Altered serum levels of neuronal proteins and antibodies to them in occupational diseases of the nervous system.

Aclinical and immunological examination of men with occupational pathology, including vibration disease (VD), occupational sensorineural hearing loss (SHL), and chronic mercury intoxication (CMI), was carried out. The comparison group consisted of men comparable in age and total work experience. Serum concentrations of neurotrophins (S100ß, MBP, BDNF) and antibodies (ABs) to S100ß and MBP proteins were determined by enzyme-linked immunosorbent assay. An increase in the level of the S100ß protein was shown in CMI, VD, and a tendency for its increase was found in SHL. In parallel, an increase in AB to the S100ß protein in VD and SHL and a decrease in AB in CMI were noted. A comparative assessment of MBP levels indicated a pronounced increase in its serum concentrations in patients with CMI and VD versus the comparison group. At the same time, an increase in the level of serum ABs to MBP in individuals with VD and SHL, and a decrease in patients with CMI were noted. In patients with CMI, a significant decrease in the BDNF concentration was found, while in SHL and VD, no statistically significant differences were found in comparison with the comparison group. The results obtained confirm importance of assessing serum concentrations of neurotrophic proteins and ABs to them in the case of occupational damage to the nervous system caused by exposure to physical and chemical factors.

Association of Glycosylated Hemoglobin A1c Level With Sudden Sensorineural Hearing Loss: A Prospective Study.

Glycosylated hemoglobin A1c (HbA1c) level has strong relevance to microvascular disorders, which are also thought to be the current main aspect of sudden sensorineural hearing loss (SSNHL), so we aim to elucidate the association of the HbA1c level with the severity, types, and prognosis of SSNHL. In this study, comparative analyses based on propensity score matching of the severity, types, and prognosis of SSNHL with the HbA1c level in 116 patients diagnosed as SSNHL were conducted, where they were divided into diabetes mellitus (DM) group and non-DM group. We finally found that, among patients with SSNHL, diabetic patients had a higher HbA1c level, more severe hearing loss, and poorer prognosis than non-diabetic patients. The HbA1c level was found to be significantly correlated with the severity and types of SSNHL, while no strong relevance was found between the higher HbA1c level and the poorer prognosis of SSNHL.

Identification of dyslipidemia as a risk factor for sudden sensorineural hearing loss: A multicenter case-control study.

BACKGROUND: Recently, several studies have reported an association between lipid profiles and sudden sensorineural hearing loss (SSNHL), yet there is considerable variability between the individual studies in defining the precise association between serum lipids levels and SSNHL. This study sought to identify a possible relationship between dyslipidemia and the prevalence and prognosis of SSNHL. METHODS: A case-control study was carried out at two independent medical centers, including 2,288 SSNHL patients and 2,288 healthy controls. Clinical characteristics and serum lipid parameters were assessed, including total cholesterol (CHOL), high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglycerides (Trig), apolipoprotein AI (ApoAI), apolipoprotein B (ApoB), and lipoprotein a (Lpa). Multivariate logistic regression analysis was performed to assess the relationship between lipid profiles and SSNHL in the 4,576 subjects. RESULTS: Significant differences were identified in several conventional serum lipid markers including CHOL, Trig, HDL, LDL, ApoAI, ApoB, and Lpa, between SSNHL patients and healthy controls. Serum ApoAI levels were significantly lower in patients with bilateral SSNHL compared to unilateral SSNHL. Binary logistic regression analysis revealed that higher levels of ApoB, LDL, Trig, and lower levels of ApoAI and HDL were all associated with an increased risk of SSNHL. After clinical characterization, multivariate analysis showed that only low levels of ApoB predicted likelihood of a recovery of more than 30 dB among patients with SSNHL. CONCLUSIONS: Serum lipids are associated with the incidence and prognosis of SSNHL. Identification of dyslipidemia may improve early evaluation and management of SSNHL risks.

Iron deficiency is associated with poor prognosis in idiopathic sudden sensorineural hearing loss.

OBJECTIVE: The effects of iron deficiency on the prognosis of idiopathic sudden sensorineural hearing loss are unclear. This study aimed to investigate the association between serum iron levels and idiopathic sudden sensorineural hearing loss prognosis and its usefulness as an independent prognostic marker for idiopathic sudden sensorineural hearing loss. METHODS: The audiological and haematological data, including hearing recovery and serum iron levels, of 103 patients with idiopathic sudden sensorineural hearing loss evaluated between 2015 and 2018 were retrospectively analysed. RESULTS: The overall complete recovery rate was 16.5 per cent. Initial higher hearing threshold was associated with poor idiopathic sudden sensorineural hearing loss prognosis. Serum iron levels were significantly higher in the complete recovery group than in the non-complete recovery group (p < 0.05). CONCLUSION: The possibility of complete recovery from idiopathic sudden sensorineural hearing loss was significantly lower with lower serum iron levels, suggesting that the serum iron level might be a novel prognostic marker for idiopathic sudden sensorineural hearing loss.

The Effects of Genetic Mutations and Drugs on the Activity of the Thiamine Transporter, SLC19A2.

A rare cause of megaloblastic anemia (MA) is thiamine-responsive megaloblastic anemia (TRMA), a genetic disorder caused by mutations in SLC19A2 (encoding THTR1), a thiamine transporter. The study objectives were to (1) functionally characterize selected TRMA-associated SLC19A2 variants and (2) determine whether current prescription drugs associated with drug-induced MA (DIMA) may act via inhibition of SLC19A2. Functional characterization of selected SLC19A2 variants was performed by confocal microscopy and isotopic uptake studies of [3H]-thiamine in HEK293 cells. Sixty-three drugs associated with DIMA were screened for SLC19A2 inhibition in isotopic uptake studies. Three previously uncharacterized SLC19A2 variants identified in TRMA patients exhibited disrupted localization to the plasma membrane along with near-complete loss-of-function. Ten of 63 drugs inhibited SLC19A2-mediated thiamine transport ≥ 50% at screening concentrations; however, with the exception of erythromycin, none was predicted to inhibit SLC19A2 at clinically relevant unbound plasma concentrations. Data from electronic health records revealed reduced levels of thiamine pyrophosphate (TPP) in patients prescribed erythromycin, consistent with inhibition of SLC19A2-mediated thiamine transport. Here, we confirmed the role of three SLC19A2 variants in TRMA pathology. Additionally, we report that inhibition of SLC19A2 is a potential, but uncommon mechanism for DIMA.

Association between Vitamin D Supplements, Oxidative Stress Biomarkers, and Hyperbaric Therapy in Patients with Sudden Sensorineural Hearing Loss.

The effect of vitamin D supplementation to patients with sudden sensorineural hearing loss (SSNHL), treated with hyperbaric oxygen (HBO) therapy, on the markers of the oxidant-antioxidant equilibrium was investigated. Patients were divided into two groups: those who did and did not receive vitamin D (cholecalciferol at 4000 IU/24 h). Concentrations of the following compounds, thiobarbituric acid reactive substances (TBARS), malondialdehyde (MDA), conjugated dienes (CD) in plasma and erythrocytes and activities of catalase (CAT), superoxide dismutase (SOD) and glutathione peroxidase (GPx) in erythrocytes, were determined. Haemoglobin (HGB) and haematocrit (HCT) were measured. Blood for analyses was collected from the basilic vein at three time points: before the first HBO procedure, up to 5 min after the first procedure, and after 14 procedures. No statistically significant differences in parameters tested were found between patients who did and did not receive vitamin D. In patients without supplementation, an increase of 53.2% (P ≤ 0.05) in erythrocyte TBARS was observed after the first HBO treatment. In patients receiving vitamin D, a reduction of 27.6% (P ≤ 0.05) was observed in erythrocyte MDA after 14 HBO treatments vs. that after the first treatment. In both groups, a decrease of 33.3% in plasma CD was observed after 14 treatments vs. that after the first treatment (P ≤ 0.05 and P ≤ 0.01, respectively). No statistically significant changes were observed in the erythrocyte SOD, GPx, and CAT activities and in HCT. A reduction of HGB concentration of 10.9% (P ≤ 0.05) was demonstrated in nonsupplemented patients after 14 treatments compared with baseline. The results confirm that the effect of HBO therapy on oxidative stress markers is inconclusive and complex. Repeated HBO procedures can induce adaptive changes which protect against disruption of the oxidant-antioxidant equilibrium. It is possible that vitamin D supplementation inhibits the process of lipid peroxidation in erythrocytes.

Coagulation States in Patients With Sudden Sensorineural Hearing Loss Evaluated by Thromboelastography.

OBJECTIVE: The state of coagulation is controversial in patients with sudden sensorineural hearing loss (SSNHL). We used thromboelastography (TEG) to explore the relationships between blood coagulation parameters and SSNHL pathogenesis and recovery. STUDY DESIGN: Prospective study. SETTING: Affiliated Sixth People's Hospital, Shanghai Jiao Tong University. METHODS: A total of 104 newly diagnosed patients with SSNHL and 29 matched healthy controls were recruited. Hearing assessments, TEG, and conventional coagulation tests (CCTs) were performed, followed by standard treatments and follow-up. RESULTS: The TEG parameters of patients with SSNHL were in the normal range, but the group exhibited a significantly prolonged kinetic time (K; P = .004) and a smaller angle (P = .003) as compared with the controls. After grouping the patients with SSNHL according to audiograms and comparing them in pairs, we found that the differences were significant only when controls were compared with patients with low-frequency SSNHL (K, P = .023; angle, P = .04) and flat-type SSNHL (K, P = .017; angle, P = .014). Logistic regression analysis showed that neither TEG nor CCT parameters significantly affected hearing improvement after SSNHL treatment. CONCLUSIONS: Although the K value and angle were significantly increased and significantly reduced, respectively, in the test group as compared with the control group, the state of coagulation in patients with SSNHL was still within the normal range. No CCT or TEG coagulation parameters (except the angle) differed significantly among patients in each group according to hearing recovery status, which suggested that the coagulation status does not determine the prognosis of patients with SSNHL.

Relapse of rare diseases during COVID-19 pandemic: bicytopenia in an adult patient with thiamine-responsive megaloblastic anaemia.

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 19-year-old man known for TRMA, who presented in the emergency department with bicytopenia (haemoglobin 5,4 g/dL, thrombocytes 38×109/L) revealed by dyspnea and chest pain. Investigations excluded bleeding, hemolysis, coagulopathy and iron deficiencies. A recent infection and an acute coronary syndrome have also been eliminated. We later found out that thiamine treatment had been discontinued three months before, due to general confinement in Tunisia during the COVID-19 pandemic. Parenteral administration of 100 mg of thiamine daily resulted in the recovery of haematopoiesis within three weeks.

Differential Levels of Endoplasmic Reticulum Stress in Peripheral Blood Mononuclear Cells from Patients with Sudden Sensorineural Hearing Loss.

BACKGROUND Sudden sensorineural hearing loss (SSNHL) is currently treated with a combination of drugs, predominantly with glucocorticoids (GCs). However, the mechanisms of action of GCs in SSNHL are unknown. This study aimed to analyze the role of endoplasmic reticulum stress (ERS) in SSNHL pathogenesis and prognosis. MATERIAL AND METHODS In this study, we evaluated the expression and activation status of the protein kinase RNA-like endoplasmic reticulum kinase (PERK)-C/EBP homologous protein (CHOP) pathway in peripheral blood mononuclear cells (PBMCs) from patients with SSNHL and compared them with those in healthy controls. We also compared differences in expression of activating transcription factor 4 (ATF4) and CHOP before and after glucocorticoid treatment in patients with improved and unimproved SSNHL. RESULTS Treatment with GCs significantly improved hearing in 55% of patients with SSNHL. Levels of phosphorylated PERK (p-PERK) and phosphorylated eukaryotic initiation factor 2alpha were increased in PBMCs from patients with SSNHL compared with healthy controls. ATF4 and CHOP expression were also significantly elevated. After treatment, the amount of ATF4 and CHOP proteins in PBMCs in the patients whose SSNHL improved was significantly reduced compared with the levels measured before treatment in all patients with SSNHL. The expression of the ATF4 and CHOP proteins in PBMCs in the unimproved group, however, was not significantly changed relative to pretreatment levels. CONCLUSIONS ERS may play a significant role in the pathogenesis of SSNHL, and the responsiveness of the condition to GC-mediated mitigation of ERS may be one of the key factors that affect patient prognosis.

Circulating microRNAs as potentially new diagnostic biomarkers of idiopathic sudden sensorineural hearing loss.

BACKGROUND: Early detection of inner ear cell damage can reduce the chances of permanent damage to hearing ability. However, current inner ear cell damage detection methods can detect damage only after the patient has lost hearing ability. MicroRNA expression levels in circulating systems are affected in diseases or conditions arising from the distant lesions. Therefore, detection of circulating microRNA expression levels could be one of the best ways to obtain information on inaccessible lesion sites. AIMS/OBJECTIVES: This study aims to establish a method for monitoring idiopathic sudden sensorineural hearing loss (ISSNHL) by analyzing circulating microRNA expression levels. 21 ISSNHL patients and 24 healthy controls were enrolled. MATERIAL AND METHODS: Real-time quantitative polymerase chain reaction was performed for detecting expression levels of circulating microRNAs. RESULTS: Among eight circulating microRNAs, expression levels of five circulating microRNAs significantly differed between ISSNHL patients and healthy controls. circulating microRNA expression levels correlates with treatment outcomes and hearing ability. CONCLUSIONS AND SIGNIFICANCE: Using methods combining the evaluation of miR-183, miR-210, miR-18b, and miR-23a cut-off values identified in ISSNHL patients and healthy controls during receiver operating characteristic curve analysis, sensitivity and specificity of 80.95% (17/21) and 87.50% (21/24) were obtained, respectively.

A family with an MYH9-related disorder with different phenotypes masquerading as immune thrombocytopaenia: an underreported disorder in Taiwan.

A 66-year-old woman had experienced abnormal bleeding since the age of 7. Thrombocytopenia was not detected until she was 48, and immune thrombocytopenia was diagnosed at age 66. She also reported experiencing hearing disturbance since the age of 30 and acute renal failure since the age of 61 but reported no visual disturbance. Her younger son, who was 40 years old, also experienced abnormal bleeding since the age of 6, but immune thrombocytopenia was diagnosed as late as age 35. He had no other associated disorders. Laboratory examinations of both mother and son revealed a low platelet count (8000 and 29,000 µL, respectively), giant platelets and Döhle body-like granulocyte inclusion bodies. The mother had a high creatinine level (15.4 mg/dL) and normal liver enzyme levels. MYH9 genetic analysis identified a heterozygous mutation, c.101T>A, p.Val34Glu at exon 2 in both patients. These clinical and laboratory findings were consistent with a diagnosis of an MYH9-related disorder with different phenotypes observed in the same family. MYH9-related disorders were recognised in 2003, but were often misdiagnosed as immune thrombocytopenia, and hence, they have rarely been reported in Taiwan.

Plasma Serotonin is Elevated in Adult Patients with Sudden Sensorineural Hearing Loss.

BACKGROUND: The roles of thrombophilia and cardiovascular risk factors in sudden sensorineural hearing loss (SSNHL) remain controversial. Cochlear microthrombosis and vasospasm have been hypothesized as possible pathogenic mechanisms of SSNHL. This article investigates the circulating serotonin and homocysteine levels besides thrombophilia screening in patients with idiopathic SSNHL. METHODS: A total of 133 SSNHL patients and age- and sex-matched controls were investigated (discovery cohort). Measurement included common inherited natural coagulation inhibitors, factor VIII, von Willebrand factor (VWF), antiphospholipid antibodies, homocysteine, and serotonin (whole blood, platelet, and plasma) levels, along with frequent relevant genetic variants. A validation cohort (128 SSNHL patients) was studied for homocysteine and serotonin levels. RESULTS AND CONCLUSION: In the discovery cohort, 58.6% of patients exhibited thrombophilia, of which most had a low to moderate titers of antiphospholipid antibodies and high levels of factor VIII/VWF. Twenty-seven patients (20%) had mild-to-moderate hyperhomocysteinemia or were homozygous for the methylenetetrahydrofolate reductase (MTHFR) C677T mutation. Regarding serotonin, SSNHL patients had elevated whole blood levels that remained within the normal range and normal platelet content. However, approximately 90% patients of both cohorts had elevated plasma serotonin. Elevated plasma serotoninemia was accompanied by serotonylation of platelet rhoA protein. This study shows that increased plasma serotonin appears as a biomarker of SSNHL (specificity: ∼96%, sensitivity: ∼90%) and could participate in the pathophysiology of SSNHL.

The Prognostic Value of Circulating Inflammatory Cell Counts in Sudden Sensorineural Hearing Loss and the Effect of Cardiovascular Risk Factors.

OBJECTIVES: Recent studies suggest that elevated neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) are poor prognostic factors in sudden sensorineural hearing loss (SSNHL). We aimed to investigate the accuracy of this hypothesis by taking into account the effect of cardiovascular risk (CVR) factors. METHODS: Medical records of 122 patients with SSNHL were reviewed retrospectively and grouped into 2 as; patients without CVR (group 1; n = 68) and patients having CVR (group 2; n = 54). Moreover, 60 control cases who did not have SSNHL were also included and grouped into 2 as; group 3 (n = 30) with CVR and group 4 (n = 30) healthy controls without having SSNHL or CVRs. Neutrophil (N), lymphocyte (L), platelet (Plt), NLR, and PLR between the groups and their relationship with the severity of hearing loss, recovery rates, and audiogram configurations were analyzed. RESULTS: The highest N and NLR values were in group 1 and were significantly higher than the values of group 4 (P < .05, P < .01). There was no significant relationship between the groups in terms of L, Plt, or PLR values. The highest NLR and PLR values were determined in SSNHL patients with mild hearing loss, complete recovery, and up-sloping audiogram configuration (P > .05). CONCLUSIONS: Elevated levels of N and NLR may be considered as strong laboratory findings showing an inflammatory response in the diagnosis of SSNHL, but the presence of CVR factors does not seem to increase the inflammatory response in SSNHL as expected. In patients with SSNHL, NLR and PLR elevation may indicate better prognosis.

Serum Renin Levels in Sudden Sensorineural Hearing Loss.

OBJECTIVE: The aim of this study was to investigate the serum renin levels of patients with idiopathic sudden sensorineural hearing loss (ISSNHL). MATERIAL AND METHODS: Twenty-four patients with ISSNHL and 24 asymptomatic healthy volunteers were included in the study. Subjects underwent pure-tone audiometry and serum renin levels were measured. RESULTS: There were 14 women (mean age:42.35 ± 9.53) and 10 men (mean age:43.8 ± 6.87) in the patient group. There were 14 women (mean age:42.4 ± 4.7) and 10 men (mean age:41.4 ± 4.59) in the control group. ISSNHL was detected on the right side in 13 patients and on the left side in 11 patients. Serum renin levels of the patients and controls were 788.01 ± 327.8 and 282.37 ± 107.73 pg/mL, respectively. The serum renin levels were found to be significantly higher in the patient group compared to the control group (P ≤ .001). There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss (r = 0.77; P = .001). CONCLUSION: Serum renin levels of patients with ISSNHL were higher than controls. There was a statistically significant strong positive correlation between serum renin level and the severity of hearing loss.

A rare genomic duplication in 2p14 underlies autosomal dominant hearing loss DFNA58.

Here we define a ~200 Kb genomic duplication in 2p14 as the genetic signature that segregates with postlingual progressive sensorineural autosomal dominant hearing loss (HL) in 20 affected individuals from the DFNA58 family, first reported in 2009. The duplication includes two entire genes, PLEK and CNRIP1, and the first exon of PPP3R1 (protein coding), in addition to four uncharacterized long non-coding (lnc) RNA genes and part of a novel protein-coding gene. Quantitative analysis of mRNA expression in blood samples revealed selective overexpression of CNRIP1 and of two lncRNA genes (LOC107985892 and LOC102724389) in all affected members tested, but not in unaffected ones. Qualitative analysis of mRNA expression identified also fusion transcripts involving parts of PPP3R1, CNRIP1 and an intergenic region between PLEK and CNRIP1, in the blood of all carriers of the duplication, but were heterogeneous in nature. By in situ hybridization and immunofluorescence, we showed that Cnrip1, Plek and Ppp3r1 genes are all expressed in the adult mouse cochlea including the spiral ganglion neurons, suggesting changes in expression levels of these genes in the hearing organ could underlie the DFNA58 form of deafness. Our study highlights the value of studying rare genomic events leading to HL, such as copy number variations. Further studies will be required to determine which of these genes, either coding proteins or non-coding RNAs, is or are responsible for DFNA58 HL.

Is the C-reactive protein/albumin ratio a prognostic and predictive factor in sudden hearing loss? / A relação proteína C-reativa/albumina é um fator prognóstico e preditivo na surdez súbita?

Abstract Introduction: Sudden hearing loss is a significant otologic emergency. Previous studies have revealed a coexistence of sudden hearing loss with chronic inflammation. The predictive importance of C-reactive protein/albumin values as a prognostic factor has been shown in various inflammatory and tumoral conditions. Objectives: The aim of this study was to determine whether the C-reactive protein/albumin ratio in sudden hearing loss can be used for prognostic purposes and whether there is a relationship between the neutrophil/lymphocyte ratio and the C-reactive protein/albumin ratio. Methods: A retrospective examination was made of 40 patients diagnosed with idiopathic sudden hearing loss and a control group of 45 healthy subjects. The pure tone averages of all the patients were determined on first presentation and repeated at 3 months after the treatment. The patients were separated into 2 groups according to the response to treatment. The neutrophil/lynphocyte ratio and the C-reactive protein/albumin ratios were calculated from the laboratory tests. Results: The patients included 16 females and 24 males with a mean age of 44.1 ± 14.2 years and the control group was composed of 23 females and 22 males with a mean age of 42.2 ± 13.8 years. The mean C-reactive protein/albumin ratio was 0.95 ± 0.47 in the patient group and 0.74 ± 0.13 in the control group. The difference was statistically significant (p = 0.009). The mean C-reactive protein/albumin ratio was 0.79 ± 0.12 in the response to treatment group and 1.27 ± 0.72 in the non-response group, with no significant difference determined between the groups (p = 0.418). The mean neutrophil/lymphocyte ratio was 3.52 ± 3.00 in the response to treatment group and 4.90 ± 4.60 in the non-response group, with no statistically significant difference determined between the groups (p = 0.261). Conclusion: C-reactive/albumin ratio was significantly higher in patients with sudden hearing loss than in the control group. Although C-reactive protein/albumin ratio was found to be lower in sudden hearing loss patients who responded to treatment compared to those who did not, the difference between two groups was not statistically significant.

Resumo Introdução: A perda auditiva neurossensorial súbita ou surdez súbita é uma emergência otológica significativa. Estudos anteriores revelaram uma coexistência dessa condição com inflamação crônica. A importância preditiva dos valores da relação proteína C-reativa/albumina como fator prognóstico tem sido demonstrada em várias condições inflamatórias e tumorais. Objetivos: O objetivo deste estudo foi determinar se a relação proteína C-reativa/albumina na perda auditiva neurossensorial súbita pode ser usada para fins prognósticos e se existe uma associação entre as relações neutrófilo/linfócito e proteína C-reativa/albumina. Método: Foram avaliados retrospectivamente 40 pacientes com diagnóstico de perda auditiva neurossensorial súbita idiopática e um grupo controle de 45 indivíduos saudáveis. As médias de tons puros de todos os pacientes foram determinadas na primeira consulta e repetidas 3 meses após o tratamento. Os pacientes foram separados em 2 grupos de acordo com a resposta ao tratamento. As relações neutrófilo/linfócito e proteína C-reativa/albumina foram calculadas a partir de testes laboratoriais. Resultados: Os pacientes incluíam 16 mulheres e 24 homens, com média de 44,1 ± 14,2 anos, e o grupo controle por 23 mulheres e 22 homens, com média de 42,2 ± 13,8 anos. A média da relação proteína C-reativa/albumina foi de 0,95 ± 0,47 no grupo de pacientes e de 0,74 ± 0,13 no grupo controle e a diferença foi estatisticamente significante (p = 0,009). A média da relação proteína C-reativa/albumina foi de 0,79 ± 0,12 do grupo com resposta ao tratamento e de 1,27 ± 0,72 no grupo sem resposta, sem diferença significante entre os grupos (p = 0,418). A média da relação neutrófilo/linfócito foi de 3,52 ± 3,00 no grupo com resposta ao tratamento e de 4,90 ± 4,60 no grupo sem resposta, sem diferença estatisticamente significativa entre os grupos (p = 0,261). Conclusão: A relação proteína C-reativa/albumina foi significantemente maior nos pacientes com perda auditiva neurossensorial súbita do que no grupo controle. No entanto, embora a relação proteína C-reativa/albumina tenha sido menor nos pacientes com perda auditiva neurossensorial súbita que responderam ao tratamento em comparação a aqueles que não apresentaram resposta, a diferença entre os dois grupos não foi estatisticamente significante.

Can miR-34a be suitable for monitoring sensorineural hearing loss in patients with mitochondrial disease? A case series.

Purpose: We aimed at evaluating the feasibility of using MicroRNA (miR)-34a and miR-29b to detect inner ear damage in patients with mitochondrial disease (MD) and sensorineural hearing loss (SNHL).Material and Methods: Three patients with MD and SNHL and seven healthy control subjects were included in this case series. MD patients underwent pure tone audiometry (PTA), distortion product otoacoustic emission (DPOAE) and auditory brain response tests to investigate the specific cochlear and retrocochlear functions; control patients underwent PTA. MiR-34a and miR-29b were extracted from blood in all subjects included in the study. The expression of miR-34a and miR-29b in MD patients and healthy controls were statistically compared, then the expression of these two miRs was compared with DPOAE values.Results: In MD patients, miR-34a was significantly up-regulated compared to healthy controls; miR-34a and DPOAEs were negatively correlated. Conversely, miR-29b was up-regulated only in the youngest patient who suffered from the mildest forms of MD and SNHL, and negatively correlated with DPOAEs.Conclusion: In MD patients, miR-34a and miR-29b might be a marker of inner ear damage and early damage, respectively. Additional studies on larger samples are necessary to confirm these preliminary results.

Serum non-high-density lipoprotein cholesterol is associated with the risk of sudden sensorineural hearing loss.

The aim of this study was to investigate the association between the non-high-density lipoprotein cholesterol (non-HDL-C) with sudden sensorineural hearing loss (SSHL) and the predictive value of non-HDL-C for SSHL.A total of 324 patients with SSHL and 972 well-matched controls were enrolled from 2009 to 2012 in Korea. The association of serum non-HDL-C with the risk of SSHL was evaluated using multivariate regression analysis, smooth curve fitting after adjusting for potential confounders. The discrimination ability of non-HDL-C in predicting SSHL was determined by calculating the area under the curve (AUC), and its clinical usefulness was evaluated by decision curve analysis. This was a secondary analysis of a case-control study.There was a non-linear relationship between the serum non-HDL-C and the incidence of SSHL. After adjustment for potential confounders, the incidence of SSHL rose significantly with ascending quartiles of serum non-HDL-C (using Q1 as the reference group, the OR [95% CI] of Q2, Q3, and Q4 were 4.34 [2.43-7.74], 7.08 [3.99-12.56], and 20.88 [11.86-36.75], respectively [P for trend <.0001]). The discrimination ability of serum non-HDL-C in predicting SSHL was 0.747 (95% CI, 0.717-0.776), and the AUC was 0.733 (95% CI, 0.705-0.777) in the internal validation.Elevated serum non-HDL-C was strongly associated with increased risk of SSHL, and it may play a role as a useful biomarker in predicting the risk of SSHL.