conversation" and when "noisy." RESULTS: The mean DSLv5 real ear insertion gain for an input level of 65 dB sound pressure level (SPL) was higher than that of the NAL-NL2 at 250 and 500 Hz. The average speech discrimination score was 78 ± 14% at a 65-dB SPL and 75 ± 17% at an 80-dB SPL for the NAL-NL2, and 79 ± 11% at a 65-dB SPL and 77 ± 17% at an 80-dB SPL for the DSLv5. These differences were not significant. No significant differences were observed in APHAB subscale scores between the two prescription methods. Ultimately, 11 (61%) and 7 (39%) participants preferred the NAL-NL2 and DSLv5, respectively, with no significant differences. CONCLUSION: Although the gain of the NAL-NL2 is smaller than that of the DSLv5, both had the same hearing effect. Therefore, the NAL-NL2 may be more useful than the DSLv5 in Japanese.

Clinical Characteristics and Corticosteroid Responses of Acoustic Neuroma Treated as Idiopathic Sudden Sensorineural Hearing Loss.

BACKGROUND: Few investigations have been conducted on the clinical characteristics of the differential diagnosis of acoustic neuroma with acute sensorineural hearing loss and idiopathic sudden sensorineural hearing loss. The aim of the study was to investigate the clinical characteristics of the differential diagnoses between acoustic neuroma and idiopathic sudden sensorineural hearing loss. METHODS: The medical records of patients with acute sensorineural hearing loss (142 ears), including acoustic neuroma (19 ears) and idiopathic sudden sensorineural hearing loss (123 ears), who underwent audiometric and hematologic examinations and received systemic corticosteroid treatment, were retrospectively reviewed. RESULTS: Hematological examination revealed that the erythrocyte sedimentation rate and fibrinogen values were significantly higher in the idiopathic sudden sensorineural hearing loss group compared to the acoustic neuroma group. Although all patients received corticosteroid treatment, hearing thresholds at the initial examination and 3 months after corticosteroid treatment were significantly higher in the idiopathic sudden sensorineural hearing loss group compared to the acoustic neuroma group at all frequencies. However, hearing recovery was worse in the acoustic neuroma group compared to the idiopathic sudden sensorineural hearing loss group. Furthermore, speech discrimination and short increment sensitivity index tests were not significantly different between the acoustic neuroma and idiopathic sudden sensorineural hearing loss groups. CONCLUSION: This is the first study to reveal that speech discrimination and short increment sensitivity index tests are not useful for the differential diagnoses between acoustic neuroma and idiopathic sudden sensorineural hearing loss, whereas erythrocyte sedimentation rate and fibrinogen, blood biomarkers of inflammation and blood viscosity, would be considered valuable. Furthermore, acoustic neuroma should be considered in cases where acute sensorineural hearing loss did not recover after corticosteroid treatment, although the initial hearing loss was mild.

Investigation of the current supply system for hearing aids at stores in Japan.

OBJECTIVE: This study aimed to examine the status of the supply system for hearing aids at stores in Japan. METHODS: This was a cross-sectional, observational study. We mailed questionnaires to 4502 hearing aid stores in Japan and analyzed the responses. RESULTS: Of the 4502 stores, 1032 (23%) responded. Most stores accessed audiogram information and adjusted hearing aids using computer fitting software. Overall, 43% of the stores only used the manufacturer's original prescription formula to adjust the hearing aid, and 47% of stores lacked a device for measuring the frequency response of the hearing aids. Moreover, 20% of the stores lacked both a sound field threshold measurement system and a real-ear measurement device. Objective evaluations, including functional gain, rear-ear insertion gain, and frequency response data, were not performed by 14% stores. Speech-language-hearing therapists were present in only 5.6% stores. CONCLUSION: The findings revealed several key shortcomings in the hearing aid supply system at hearing aid stores in Japan.

Abatacept ameliorates both glandular and extraglandular involvements in patients with Sjögren's syndrome associated with rheumatoid arthritis: Findings from an open-label, multicentre, 1-year, prospective study: The ROSE (Rheumatoid Arthritis with Orencia Trial Toward Sjögren's Syndrome Endocrinopathy) and ROSE II trials.

OBJECTIVE: To clarify the efficacy and safety of intravenous abatacept for glandular and extraglandular involvements in Sjögren's syndrome (SS) associated with rheumatoid arthritis (RA). MATERIALS AND METHODS: We performed an open-label, prospective, 1-year, observational multicenter study (ROSE and ROSE II trials). The primary endpoint was the remission rate as measured by SDAI at 52 weeks. The secondary endpoints included the changes in the Saxon's test, Schirmer's test, ESSDAI and ESSPRI. Adverse events and adherence rates were also analyzed. RESULTS: 68 patients (36 in ROSE and 32 in ROSE II, all women) were enrolled. SDAI decreased significantly from 23.6 ± 13.2 at baseline to 9.9 ± 9.5 at 52 weeks. Patients with SDAI remission increased from 0 (0 weeks) to 19 patients (27.9%) at 52 weeks. Saliva volume increased significantly at 24 weeks. Tear volume increased significantly at 52 weeks. Both ESSDAI and ESSPRI were significantly decreased at 12 weeks, and these responses were maintained up to 52 weeks. The rate of adherence to abatacept over the 52-week period was 83.8%. Twenty-two adverse events occurred in 15 patients. CONCLUSION: Abatacept ameliorated both glandular and extraglandular involvements, as well as the systemic disease activities and patient-reported outcomes based on composite measures, in SS associated with RA.

Long-term outcomes of stapes surgery.

OBJECTIVE: Hearing improvement following stapes surgery is generally good; however, we sometimes encounter patients where hearing loss gradually progresses over the long term. In this study, we investigated the causes of hearing loss in these cases. METHODS: A total of 30 ears from 23 patients, who underwent stapes surgery at Kitasato University Hospital from 2001 to 2021 and were followed up for ≥5 years, were included in the study. Changes in air conduction (AC) and bone conduction (BC) thresholds were measured, and hearing evaluation was performed by calculating the air-bone gap (ABG) at the mean of 4 frequencies. Cases with a postoperative ABG of ≤10 dB at 6 months after surgery were classified into the following groups according to their hearing changes at 5 years after surgery: Group A (no ABG increase of ≥10 dB and no AC threshold increase of ≥10 dB) and Group B (an ABG increase of ≥10 dB and an AC threshold increase of ≥10 dB). In groups A and B, we examined factors affecting long-term postoperative results. In addition, the patients who underwent reoperation were examined. RESULTS: The AC thresholds 6 months and 5 years after surgery decreased significantly compared with those before surgery (p  <  0.01); however, the BC thresholds 6 months and 5 years after surgery did not vary significantly from those before surgery (p  >  0.05). Group A included 16 ears from 13 patients (53.3%), and Group B included 3 ears from 3 patients (10.0%). There were no significant differences in age, sex, surgical method, piston type, piston length, presence of incudostapedial joint subluxation, computed tomography findings, and preoperative ABGs between groups A and B (p  >  0.05). A total of 6 reoperations were performed in 3 ears from 3 patients, and there were 5 operations with the platinotomy hole to be closed by bone regrowth and 3 operations with the narrowed long process of the incus. CONCLUSION: In 10.0% of the patients who underwent stapes surgery, the ABG improved 6 months after surgery; however, the AC threshold and ABG increased 5 years after surgery. Our findings suggested that piston displacement and the platinotomy hole to be closed by bone regrowth are possible causes of hearing loss in cases where hearing loss progresses in the long term after stapes surgery.

Retrofacial Approach for Cochlear Implantation in Chronic Otitis Media: A Case Report.

Cochlear implantation is anatomically more difficult in postoperative ears than in normal ears due to chronic otitis media. In cochlear implantation after canal wall down mastoidectomy, the electrodes must be protected. We report a case of cochlear implantation with partial mastoid obliteration using a retrofacial approach in an 81-year-old woman. Bilateral tympanoplasty was performed for bilateral chronic otitis media, but no improvement in hearing was observed. Hearing aids were used, but their effect was insufficient and cochlear implantation was needed. Since the patient was an older adult with diabetes, it was necessary to avoid the risk of wound infection associated with fat harvesting from the abdomen, which is necessary for overclosure of the external ear. Therefore, we chose to perform partial mastoid obliteration using a retrofacial approach to ensure the prevention of electrode exposure. One year after surgery, the electrode had not been exposed or infected. The ear canal was preserved, and the eardrum could be observed. Although long-term follow-up is required, this approach may be useful for cochlear implantation in patients with chronic otitis media after canal wall down mastoidectomy.

Immune-Nutritional Status as a Novel Prognostic Predictor of Bell's Palsy.

INTRODUCTION: The prognosis of Bell's palsy, idiopathic facial nerve palsy (FNP), is usually predicted by electroneuronography in subacute phase. However, it would be ideal to establish a reliable and objective examination applicable in acute phase to predict the prognosis of FNP. Immune-nutritional status (INS) calculated from peripheral blood examination is recently reported as the prognostic factor in various disease. However, the validity of INS as the prognostic factor in Bell's palsy is not well known. Thus, we conducted a retrospective study to investigate the usefulness of INS as prognostic predictors of Bell's palsy. METHODS: We reviewed the medical records of 79 patients with Bell's palsy and divided into two groups as "complete recovery" and "incomplete recovery" groups. Clinical features such as severity of FNP and INS, including neutrophil-lymphocyte ratio (NLR), lymphocyte-monocyte ratio (LMR), prognostic nutritional index (PNI), and controlling nutrition status (CONUT) score, were assessed. RESULTS: In univariate analysis, statistically significant differences were observed in clinical score of facial movement, NLR, LMR, PNI, and CONUT score at the initial examination between the two groups (p < 0.05). Furthermore, in multivariate analysis, statistically significant differences were also observed in facial movement score and PNI at the initial examination (p < 0.05). CONCLUSION: Immune and nutritional condition play important roles in the pathogenesis of Bell's palsy, suggesting that INS would be one of the useful prognostic factors in Bell's palsy.

Clinical features and hearing prognosis of idiopathic sudden sensorineural hearing loss in patients undergoing hemodialysis: A retrospective study.

BACKGROUND: Patients undergoing hemodialysis (HD) tend to experience hearing loss, including idiopathic sudden sensorineural hearing loss (ISSHL). However, little is known about the relationship between HD and ISSHL. OBJECTIVE: To investigate the effects of HD on the hearing level and the treatment prognosis of ISSHL. METHODS: We reviewed the medical records of 23 patients with ISSHL receiving HD treatment (HD group) and 101 patients with ISSHL not receiving HD treatment (non-HD group), and assessed clinical features, results of audiometric tests and blood examination results. RESULTS: Statistically significant differences were not observed in pretreatment hearing level and hearing recovery of the ear affected with ISSHL between the two groups (P > .05). Conversely, hearing thresholds in the unaffected ear were statistically different (P < .0001), and the hearing thresholds of the HD groups were significantly increased compared with those of the non-HD groups, especially at high frequency. In addition, patients with renal dysfunction not receiving HD treatment showed similar hearing thresholds in the unaffected ear when compared with patients receiving HD treatment. CONCLUSION: HD itself did not influence the treatment prognosis of ISSHL. Renal dysfunction itself, and not HD treatment, worsened the hearing level. As similar treatment results are expected, standard treatment should be administered to patients undergoing HD. LEVEL OF EVIDENCE: 3b.

Evaluation of changes in oral health-related quality of life over time in patients with Sjögren's syndrome.

OBJECTIVES: To assess oral health-related quality of life (OHRQoL) and changes in OHRQoL in 3 years of patients with Sjögren's symdrome (SS). METHODS: Thirty-five SS patients and 23 non-SS individuals were enrolled. OHRQoL were quantitatively evaluated using the shortened Oral Health Impact Profile (OHIP-14). After 3 years, 22 patients and 14 controls tool the OHIP-14 survey again. RESULTS: The SS group had a significantly higher OHIP-14 score, which indicated a lower OHRQoL, than the non-SS group. Among individual questions in the OHIP-14, scores for 'trouble pronouncing words', 'uncomfortable to eat foods', 'self-conscious', and 'diet unsatisfactory' were markedly higher in the SS group than in the non-SS group. The OHIP-14 score significantly increased in 3 years in the SS group. Furthermore, there was an inverse correlation between the change rate of salivary flow rate and change of OHIP-14 scores in 3 years in patients with SS whose OHIP-14 score increased. Scores for 'irritable with other people', 'difficulty doing usual jobs', 'felt life less satisfying', and 'unable to function' significantly increased in 3 years. CONCLUSION: In SS, OHRQoL decreased in 3 years, which was associated with a decrease in saliva secretion. Moreover, troubles related to psychosocial aspects in SS patients were found to intensify over time.

Comparison of real-ear insertion gains in Japanese-speaking individuals wearing hearing aids with DSLv5 and NAL-NL2.

OBJECTIVE: We used real-ear insertion gain (REIG), with the international speech test signal (ISTS), to evaluate the amplifying characteristics of hearing aids, set for patients who have been wearing such aids for a long time in a stable manner. We further compared this to the target values of the DSLv5 and NAL-NL2 methods. METHODS: The subjects were adults with moderate sensorineural hearing loss. We examined 40 ears in 25 individuals (15 people wearing hearing aids in both ears and ten people wearing aid in only one ear). Fit assessments were performed based on the speech performance-intensity functions and tolerance of environmental noise, and the ears studied were categorized as either ears with sufficient benefit or ears with insufficient benefit. Additionally, we evaluated the REIG for international speech test signals at 65-dB and 80-dB sound pressure level (SPL). We compared the REIG and target values for voice input at 65-dB and 80-dB SPL, calculated from the DSLv5 and NAL-NL2 methods. RESULTS: Among the 40 ears, 34 received sufficient benefit and six received an insufficient benefit from hearing aids. The REIG for ISTS at 65-dB in the sufficient benefit ears, at frequencies of 1,000 Hz and 2,000 Hz, were similar to the target values of NAL-NL2 and DSLv5 but were significantly lower at 250 Hz, 500 Hz, and 4,000 Hz frequencies. The compression ratio of REIG for sufficient benefit ears was similar to that of DSLv5. The REIG for ISTS at 65-dB in the insufficient benefit ears was smaller than that in the sufficient benefit ears at frequencies of 2,000 Hz and 4,000 Hz. CONCLUSION: This study suggested that the target values of NAL-NL2 and DSLv5 are appropriate, even for Japanese-speaking individuals, at mid-pitch sounds. Although it is necessary to investigate the necessity for low-pitch and high-pitch gains further in the future, this study provides meaningful data regarding the amplifying characteristics in Japanese-speaking individuals who have been wearing hearing aids in a stable manner.

Speech discrimination impairment of the worse-hearing ear in asymmetric hearing loss.

OBJECTIVE: This study aimed to compare the difference in maximum speech discrimination score (SDSmax) of the worse-hearing ear in asymmetric hearing loss (ASHL) patients with that in symmetric hearing loss (SHL) patients. DESIGN: We retrospectively reviewed medical records of patients with suspected hearing loss (HL) who underwent audiometric examinations. Patients were divided into two groups according to the difference in air conduction (AC) threshold between the right and left ears: the SHL group and the ASHL group. STUDY SAMPLE: Of the 102 patients (204 ears), 74 (148 ears) had SHL, and 28 had ASHL. RESULTS: The worse-hearing ear of ASHL patients exhibited a statistically significantly higher AC threshold and lower SDSmax, compared with ears of SHL patients and better-hearing ears of ASHL patients, and SDSmax exhibited a statistically significant negative correlation with AC threshold. The SDSmax was statistically significantly lower in the worse-hearing ear of the ASHL group than in moderate to severe HL ears of the SHL group, even though these groups had comparable AC thresholds. CONCLUSIONS: ASHL patients' worse-hearing ear exhibited a lower SDSmax than SHL patients' ears, despite a comparable AC threshold. Management of hearing impairment in ASHL patients should receive more attention.

Long-term administration of vitamin B12 and adenosine triphosphate for idiopathic sudden sensorineural hearing loss: a retrospective study.

BACKGROUND: In idiopathic sudden sensorineural hearing loss (ISSNHL), the relationship between the administration duration of vitamin B12 (vit B12) with adenosine triphosphate (ATP) and their therapeutic effect is not fully understood. OBJECTIVE: To investigate the therapeutic effect of long-term 16 (≥weeks) administration of vit B12 with ATP on the prognosis of ISSNHL patients and compare it with those of short-term (<8 weeks) and middle-term (≥8 weeks, <16 weeks) administration. METHODS: We retrospectively reviewed the medical records of 117 patients with ISSNHL treated between 2015 and 2018. RESULTS: The overall recovery rate was 32.5%. Initial higher hearing threshold and initial higher grade of hearing loss (HL) were associated with a poor prognosis. However, the administration duration of vit B12 and ATP did not influence the overall hearing improvement. With regard to the time course of hearing recovery, there was no significant difference in hearing recovery among the long-, middle-, and short-term administration groups until 16 weeks after treatment. However, at 16-24 weeks after initial treatment, the short-term administration group exhibited significantly lower hearing recovery than did the long-term administration groups. CONCLUSIONS: The administration duration of vit B12 and ATP did not influence the overall hearing prognosis in ISSNHL, but long-term administration of vit B12 and ATP helped prevent the progression of HL after ISSNHL. Our results suggest that long-term administration of vit B12 and ATP is not necessarily required to treat ISSNHL patients, except for slowly progressing HL in the affected ears.

Recurrent myocardial infarction associated with vasculo-Behçet's disease: a case report.

A 39-year-old Japanese man presented with chest oppression in February 2017. Electrocardiogram showed ST-elevation myocardial infarction (MI), and cardiac catheterisation revealed thrombotic occlusion of the right coronary artery (RCA), which was treated with thrombectomy, and he received warfarin. Three days after discharge, he complained of chest oppression again, and re-cardiac catheterisation showed thrombi occlusion of the circumflex artery (LCX) and 90% stenosis with thrombosis in the proximal site of the anterior descending artery (LAD) and RCA. Drug eluting stent was implanted in the LAD and RCA; aspirin and prasugrel hydrochloride were added to warfarin. Before discharge, coronary computed tomography angiography (CTA) found new thrombi in the RCA, LAD, and LCX, and he was referred to our hospital on suspicion of Behçet's disease (BD). Past medical history was notable for recurrent aphthous stomatitis, a pudendal ulcer, and Crohn's disease, for which he had been taking infliximab (5 mg/kg) every 8 weeks until December 2016. Notably, his C-reactive protein (CRP) level increased before and after each MI, suggesting that the thrombi were caused by inflammation. Consequently, we concluded that his abnormalities were manifestations of vasculo-BD. After 3 days of hospitalisation, treatment with prednisolone and colchicine was started. His CRP and D-dimer levels decreased, and coronary CTA after 8 days showed disappearance of the thrombi. We tapered the prednisolone dose, and cardiovascular events have not been observed for 7 months after the treatment initiation. In summary, we report a rare case of MI associated with vasculo-BD and review the relevant literature.

Effects of the Conductive Component of Hearing Loss on Speech Discrimination Ability.

OBJECTIVES: Effects of decreasing auditory activity on speech discrimination ability are not fully understood. To investigate influence of decrease in auditory activity due to conductive and sensorineural components of hearing loss (HL) on speech discrimination ability. MATERIALS AND METHODS: We retrospectively reviewed medical records of patients with suspected HL at Kitasato University Hospital in 2017 and 2018. Patients were divided according to pure-tone audiometry findings: no HL (N-HL), conductive HL (C-HL), sensorineural HL (S-HL), and mixed HL (M-HL) groups. RESULTS: In total, 149 patients (224 ears) were eligible. The maximum speech discrimination score (SDSmax) for all ears significantly negatively correlated with age (r = -0.29, p<0.0001) and bone conduction (BC) threshold (r = -0.55, p<0.0001). For patients aged <50 years in N-HL and C-HL groups, SDSmax was nearly 100%, with no significant difference. SDSmax was significantly lower for older patients (≥50 years) in the M-HL group than in the S-HL group, even though there were no significant differences in age and BC thresholds between groups. CONCLUSION: Decrease of auditory activity due to the conductive component of M-HL may worsen speech discrimination ability. Early treatment of M-HL would be desirable for the preservation of auditory function.

First external validation of sensitivity and specificity of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for idiopathic inflammatory myopathies with a Japanese cohort.

OBJECTIVE: To externally validate the performance of the new European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria set for idiopathic inflammatory myopathies (IIM) with a Japanese cohort. METHODS: This study included 420 IIM and 402 non-IIM cases. Probability of having IIM in each patient was calculated using the collected data set. The cut-off probability was set at 55%, as recommended by EULAR/ACR. Patients classified as IIM by the criteria were further subclassified with classification trees. RESULTS: When the probability cut-off was set at 55%, the sensitivity/specificity of the new criteria to diagnose IIM were 89.3%/91.0% in the total cohort, 88.1%/95.1% without muscle biopsy data and 90.4%/65.5% with biopsy data. The cohort included 12 overlap syndrome patients with biopsy data, who were included as non-IIM cases in accordance with traditional Japanese methods. When they were included in the IIM cases, the specificity in patients with biopsy increased to 74.4%. The sensitivity/specificity of the new criteria to diagnose polymyositis/dermatomyositis (PM/DM) plus juvenile and amyopathic DM in the Japanese cohort was 87.4%/92.4%, which were greater than those of the Tanimoto's criteria revised to enable classification of amyopathic DM (ADM) (71.2%/87.8%) and were comparable with those of Bohan & Peter's criteria to diagnose those diseases except for ADM (88.4%/88.3%). CONCLUSIONS: Our study externally validated high specificity of the new criteria for the first time, although with several limitations, including low percentage of child patients. The new criteria have higher sensitivity and/or specificity in classification of PM/DM than the previously reported criteria, demonstrating its usefulness for interethnic patients.

Association of HLA-DRB1 genotype with younger age onset and elder age onset rheumatoid arthritis in Japanese populations.

Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by joint destructions and human leukocyte antigen (HLA)-DRB1 is an important genetic risk factor for RA and influences the phenotype of RA. The clinical features of elder age onset RA (EORA) were known to be different from those of younger age onset RA (YORA). Previous studies reported the different association pattern of DRB1 alleles with YORA or EORA. The associations of DRB1 genotype with these RA subsets remained almost unknown. We investigated the genotype association of DRB1 with YORA or EORA in Japanese populations.HLA genotyping was performed in Japanese RA patients and the association of allele or genotype carrier frequencies were analyzed.The genotype frequency of DRB104:05/DRB104:06 (P = .0204, OR 7.69, 95%CI 1.39-42.72), DRB104:05/DRB112:01 (P = .0050, OR 5.53, 95%CI 1.71-17.88), and DRB104:05/DRB115:01 (P = .0124, OR 3.34, 95%CI 1.39-8.02) in YORA was higher than EORA. However, the frequencies of DRB101:01/DRB104:05 in YORA was tended to be lower than EORA (P = .0784, OR 0.14, 95%CI 0.01-2.42). The gene dosage effect of the shared epitope alleles was detected in EORA, but not in YORA. Trans-complementing DQ heterodimer molecules, formed by DQA1 and DQB1 of the haplotypes with and without shared epitope alleles, might explain the higher genotype frequencies of "shared epitope /not shared epitope". Linear regression analyses showed the primary role of DQB104:01 allele for the age at onset of RA.This is the first report for the associations of DRB1 genotype with YORA or EORA in the Japanese population and the differential distribution of the genotypes was noted between these RA subsets. The involvement of DQ molecules for the age at onset of RA was suggested.