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Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and mo...
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Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and most of these have been mapped. Here, we report a Chinese family affected by a congenital profound sensorineural hearing loss. All phenotypes of this family are clinically compatible with non-syndromic sensorineural deafness (DFN2). A maximum two-point Lod score of 2.32 was obtained at marker DXS6797 (6= 0.00). Recombinants define a region of 4.3 cm flanked by markers DXS6799 and GATA172D05, This region overlaps the previously reported DFN2 region by 2.0 cm.
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摘要 :
Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and mo...
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Non-syndromic X-linked deafness is a rare form of genetic deafness in humans accounting for a small proportion of all hereditary hearing loss. Different clinical forms of non-syndromic X-linked deafness have been described, and most of these have been mapped. Here, we report a Chinese family affected by a congenital profound sensorineural hearing loss. All phenotypes of this family are clinically compatible with non-syndromic sensorineural deafness (DFN2). A maximum two-point Lod score of 2.32 was obtained at markerDXS6797 (θ = 0.00). Recombinants define a region of 4.3 cm flanked by markersDXS6799 andGATA172D05. This region overlaps the previously reported DFN2 region by 2.0 cm.
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Based on the clinical characteristics of patients, a nomogram predicting the prognosis of patients suffering from sudden sensorineural hearing loss (SSNHL) was constructed, which could aid in personalized treatment. Methods: Data ...
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Based on the clinical characteristics of patients, a nomogram predicting the prognosis of patients suffering from sudden sensorineural hearing loss (SSNHL) was constructed, which could aid in personalized treatment. Methods: Data on the clinical characteristics of patients with SSNHL were collected and statistically analyzed. A nomogram for predicting the hearing prognosis of SSNHL patients were then constructed. Results: A total of 356 patients were included in this study, including 227 and 129 in the recovery group (63.76%) and non-recovery group (36.24%), respectively. Univariable logistic regression demonstrated that age, gender, body mass index (BMI), marital, Audiogram curve, vertigo, hearing loss degree, and time to initial treatment were associated with hearing outcomes. Multivariate logistic models showed that age [odds ratio (OR): 0.479, 95% confidence interval (CI): 0.301–0.748, P<0.001], descending (OR: 0.116, 95% CI: 0.047–0.275, P<0.001) and flat audiogram curves (OR: 0.397, 95% CI: 0.159–0.979, P=0.045), profound hearing loss (OR: 0.047, 95% CI: 0.013–0.152, P<0.001), and treatment initiation after 1 week (8–14 days: OR: 0.047, 95% CI: 0.013–0.152, P 14 days: OR: 0.131, 95% CI: 0.039–0.413) were risk factors for the hearing recovery. Logistic regression analyses were conducted to construct the prognostic nomogram. As estimated by the area under the receiver operating characteristic curve (ROC), the model had an accuracy of 0.867 (95% CI: 0.709–0.747). The validation analysis confirmed the high accuracy of the nomogram, and the decision curve showed that the model has potential clinical application value. Conclusions: This study demonstrated that age, descending and flat audiogram curves, profound hearing loss, and initiating treatment after 1 week of SSNHL onset were independent risk factors associated with a worse hearing recovery prognosis. Using these factors, a nomogram with a high prediction accuracy was developed to predict the hearing recovery rate of SSNHL patients.
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Objective: This study aims to determine whether the risks of cardiocerebrovascular disease are relevant to sensorineural hearing loss (SNHL) based on a national database.Methods: A total of 1,321 participants aged from 18 to 69 wi...
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Objective: This study aims to determine whether the risks of cardiocerebrovascular disease are relevant to sensorineural hearing loss (SNHL) based on a national database.Methods: A total of 1,321 participants aged from 18 to 69 with complete data including medical history and audiometry from the NHANES database (2015–2018) were analyzed. All included participants had available hearing data and the average thresholds of the hearing data were measured and calculated as low-frequency pure-tone average (LFPTA; 500, 1,000, and 2,000 Hz) and high-frequency pure-tone average (HFPTA; 3,000, 4,000, 6,000, and 8,000 kHz). SNHL was defined as an average pure tone of more than or equal to 20 dB in at least one better ear. Multivariable models to assess the association between cardiocerebrovascular risks and SNHL were used in this study.Results: The prevalence of stroke was 1.6% in individuals with SNHL and 0.4% in individuals without SNHL (p = 0.023). A higher cardiovascular risk score was observed in SNHL patients compared to participants without SNHL (1.58 vs. 0.90, p < 0.001). Stroke was associated with a 3.67-fold increase in the risk of SNHL (95% CI: 1.12–12.00, p = 0.032) in univariable logistic regression, and the association (OR = 4.22, 95%CI = 1.28–13.93, p = 0.020) remained significant after adjusting for several covariates. Multivariable logistic regression models indicated a positive correlation between cardiovascular risk and SNHL (OR = 1.66, 95% CI = 1.40–1.96, p < 0.001), but no significant relationship was shown with all covariates adjusted. However, significant associations were found between SNHL and both age and sex in both univariable and multivariable logistic regression models.Conclusion: Our findings suggested that a higher cardiocerebrovascular risk burden was associated with an increased risk of SNHL, and the relationship may be influenced by age and sex. Future longitudinal studies are needed to investigate the mechanistic and pathologic vascular hypothesis of SNHL.
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Objective To investigate the clinical features and factors affecting the prognosis of children with profound sudden sensorineural hearing loss (SSNHL). Methods We retrospectively analyzed the clinical data of 147 children with pro...
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Objective To investigate the clinical features and factors affecting the prognosis of children with profound sudden sensorineural hearing loss (SSNHL). Methods We retrospectively analyzed the clinical data of 147 children with profound SSNHL who received inpatient treatment at our department from January 2016 to January 2021. All children were administered with systemic steroid therapy and/or intratympanic steroid (ITS) treatment for 2 weeks. Statistical analyses were performed for the clinical features, treatment effectiveness, and factors affecting the prognosis using SPSS 23.0. Results The median age of the study population was 8 (6–10) years. The median treatment onset time was 8 (4–20) days. The most common concomitant symptom was tinnitus (45.58%). Laboratory findings showed that the percentages of children with abnormal leukocytes was 25.85%, abnormal platelet counts was 17.01%, abnormal cytomegalovirus IgG antibodies was 36.73% and abnormal Epstein–Barr (EB) virus IgG antibodies was 41.50%. The overall recovery rate of the treatment was 20.04%. The univariate analysis showed that age, treatment onset time, tinnitus, and ITS treatment were associated with the prognosis ( p ?<?0.05). Regarding laboratory findings, the neutrophil count, lymphocyte count, and neutrophil-to-lymphocyte ratio differed significantly between the effective and invalid treatment effect groups ( p ?<?0.05). The multivariable logistic regression analysis showed that treatment onset time [odds ratio (OR)?=?0.936, 95% confidence interval (CI): 0.881–0.994] and ITS treatment (OR?=?0.174, 95% CI: 0.044–0.0687) correlated with hearing recovery ( p ?<?0.05). Conclusion In this study, the earlier the treatment start time of children with profound SSNHL, the better was the prognosis. Further, ITS could be an effective treatment option.
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Sudden sensorineural hearing loss (SSNHL) is most often defined as a rapid hearing loss of ≥ 30 decibels across at least three contiguous audiometric frequencies over a time of ≤ 72 hours. Cupping therapy has been practiced acro...
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Sudden sensorineural hearing loss (SSNHL) is most often defined as a rapid hearing loss of ≥ 30 decibels across at least three contiguous audiometric frequencies over a time of ≤ 72 hours. Cupping therapy has been practiced across the world for thousands of years. Cupping therapy is practiced by creating suction inside cups that are placed on predefined skin areas. Our case is a 48-year-old female with a four-year history of Meniere’s disease, recurrent tinnitus, episodes of dizziness attacks, and fullness of the right ear. The patient developed sudden sensorineural hearing loss. She received conventional treatment and wet cupping therapy as a complementary integrative treatment. After the integrative management protocol was completed, pure tone audiometry tests revealed significant hearing improvement across almost all frequencies. To the best of our knowledge, this case presentation is the first reported case of this type. A positive effect of cupping was reported in our case as an integrative complementary treatment. Large, well-designed quality clinical trials to evaluate the efficacy and safety of wet cupping therapy (WCT) as a complementary treatment of SSNHL is highly recommended.
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Background: Congenital cytomegalovirus (CMV) infection affects nearly 1% of live births in the United States. Ten percent of these infants have symptoms at birth and another 10 to 15% develop hearing loss or developmental problems...
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Background: Congenital cytomegalovirus (CMV) infection affects nearly 1% of live births in the United States. Ten percent of these infants have symptoms at birth and another 10 to 15% develop hearing loss or developmental problems. The aim of this study was to compare CMV infection (IgM and IgG) rate in infants suspected for intrauterine infectionwith the control group. Patients and Methods: A case-control study was performed in the Pediatrics Department of Hazrat Rasool Akram Hospital in Tehran. The study population included 74 suspected cases of intrauterine infection (mean age, 4.73.7 months) and 65 normal healthy controls (mean age, 5.33.1 months). We compared serum CMV antibodies (IgM, IgG) with ELISA kits. Results: Acute and previous immunity to CMV (IgM and IgG) was found in 41.9% (31/74) and 74% (54/74) of cases, respectively. These figures were 6.2% (4/65) and 95.4% (62/65) in controls, respectively. Acute infection (CMV-IgM) was more common among cases (p<0.0001), but previous immunity (CMV-IgG) was more prevalent among controls (p<0.001). Conclusion: We concluded that CMV is the most common cause of intrauterine infection in infants aged less than 6 months as compared to the healthy ones. We prefer, at least in our country, to consider seropositive (CMV-IgM) infants suspected of intrauterine infection (less than 6 months) as congenital form. To arrest the natural progression of congenital CMV, we recommend prolonged course of oral analogues of ganciclovir for children with symptomatic congenital CMV.
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BackgroundHyperlipidemia may be part of the important mechanisms for the development of idiopathic sudden sensorineural hearing loss (ISSNHL).AimsSo the purpose of this study was to evaluate the relationship between changes in blo...
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BackgroundHyperlipidemia may be part of the important mechanisms for the development of idiopathic sudden sensorineural hearing loss (ISSNHL).AimsSo the purpose of this study was to evaluate the relationship between changes in blood lipid levels and ISSNHL.Materials and MethodsWe enrolled 90 ISSNHL patients in our hospital using a retrospective study design from 2019.1 to 2021.12. Blood levels of total cholesterol (TC), triglycerides (TG), and low-density lipoprotein (LDL-C). Hearing recovery was analyzed using the chi-square test and one-way analysis of variance (ANONA). Univariate and multifactorial Logstic retrospective analyses to establish the relationship between LDL-C/HDL-C ratio and hearing recovery after adjustment for potential confounding factors.ResultsIn our study, 65 (72.2%) patients had their hearing recovered. All group analyses and three group analyses (i.e. Excluding the no-recovery group) found that LDL/HDL was on an upward trend from complete recovery to a slight recovery group and strongly associated with hearing recovery. Univariate and multivariate logistic regression analysis found high levels of LDL and LDL/HDL in the partial hearing recovery group, relative to the full hearing recovery group. Curve fitting intuitively demonstrates the influence of blood lipids on prognosis.ConclusionsOur findings suggest that LDL. TC, TC/HDL, and LDL/HDL concentrations may be closely related to the pathogenesis of ISSNHL.SignificanceImproving the relevant lipid test at the time of admission to the hospital has good clinical significance for improving the prognosis of ISSNHL.
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Objective: Limited research has focused on the clinical features of sudden sensorineural hearing loss (SSNHL) in pediatric patients. This study is aimed to investigate the relationship between clinical features and the baseline he...
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Objective: Limited research has focused on the clinical features of sudden sensorineural hearing loss (SSNHL) in pediatric patients. This study is aimed to investigate the relationship between clinical features and the baseline hearing severity and outcomes of SSNHL in the pediatric population.Method: We conducted a bi-center retrospective observational study in 145 SSNHL patients aged no more than 18 years who were recruited between November 2013 and October 2022. Data extracted from medical records, audiograms, complete blood count (CBC) and coagulation tests have been assessed for the relationship with the severity (the thresholds of the initial hearing) and outcomes (recovery rate, hearing gain and the thresholds of the final hearing).Results: A lower lymphocyte count (P = 0.004) and a higher platelet-to-lymphocyte ratio (PLR) (P = 0.041) were found in the patient group with profound initial hearing than in the less severe group. Vertigo (β = 13.932, 95%CI: 4.082–23.782, P = 0.007) and lymphocyte count (β = ?6.686, 95%CI: ?10.919 to ?2.454, P = 0.003) showed significant associations with the threshold of the initial hearing. In the multivariate logistic model, the probability of recovery was higher for patients with ascending and flat audiograms compared to those with descending audiograms (ascending: OR 8.168, 95% CI 1.450–70.143, P = 0.029; flat: OR 3.966, 95% CI 1.341–12.651, P = 0.015). Patients with tinnitus had a 3.2-fold increase in the probability of recovery (OR 3.222, 95% CI 1.241–8.907, P = 0.019), while the baseline hearing threshold (OR 0.968, 95% CI 0.936–0.998, P = 0.047) and duration to the onset of therapy (OR 0.942, 95% CI 0.890–0.977, P = 0.010) were negatively associated with the odds of recovery.Conclusions: The present study showed that accompanying tinnitus, the severity of initial hearing loss, the time elapse and the audiogram configuration might be related to the prognosis of pediatric SSNHL. Meanwhile, the presence of vertigo, lower lymphocytes and higher PLR were associated with worse severity.
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