The etiology of sudden deafness or idiopathic sudden sensorineural hearing loss(ISSHL) remains unclear. Over the past 15 years, we have investigated the mechanisms of ischemic-induced hearing loss using a gerbil model...The etiology of sudden deafness or idiopathic sudden sensorineural hearing loss(ISSHL) remains unclear. Over the past 15 years, we have investigated the mechanisms of ischemic-induced hearing loss using a gerbil model of transient cochlear ischemia. In the gerbil, cochlear ischemia can be induced by occluding the bilateral vertebral arteries simultaneously at the neck, because the posterior communicating arteries of the Circle of Willis close spontaneously around 1 mo after birth. When 15 min ischemia was loaded on this animal, permanent hearing loss of about 25 d B and the death of hair cells, especially inner hair cells were induced. These pathological changes were mainly due to lack of an energy source, glutamate excitotoxicity, and the production of free radicals, especially superoxide and nitrous oxide species. Ischemic damage could be prevented by various procedures, such as cooling the cochlea, intratympanic administration of insulin-like growth factor 1 or AM-111(an anti-apoptotic agent), and systemic administration of prednisolone(steroid), edarabone(free radical scavenger), ginsenoside Rb1(Kanpo), hematopoietic stem cells, glia-cell derived neurotrophic factor, and liposome-encapsulated hemoglobin(artificial red blood cells). We also found that the cochlea was protected by the ischemic tolerance, indi-cating that minor cochlear ischemia alleviates or prevents inner ear damage in subsequent severe cochlear ischemia. As ISSHL usually occurs suddenly, with no preceding sign or symptom, we suggest that most ISSHL cases are caused by circulatory disturbance, probably at the stria vascularis.展开更多
X-linked deafness is a rare genetic disorder causing a severe mixed hearing loss. This is due to an abnormal connection between the internal auditory meatus(IAM) and the basal turn of the cochlear leading to a "3...X-linked deafness is a rare genetic disorder causing a severe mixed hearing loss. This is due to an abnormal connection between the internal auditory meatus(IAM) and the basal turn of the cochlear leading to a "3rd window effect" and cochlear conductive hearing loss. Patients are traditionally treated with conventionalhearing aids however these are often unsatisfactory. Cochlear implantation is a high-risk procedure in such cases due to the risk of inadvertent electrode placement in the IAM. We present three paediatric cases where the hearing loss was managed with a combination of a bone anchored hearing aid in combination with a conventional behind the ear hearing aid. We also present a review of the current literature regarding the management of X-linked deafness.展开更多
Objective: In order to provide a theoretical basis for the revision of the current diagnostic criteria for occupational noise-induced deafness (ONID), we evaluated the degree of ONID by analyzing different high-freque...Objective: In order to provide a theoretical basis for the revision of the current diagnostic criteria for occupational noise-induced deafness (ONID), we evaluated the degree of ONID by analyzing different high-frequency-hearing- threshold-weighted values (HFTWVs). Methods: A retrospective study was conducted to evaluate the diagnosis of patients with ONID from January 2016 to January 2017 in Guangdong province, China. Based on 3 hearing tests (each interval between the tests was greater than 3 days), the minimum threshold value of each frequency was obtained using the 2007 edition’s diagnostic criteria for ONID. The speech frequency and the HFTWVs were analyzed based on age, noise exposure, and diagnostic classi-fication using SPSS21.0. Results: 168 patients in total were involved in this study, 154 males and 14 females, and the average age was 41.18 ± 6.07. The diagnosis rate was increased by the weighted value of the high frequencies and was more than the mean value of the pure speech frequency (MVPSF). The diagnosis rate for the weighted 4 kHz frequency level increased by 13.69% (χ2 = 9.880, P = 0.002), the weighted 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002), and the weighted 4 kHz + 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002). The differences were all statistically significant. The diagnostic rate of the different thresholds showed no obvious difference between the genders. The age groups were divided into less than or equal to 40 years old (group A) and 40 - 50 years old (group B). There were several groups with a high frequency: high frequency weighted 4 kHz ( group A χ2 = 3.380, P = 0.050;group B χ2 = 4.054, P = 0.032), high frequency weighted 6 kHz (group A χ2 = 6.362, P = 0.012;group B χ2 = 4.054, P = 0.032), weighted 4 kHz + 6 kHz (group A χ2 = 6.362 P = 0.012;B χ2 = 4.054, P = 0.032) than those of MVPSF in the same group on ONID diagnosis rate. The differences between the groups were statistically significant. There was no significant difference between the age groups (χ2 = 2.265, P = 0.944). The better ear’s (the smaller hearing threshold weighted value) MVPSF and the weighted values for the different high frequencies were examined in light of the number of working years;the group that was exposed to noise for more than 10 years had significantly higher values than those of the average thresholds of each frequency band in the groups with 3 - 5 years of exposure (F = 2.271, P = 0.001) and 6 - 10 years of exposure (F = 1.563, P = 0.046). The differences were statistically significant. The different HFTWVs were higher than those of the MVPSF values, and the high frequency weighted 4 kHz + 6 kHz level showed the greatest difference, with an average increase of 2.83 dB. The diagnostic rate that included the weighted high frequency values was higher for the mild, moderate, and severe cases than those patients who were only screened with the pure frequency tests. The results of the comparisons of the diagnosis rates for mild ONID were as follows: the weighted 3 kHz high frequency level (χ2 = 3.117, P = 0.077) had no significant difference, but the weighted 4 kHz level (χ2 = 10.835, P = 0.001), 6 kHz level (χ2 = 9.985, P = 0.002), 3 kHz + 4 kHz level (χ2 = 6.315, P = 0.012), 3 kHz + 6 kHz level (χ2 = 6.315, P = 0.012), 4 kHz + 6 kHz level (χ2 = 9.985, P = 0.002), and 3 kHz + 4 kHz + 6 kHz level (χ2 = 7.667, P = 0.002) were significantly higher than the diagnosis rate of the mean value of the PSF. There was no significant difference between the 2 groups for the moderate and severe grades (P > 0.05). Conclusion: Different HFTWVs increase the diagnostic rate of ONID. The weighted 4 kHz, 6 kHz, and 4 kHz + 6 kHz high frequency values greatly affected the diagnostic results, and the weighted 4 kHz + 6 kHz high frequency hearing threshold value has the maximum the effect on the ONID diagnosis results.展开更多
BACKGROUND Managing sudden deafness(SD)in patients with diabetes mellitus(DM)is partic-ularly challenging due to the heightened risk of adverse effects associated with systemic drug administration.This study explores ...BACKGROUND Managing sudden deafness(SD)in patients with diabetes mellitus(DM)is partic-ularly challenging due to the heightened risk of adverse effects associated with systemic drug administration.This study explores the potential of retroauricular subperiosteal injection as a localized drug delivery method for a more effective and safe treatment.AIM To compare the efficacy of retroauricular subperiosteal injection vs systemic intravenous glucocorticoid(GC)administration for SD in patients with DM and assess the effects on blood glucose levels.METHODS A total of 128 cases of type 2 DM(T2DM)with SD diagnosed and treated in Zibo Central Hospital from February 2021 to July 2023 were divided into two groups:An observation group(66 cases receiving retroauricular subperiosteal injection of methylprednisolone)and a control group(62 cases receiving systemic intravenous administration of methylprednisolone).The two groups were compared in terms of therapeutic efficacy,hearing recovery,blood glucose level changes,and incidence of adverse reactions.Binary logistic regression was used to analyze the factors affecting therapeutic efficacy.RESULTS The observation group showed a significantly higher total effective rate(90.91%)compared with the control group(75.81%,P<0.05).Additionally,pure-tone hearing threshold,fasting plasma glucose,and 2-hour postprandial blood glucose were significantly lower in the observation group compared with the control group(P<0.05).The incidence of adverse reactions was also lower in the observation group than in the control group(7.58%vs 22.58%,P<0.05).A T2DM course longer than 5 years and systemic intravenous GC administration were identified as independent risk factors for treatment inefficacy(P<0.05).INTRODUCTION Sudden deafness(SD)is a clinical emergency characterized by rapid-onset hearing loss that is often accompanied by clinical symptoms such as tinnitus and vertigo[1].Although its pathogenesis remains unclear,it is supposedly associated with factors,including inner ear microcirculation disorders,autoimmune diseases,and viral infections[2,3].Patients with diabetes are particularly susceptible to microvascular complications due to poor long-term glycemic control,affecting the ear’s microcirculation,subsequently increasing the risk of SD[4].Type 2 diabetes mellitus(T2DM),a chronic metabolic disease,causes multiple microvascular damages throughout the body,complicating SD treatment in patients with diabetes[5,6].Inner ear microcirculation disturbances in patients with diabetes may exacerbate the risk of SD,and its pathological process may be related to vascular endothelial dysfunction,inflammatory reactions,and hemorrhological changes caused by diabetes mellitus(DM)[7].Current SD treatments include glucocorticoids(GCs),vasodilators,and hyperbaric oxygen therapy[8].GCs are widely used due to their anti-inflammatory and immunosuppressive effects[9].However,systemic GCs may cause blood glucose(BG)fluctuations and even increase the risk of complications in patients with DM,limiting their clinical use in this population[10].Therefore,there is a compelling and immediate need for a local alternative solution that minimally affects the metabolic mechanisms.In recent years,retroauricular subperiosteal injection has emerged as a localized administration modality for treating SD[11].This method allows drugs to directly act on the inner ear,avoiding the side effects of systemic administration and having a minor impact on BG levels,providing a potentially effective treatment for patients with diabetes[12].However,there is limited clinical evidence to compare the efficacy and glycaemic effects of retroauricular subperiosteal injection vs systemic intravenous GC administration in patients with SD and diabetes.This study aimed to explore the efficacy of retroauricular subperiosteal injection and systemic intravenous GC administration for treating patients with SD and DM and their effects on BG,providing a safer and more effective clinical treatment approach.展开更多
Objective:To investigate the expressions of microRNA-155 (miR-155) and histone deacetylase 2 (HDAC2) in peripheral blood mononuclear cells (PBMC) of patients with sudden deafness (SSNHL) and their clinical significanc...Objective:To investigate the expressions of microRNA-155 (miR-155) and histone deacetylase 2 (HDAC2) in peripheral blood mononuclear cells (PBMC) of patients with sudden deafness (SSNHL) and their clinical significances.Methods:112 patients with SSNHL who were treated in our hospital from February 2017 to September 2018 were selected as the study subjects, and they were referred to as sudden deafness group, and another 115 healthy examinees in the same period were compared and studied as normal group. The relative expression levels of miR-155, HDAC2, ICAM-1, TNF-α and interleukin-8 (IL-8) in PBMC of all subjects were detected by real-time fluorescence quantitative analysis (qRT-PCR). Pearson method was used to analyze the relationships between miR-155, HDAC2 and the expressions of ICAM-1, TNF-α, IL-8, and miR-155 and the expression of HDAC2 in PBMC of SSNHL patients. Logistic regression analysis was used to analyze the risk factors of SSNHL. The predictive diagnostic values of miR-155 and HDAC2 in PBMC for SSNHL were evaluated. Results:The relative expression levels of miR-155, ICAM-1, TNF-α and IL-8 in PBMC of sudden deafness group were significantly higher than those of normal group (P<0.05), while the relative expression levels of HDAC2 were significantly lower than those of normal group (P<0.05). In SSNHL patients, miR-155 were positively correlated with the expression levels of ICAM-1, TNF-α and IL-8 (P<0.05). The expression level of HDAC2 was negatively correlated with the expression levels of TNF-α and IL-8 (P<0.05). The expression level of miR-155 in PBMC of SSNHL patients was negatively correlated with HDAC2 (P<0.05). MiR-155, ICAM-1, TNF-α and IL-8 were risk factors for SSNHL (P<0.05), while HDAC2 was protective factor for SSNHL (P<0.05). The areas under curve (AUC) of miR-155 and HDAC2 in PBMC for SSNHL diagnosis were 0.855 and 0.835 respectively, the truncation values of which were 1.449 and 0.959 respectively, at this moment, the sensitivities were 75.0% and 81.3% respectively, and the corresponding specificities were 86.1% and 70.1% respectively. The AUC of combined diagnosis of SSNHL was 0.927, and the sensitivity and specificity were 86.7% and 85.3% respectively.Conclusions:The expressions of miR-155 is high and HDAC2 is low in PBMC of SSNHL patients, which are negatively correlated, and both of them may participate in the occurrence and development of SSNHL through mutual influence, which is a risk factor of SSNHL. The combination of the two can effectively improve the predictive diagnostic value of SSNHL.展开更多
BACKGROUND Wolfram syndrome is a rare autosomal recessive genetic disorder characterized by early-onset diabetes and progressive neurodegeneration,most notably sensorineural hearing loss and optic atrophy.Because its ...BACKGROUND Wolfram syndrome is a rare autosomal recessive genetic disorder characterized by early-onset diabetes and progressive neurodegeneration,most notably sensorineural hearing loss and optic atrophy.Because its initial manifestations are usually similar to those of type 1 diabetes,the diagnosis may be delayed until other manifestations appear.Pathogenic variations of the WFS1 gene can disrupt endoplasmic reticulum function and cellular homeostasis,but the complete mutation spectrum of WFS1 has not been fully determined.Early identification of monogenic diabetes caused by Wolfram syndrome is of vital importance,as it enables the provision of targeted multidisciplinary care and genetic counseling for affected families.CASE SUMMARY A 2-year-old Han Chinese girl was admitted with a 1-month history of polydipsia,polyuria,and polyphagia,and was diagnosed with diabetic ketoacidosis and impaired insulin secretion.Sensorineural hearing loss was also detected.Wholeexome sequencing identified a previously unreported heterozygous mutation,WFS1 c.986T>C(p.Phe329Ser),in the patient and her father,confirming the diagnosis of Wolfram syndrome.Bioinformatic analysis supported the likely pathogenicity of this mutation.In silico pathogenicity predictors(REVEL,SIFT,Poly-Phen-2,MutationTaster,and GERP+)supported a deleterious effect on wolframin structure and function.The patient was initially treated with intravenous insulin and fluid resuscitation,then transitioned to a basal–bolus insulin regimen.Glycemic control was subsequently maintained with continuous subcutaneous insulin infusion and intermittent subcutaneous injections.At the 1-and 6-month follow-ups,blood glucose remained well controlled(hemoglobin A1c:5.89%and 6.58%,respectively),with no evidence of organ dysfunction or further complications.CONCLUSION This case identifies WFS1 c.986T>C(p.Phe329Ser)as a novel pathogenic variant causing Wolfram syndrome.It highlights the importance of early genetic testing in pediatric patients with atypical diabetes presentations to enable timely diagnosis,individualized therapy,and comprehensive family support.展开更多
100 cases of nerve deafness were treated with Injectio Radix Salviae Miltiorrhizae(丹参注射注)by i.v.drip,and additional drugs that promoted blood circulation were used accordingto symptom differentiation.Results were...100 cases of nerve deafness were treated with Injectio Radix Salviae Miltiorrhizae(丹参注射注)by i.v.drip,and additional drugs that promoted blood circulation were used accordingto symptom differentiation.Results were 28 cases cured,45 cases improved,and 27 casesfailed,for a total effective rate of 73%.展开更多
In this paper, the auricular reaction spots of 40 affected children with ner-vous and drug-induced deafness, and 20 healthy children were observed. The result of the re-gular distribution of these spots, which is very...In this paper, the auricular reaction spots of 40 affected children with ner-vous and drug-induced deafness, and 20 healthy children were observed. The result of the re-gular distribution of these spots, which is very significantly different between deafness group andcontrol group, suggests that otopoints Kidney, Brain and Ear where the reaction spots concen-trate play a role in diagnosis, treatment and prevention of child’s deafness.展开更多
Profound congenital sensorineural hearing loss(SNHL) is not so infrequent,affecting 1 to 2 of every 1000 newborns in western countries.Nevertheless,universal hearing screening programs have not been widely applied,alt...Profound congenital sensorineural hearing loss(SNHL) is not so infrequent,affecting 1 to 2 of every 1000 newborns in western countries.Nevertheless,universal hearing screening programs have not been widely applied,although such programs are already established for metabolic diseases.The acquisition of spoken language is a time-dependent process,and some form linguistic input should be present before the first 6 mo of life for a child to become linguistically competent.Therefore,profoundly deaf children should be detected early,and referred timely for the process of auditory rehabilitation to be initiated.Hearing assessment methods should reflect the behavioural audiogram in an accurate manner.Additional disabilities also need to be taken into account.Profound congenital SNHL is managed by a multidisciplinary team.Affected infants should be bilaterally fitted with hearing aids,no later than 3 mo after birth.They should be monitored until the first year of age.If they are not progressing linguistically,cochlear implantation can be considered after thorough preoperative assessment.Prelingually deaf children develop significant speech perception and production abilities,and speech intelligibility over time,following cochlear implantation.Age at intervention and oral communication,are the most important determi-nants of outcomes.Realistic parental expectations are also essential.Cochlear implant programs deserve the strong support of community members,professional bodies,and political authorities in order to be successful,and maximize the future earnings of pediatric cochlear implantation for human societies.展开更多
Objective To compare the difference in the clinical efficacy on the different frequency hearing damages for the patients with sudden deafness between the comprehensive therapy of electroacupuncture and western medicin...Objective To compare the difference in the clinical efficacy on the different frequency hearing damages for the patients with sudden deafness between the comprehensive therapy of electroacupuncture and western medicine and the simple western medicine comprehensive therapy. Methods Fifty-eight cases were randomized into an acupuncture plus medication group （28 cases, 32 damaged ears） and a western medicine group （30 cases, 34 damaged ears）. In the acupuncture plus medication group, the comprehensive therapy of electroacupuncture and western medicine was used. The main points were ěrmén （耳门 TE 21）, Tīnggōng （听宫 SI 19）, Tīnghuì （听会 GB 2）, Yìfēng （翳风 TE 17）, etc. The western medicine therapy was the comprehensive treatment with vasodilators, the medicines for promoting nerve function and releasing inner ear edema and hyperbaric oxygen. In the western medicine group, the western medicine comprehensive therapy was adopted. 10-day treatment made one session. Two sessions of treatment were required. In 1 or 2 sessions of treatment, the efficacy was observed, the electric audiometry was evaluated. Additionally, the blood routine and liver and kidney functions were detected. Results The total effective rate was 50.0% （16/32） in the acupuncture ＋ medication group and was 55.9% （19/34） in the western medicine group. The efficacies were similar between the two groups （P〉0.05）. In 1 and 2 sessions of treatment, at 250 Hz, 500 Hz and 1 kHz, the electric audiometry values in either group were recovered significantly as compared with those before treatment （all P〈0.01）. At 2 kHz, in 1 and 2 sessions of treatment, the improvements of the electric audiometry values were significant as compared with those before treatment in the acupuncture plus medication group separately （P〈0.05, P〈0.01）. At 4 kHz, the differences were not significant statistically before and after treatment in either group （all P〉0.05）. There were no abnormalities in the blood routine and liver and kidney function detections before and after treatment in either group. Conclusion Either the comprehensive therapy of electroacupuncture and western medicine or the simple western medicine comprehensive therapy is effective in the treatment of low frequency hearing damage of sudden deafness. Either of them has no effect on high frequency hearing damage. But for the middle frequency hearing damage, the efficacy of the comprehensive therapy of electroacupuncture and western medicine is superior to that of the simple western medicine comprehensive therapy.展开更多
Objective:To analyze the necessity and effectiveness of vestibular rehabilitation training instruction in the treatment of sudden deafness accompanied by vertigo.Methods:Sixty medical staff who learned the treatment m...Objective:To analyze the necessity and effectiveness of vestibular rehabilitation training instruction in the treatment of sudden deafness accompanied by vertigo.Methods:Sixty medical staff who learned the treatment methods for sudden deafness accompanied by vertigo(vestibular rehabilitation training)from January 2024 to February 2025 were selected as the research subjects.They were divided into two groups according to the time of study:the control group,who participated in the training teaching first,and the observation group,who participated later.Each group had 30 people.The control group received conventional training,while the observation group underwent continuous quality improvement.The effects and assessment results of different teaching methods were compared.Results:The theoretical and practical assessment scores of medical staff in the observation group were higher than those in the control group(p<0.05);the satisfaction of medical staff in the observation group with the teaching mode was higher than that in the control group(p<0.05).Conclusion:Vestibular rehabilitation training has significant effects on sudden deafness accompanied by vertigo.Training medical staff not only enhances their understanding of training methods but also enables them to provide specialized guidance to patients,improving their vertigo condition.展开更多
Extrapontine myelinolysis and pure word deafness are very uncommon disorders.Here,we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness...Extrapontine myelinolysis and pure word deafness are very uncommon disorders.Here,we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness.As a consequence of rapid correction of hyponatremia,the patient demonstrated an initial onset of cortical deafness,and then progressed to generalized auditory agnosia,which eventually developed into confined verbal auditory agnosia(pure word deafness) .Bilateral extrapontine myelinolysis was confirmed using brain magnetic resonance imaging.This case suggests that verbal and nonverbal stimuli may involve separate thalamocortical pathways.展开更多
The present study was undertaken to measure a-b, a-d and td ridge counts of deaf patients and to compare the dermatoglyphic differences among deaf individuals. The data consists of 208 heredo-familial Punjabi Khatri a...The present study was undertaken to measure a-b, a-d and td ridge counts of deaf patients and to compare the dermatoglyphic differences among deaf individuals. The data consists of 208 heredo-familial Punjabi Khatri and Arora deaf subjects, including 118 males and 90 femeles. The control sample consists of 228 Punjabis, comprising of 128 males and 100 females. The present study indicates lower incidence of ’a-b’, ’a-d’ and ’td’ ridge counts in deaf groups when compared to the corresponding normal groups.展开更多
Objective: Observing the expression changes of serum proteome in model rats after intervention of the Granules of Eliminating Phlegm and Removing Blood Stasis (豁痰祛瘀颗粒 also known as GEPRB), screening out and iden...Objective: Observing the expression changes of serum proteome in model rats after intervention of the Granules of Eliminating Phlegm and Removing Blood Stasis (豁痰祛瘀颗粒 also known as GEPRB), screening out and identifying the differentially expressed proteins by mass spectrometry and bioinformatics analysis, discussing the molecular mechanism of control the Diabetes deafness by GEPRB. Methods: By use of proteomics technology, the serum protein serum proteome of the control group, model control group, Duxil and each observation group were observed for 2-DE gel pattern matching, and the difference in the relative content of 2 times was chosen for the differentially expressed proteins. Identification of differentially expressed proteins by MALDI-TOF MS/MS, the authors further analysis the phosphorylation, subcellular localization, interaction, direct regulation, and transmembrane of the differences proteins by the way of bioinformatics analysis. Sixty SPF level SD rats elected in diabetic rats model group (abbreviated as DM group) were be randomly divided into 5 groups based on random number sheet, namely model control group, positive drug control group (Du-ke-xi group) and Mai-tong-fang high, medium and low dose group respectively. In addition, set of normal control group. 10 rats in each group. Results: By Coomassie brilliant blue staining, identified 51 differential protein spots dug from 2-D gel by mass spectrometry, successfully identified 13 non-redundant proteins. Most of the identified proteins were secreted protein and belong to different protein families. There were about 12 proteins have the transmembrane region from the authors’ result, ten of them were plasma membrane proteins. Conclusion: It’s suggesting that 13 differential proteins is most likely the protein response to GEPRB in vivo, these proteins may play key role for the treatment of GEPRB to Diabetes deafness. The two highly differentially expressed proteins Apolipoprotein E (apoE) and C3 may be a potential drug target of GEPRB.展开更多
Objective： To observe the effect of acupuncture at Jǐngjiājǐ（颈夹脊）on the repair and regeneration of cochlear hair cells of guinea pigs with sensorineural deafness.Methods： Sixty healthy guinea pigs were selec...Objective： To observe the effect of acupuncture at Jǐngjiājǐ（颈夹脊）on the repair and regeneration of cochlear hair cells of guinea pigs with sensorineural deafness.Methods： Sixty healthy guinea pigs were selected, 20 guinea pigs were randomly assigned to the normal control group（group A）, and other guinea pigs were randomly divided into model control group（group B） and acupuncture treatment group（group C） after injection with gentamicin sulfate in order to induce deafness. No intervention was given to the guinea pigs in group A and group B, and acupuncture at Jǐngjiājǐ was given to the guinea pigs in group C for 30 days. ABR threshold, DPOAE amplitudes and hair cells counting of guinea pigs in each group were recorded after intervention for 30 days.Results： After intervention for 30 days, ABR threshold in group C was significantly lower than that in group B（38.46 ±7.36 vs 82.94 ±6.47, P〈0.01）. and the DPOAE amplitudes in group C were obviously higher than that in group B（28.06 ±5.64 vs 25.23 ±5.38, P〈0.01）. The number of cochlear hair cells in group C increased significantly, over 50% of the hair cells survived, accounting for 66.67% of the observation cases. The number of cochlear hair cells in the 3 rd and 4 th gyri was close to the normal level, and plenty of proliferous sustentacular cells can be seen. Compared with group B, the number of cochlear outer hair cells in each gyrus in group C significantly increased（36.76 ±1.97 vs 28.59±2.24, P〈0.01）, indicating that acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells.Conclusion： Acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells, thus improving the hearing of guinea pigs with deafness.展开更多
Mutations in mitochondrial tRNA genes have been shown to be associated with maternally inherited syn-dromic and non-syndromic deafness. Among those, mutations such as tRNALeu(UUR) 3243A>G associated with syndromic ...Mutations in mitochondrial tRNA genes have been shown to be associated with maternally inherited syn-dromic and non-syndromic deafness. Among those, mutations such as tRNALeu(UUR) 3243A>G associated with syndromic deafness are often present in heteroplasmy, and the non-syndromic deafness-associated tRNA mu-tations including tRNASer(UCN) 7445A>G are often in homoplasmy or in high levels of heteroplasmy. These tRNA mutations are the primary factors underlying the development of hearing loss. However, other tRNA mutations such as tRNAThr 15927G>A and tRNASer(UCN) 7444G>A are insufficient to produce a deafness phe-notype, but always act in synergy with the primary mitochondrial DNA mutations, and can modulate their phenotypic manifestation. These tRNA mutations may alter the structure and function of the corresponding mitochondrial tRNAs and cause failures in tRNAs metabolism. Thereby, the impairment of mitochondrial protein synthesis and subsequent defects in respiration caused by these tRNA mutations, results in mitochon-drial dysfunctions and eventually leads to the development of hearing loss. Here, we summarized the deaf-ness-associated mitochondrial tRNA mutations and discussed the pathophysiology of these mitochondrial tRNA mutations, and we hope these data will provide a foundation for the early diagnosis, management, and treatment of maternally inherited deafness.展开更多
Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatl...Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatly different.Traditional Chinese medicine(TCM)believes that the onset of sudden deafness is related to the liver,gallbladder,heart and triple energizer meridians.This article mainly explored the key points of common syndromes,TCM nursing methods and health guidance of sudden deafness.展开更多
Led by four generations of leadership from late Prof.JIANG Sichang(academician,Chinese Academy of Engineering),Prof.YANG Weiyan(Honorary President,Division of Otolaryngology Head and Neck Surgery,Chinese Medical Assoc...Led by four generations of leadership from late Prof.JIANG Sichang(academician,Chinese Academy of Engineering),Prof.YANG Weiyan(Honorary President,Division of Otolaryngology Head and Neck Surgery,Chinese Medical Association),Prof.HAN Dongyi(President Elected,Division of Otolaryngology Head and Neck Surgery,Chinese Medical Association)to now Prof.YANG Shiming(President,Division of Otolaryngologists,展开更多
Hearing loss is the most common sensorineural disorder worldwide and is associated with more than1000 mutations in more than 90 genes. While mutations in genes such as GJB2(gap-junction protein β 2)and GJB6(gap-junct...Hearing loss is the most common sensorineural disorder worldwide and is associated with more than1000 mutations in more than 90 genes. While mutations in genes such as GJB2(gap-junction protein β 2)and GJB6(gap-junction protein β 6) are highly prevalent in Caucasian, Asian, and Middle Eastern populations, they are rare in both native African populations and those of African descent. The objective of this paper is to review the current knowledge regarding the epidemiology and genetics of hearing loss in African populations with a focus on native sub-Saharan African populations. Environmental etiologies related to poor access to healthcare and perinatal care account for the majority of cases. Syndromic etiologies including Waardenburg, Pendred and Usher syndromes are uncommon causes of hearing loss in these populations. Of the non-syndromic causes, common mutations in GJB2 and GJB6 are rarely implicated in populations of African descent. Recent use of next-generation sequencing(NGS) has identified several candidate deafness genes in African populations from Nigeria and South Africa that are unique when compared to common causative mutations worldwide. Researchers also recently described a dominant mutation in MYO3α in an African American family with non-syndromic hearing loss. The use of NGS and specialized panels will aid in identifying rare and novel mutations in a more cost-and timeeffective manner. The identification of common hearing loss mutations in indigenous African populations will pave the way for translation into genetic deafness research in populations of African descent worldwide.展开更多
文摘The etiology of sudden deafness or idiopathic sudden sensorineural hearing loss(ISSHL) remains unclear. Over the past 15 years, we have investigated the mechanisms of ischemic-induced hearing loss using a gerbil model of transient cochlear ischemia. In the gerbil, cochlear ischemia can be induced by occluding the bilateral vertebral arteries simultaneously at the neck, because the posterior communicating arteries of the Circle of Willis close spontaneously around 1 mo after birth. When 15 min ischemia was loaded on this animal, permanent hearing loss of about 25 d B and the death of hair cells, especially inner hair cells were induced. These pathological changes were mainly due to lack of an energy source, glutamate excitotoxicity, and the production of free radicals, especially superoxide and nitrous oxide species. Ischemic damage could be prevented by various procedures, such as cooling the cochlea, intratympanic administration of insulin-like growth factor 1 or AM-111(an anti-apoptotic agent), and systemic administration of prednisolone(steroid), edarabone(free radical scavenger), ginsenoside Rb1(Kanpo), hematopoietic stem cells, glia-cell derived neurotrophic factor, and liposome-encapsulated hemoglobin(artificial red blood cells). We also found that the cochlea was protected by the ischemic tolerance, indi-cating that minor cochlear ischemia alleviates or prevents inner ear damage in subsequent severe cochlear ischemia. As ISSHL usually occurs suddenly, with no preceding sign or symptom, we suggest that most ISSHL cases are caused by circulatory disturbance, probably at the stria vascularis.
文摘X-linked deafness is a rare genetic disorder causing a severe mixed hearing loss. This is due to an abnormal connection between the internal auditory meatus(IAM) and the basal turn of the cochlear leading to a "3rd window effect" and cochlear conductive hearing loss. Patients are traditionally treated with conventionalhearing aids however these are often unsatisfactory. Cochlear implantation is a high-risk procedure in such cases due to the risk of inadvertent electrode placement in the IAM. We present three paediatric cases where the hearing loss was managed with a combination of a bone anchored hearing aid in combination with a conventional behind the ear hearing aid. We also present a review of the current literature regarding the management of X-linked deafness.
文摘Objective: In order to provide a theoretical basis for the revision of the current diagnostic criteria for occupational noise-induced deafness (ONID), we evaluated the degree of ONID by analyzing different high-frequency-hearing- threshold-weighted values (HFTWVs). Methods: A retrospective study was conducted to evaluate the diagnosis of patients with ONID from January 2016 to January 2017 in Guangdong province, China. Based on 3 hearing tests (each interval between the tests was greater than 3 days), the minimum threshold value of each frequency was obtained using the 2007 edition’s diagnostic criteria for ONID. The speech frequency and the HFTWVs were analyzed based on age, noise exposure, and diagnostic classi-fication using SPSS21.0. Results: 168 patients in total were involved in this study, 154 males and 14 females, and the average age was 41.18 ± 6.07. The diagnosis rate was increased by the weighted value of the high frequencies and was more than the mean value of the pure speech frequency (MVPSF). The diagnosis rate for the weighted 4 kHz frequency level increased by 13.69% (χ2 = 9.880, P = 0.002), the weighted 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002), and the weighted 4 kHz + 6 kHz level increased by 15.47% (χ2 = 9.985, P = 0.002). The differences were all statistically significant. The diagnostic rate of the different thresholds showed no obvious difference between the genders. The age groups were divided into less than or equal to 40 years old (group A) and 40 - 50 years old (group B). There were several groups with a high frequency: high frequency weighted 4 kHz ( group A χ2 = 3.380, P = 0.050;group B χ2 = 4.054, P = 0.032), high frequency weighted 6 kHz (group A χ2 = 6.362, P = 0.012;group B χ2 = 4.054, P = 0.032), weighted 4 kHz + 6 kHz (group A χ2 = 6.362 P = 0.012;B χ2 = 4.054, P = 0.032) than those of MVPSF in the same group on ONID diagnosis rate. The differences between the groups were statistically significant. There was no significant difference between the age groups (χ2 = 2.265, P = 0.944). The better ear’s (the smaller hearing threshold weighted value) MVPSF and the weighted values for the different high frequencies were examined in light of the number of working years;the group that was exposed to noise for more than 10 years had significantly higher values than those of the average thresholds of each frequency band in the groups with 3 - 5 years of exposure (F = 2.271, P = 0.001) and 6 - 10 years of exposure (F = 1.563, P = 0.046). The differences were statistically significant. The different HFTWVs were higher than those of the MVPSF values, and the high frequency weighted 4 kHz + 6 kHz level showed the greatest difference, with an average increase of 2.83 dB. The diagnostic rate that included the weighted high frequency values was higher for the mild, moderate, and severe cases than those patients who were only screened with the pure frequency tests. The results of the comparisons of the diagnosis rates for mild ONID were as follows: the weighted 3 kHz high frequency level (χ2 = 3.117, P = 0.077) had no significant difference, but the weighted 4 kHz level (χ2 = 10.835, P = 0.001), 6 kHz level (χ2 = 9.985, P = 0.002), 3 kHz + 4 kHz level (χ2 = 6.315, P = 0.012), 3 kHz + 6 kHz level (χ2 = 6.315, P = 0.012), 4 kHz + 6 kHz level (χ2 = 9.985, P = 0.002), and 3 kHz + 4 kHz + 6 kHz level (χ2 = 7.667, P = 0.002) were significantly higher than the diagnosis rate of the mean value of the PSF. There was no significant difference between the 2 groups for the moderate and severe grades (P > 0.05). Conclusion: Different HFTWVs increase the diagnostic rate of ONID. The weighted 4 kHz, 6 kHz, and 4 kHz + 6 kHz high frequency values greatly affected the diagnostic results, and the weighted 4 kHz + 6 kHz high frequency hearing threshold value has the maximum the effect on the ONID diagnosis results.
文摘BACKGROUND Managing sudden deafness(SD)in patients with diabetes mellitus(DM)is partic-ularly challenging due to the heightened risk of adverse effects associated with systemic drug administration.This study explores the potential of retroauricular subperiosteal injection as a localized drug delivery method for a more effective and safe treatment.AIM To compare the efficacy of retroauricular subperiosteal injection vs systemic intravenous glucocorticoid(GC)administration for SD in patients with DM and assess the effects on blood glucose levels.METHODS A total of 128 cases of type 2 DM(T2DM)with SD diagnosed and treated in Zibo Central Hospital from February 2021 to July 2023 were divided into two groups:An observation group(66 cases receiving retroauricular subperiosteal injection of methylprednisolone)and a control group(62 cases receiving systemic intravenous administration of methylprednisolone).The two groups were compared in terms of therapeutic efficacy,hearing recovery,blood glucose level changes,and incidence of adverse reactions.Binary logistic regression was used to analyze the factors affecting therapeutic efficacy.RESULTS The observation group showed a significantly higher total effective rate(90.91%)compared with the control group(75.81%,P<0.05).Additionally,pure-tone hearing threshold,fasting plasma glucose,and 2-hour postprandial blood glucose were significantly lower in the observation group compared with the control group(P<0.05).The incidence of adverse reactions was also lower in the observation group than in the control group(7.58%vs 22.58%,P<0.05).A T2DM course longer than 5 years and systemic intravenous GC administration were identified as independent risk factors for treatment inefficacy(P<0.05).INTRODUCTION Sudden deafness(SD)is a clinical emergency characterized by rapid-onset hearing loss that is often accompanied by clinical symptoms such as tinnitus and vertigo[1].Although its pathogenesis remains unclear,it is supposedly associated with factors,including inner ear microcirculation disorders,autoimmune diseases,and viral infections[2,3].Patients with diabetes are particularly susceptible to microvascular complications due to poor long-term glycemic control,affecting the ear’s microcirculation,subsequently increasing the risk of SD[4].Type 2 diabetes mellitus(T2DM),a chronic metabolic disease,causes multiple microvascular damages throughout the body,complicating SD treatment in patients with diabetes[5,6].Inner ear microcirculation disturbances in patients with diabetes may exacerbate the risk of SD,and its pathological process may be related to vascular endothelial dysfunction,inflammatory reactions,and hemorrhological changes caused by diabetes mellitus(DM)[7].Current SD treatments include glucocorticoids(GCs),vasodilators,and hyperbaric oxygen therapy[8].GCs are widely used due to their anti-inflammatory and immunosuppressive effects[9].However,systemic GCs may cause blood glucose(BG)fluctuations and even increase the risk of complications in patients with DM,limiting their clinical use in this population[10].Therefore,there is a compelling and immediate need for a local alternative solution that minimally affects the metabolic mechanisms.In recent years,retroauricular subperiosteal injection has emerged as a localized administration modality for treating SD[11].This method allows drugs to directly act on the inner ear,avoiding the side effects of systemic administration and having a minor impact on BG levels,providing a potentially effective treatment for patients with diabetes[12].However,there is limited clinical evidence to compare the efficacy and glycaemic effects of retroauricular subperiosteal injection vs systemic intravenous GC administration in patients with SD and diabetes.This study aimed to explore the efficacy of retroauricular subperiosteal injection and systemic intravenous GC administration for treating patients with SD and DM and their effects on BG,providing a safer and more effective clinical treatment approach.
文摘Objective:To investigate the expressions of microRNA-155 (miR-155) and histone deacetylase 2 (HDAC2) in peripheral blood mononuclear cells (PBMC) of patients with sudden deafness (SSNHL) and their clinical significances.Methods:112 patients with SSNHL who were treated in our hospital from February 2017 to September 2018 were selected as the study subjects, and they were referred to as sudden deafness group, and another 115 healthy examinees in the same period were compared and studied as normal group. The relative expression levels of miR-155, HDAC2, ICAM-1, TNF-α and interleukin-8 (IL-8) in PBMC of all subjects were detected by real-time fluorescence quantitative analysis (qRT-PCR). Pearson method was used to analyze the relationships between miR-155, HDAC2 and the expressions of ICAM-1, TNF-α, IL-8, and miR-155 and the expression of HDAC2 in PBMC of SSNHL patients. Logistic regression analysis was used to analyze the risk factors of SSNHL. The predictive diagnostic values of miR-155 and HDAC2 in PBMC for SSNHL were evaluated. Results:The relative expression levels of miR-155, ICAM-1, TNF-α and IL-8 in PBMC of sudden deafness group were significantly higher than those of normal group (P<0.05), while the relative expression levels of HDAC2 were significantly lower than those of normal group (P<0.05). In SSNHL patients, miR-155 were positively correlated with the expression levels of ICAM-1, TNF-α and IL-8 (P<0.05). The expression level of HDAC2 was negatively correlated with the expression levels of TNF-α and IL-8 (P<0.05). The expression level of miR-155 in PBMC of SSNHL patients was negatively correlated with HDAC2 (P<0.05). MiR-155, ICAM-1, TNF-α and IL-8 were risk factors for SSNHL (P<0.05), while HDAC2 was protective factor for SSNHL (P<0.05). The areas under curve (AUC) of miR-155 and HDAC2 in PBMC for SSNHL diagnosis were 0.855 and 0.835 respectively, the truncation values of which were 1.449 and 0.959 respectively, at this moment, the sensitivities were 75.0% and 81.3% respectively, and the corresponding specificities were 86.1% and 70.1% respectively. The AUC of combined diagnosis of SSNHL was 0.927, and the sensitivity and specificity were 86.7% and 85.3% respectively.Conclusions:The expressions of miR-155 is high and HDAC2 is low in PBMC of SSNHL patients, which are negatively correlated, and both of them may participate in the occurrence and development of SSNHL through mutual influence, which is a risk factor of SSNHL. The combination of the two can effectively improve the predictive diagnostic value of SSNHL.
基金Supported by Beijing Holistic Integrative Medicine Association Clinical Research Funding Program,No.ZHKY-2024-2209.
文摘BACKGROUND Wolfram syndrome is a rare autosomal recessive genetic disorder characterized by early-onset diabetes and progressive neurodegeneration,most notably sensorineural hearing loss and optic atrophy.Because its initial manifestations are usually similar to those of type 1 diabetes,the diagnosis may be delayed until other manifestations appear.Pathogenic variations of the WFS1 gene can disrupt endoplasmic reticulum function and cellular homeostasis,but the complete mutation spectrum of WFS1 has not been fully determined.Early identification of monogenic diabetes caused by Wolfram syndrome is of vital importance,as it enables the provision of targeted multidisciplinary care and genetic counseling for affected families.CASE SUMMARY A 2-year-old Han Chinese girl was admitted with a 1-month history of polydipsia,polyuria,and polyphagia,and was diagnosed with diabetic ketoacidosis and impaired insulin secretion.Sensorineural hearing loss was also detected.Wholeexome sequencing identified a previously unreported heterozygous mutation,WFS1 c.986T>C(p.Phe329Ser),in the patient and her father,confirming the diagnosis of Wolfram syndrome.Bioinformatic analysis supported the likely pathogenicity of this mutation.In silico pathogenicity predictors(REVEL,SIFT,Poly-Phen-2,MutationTaster,and GERP+)supported a deleterious effect on wolframin structure and function.The patient was initially treated with intravenous insulin and fluid resuscitation,then transitioned to a basal–bolus insulin regimen.Glycemic control was subsequently maintained with continuous subcutaneous insulin infusion and intermittent subcutaneous injections.At the 1-and 6-month follow-ups,blood glucose remained well controlled(hemoglobin A1c:5.89%and 6.58%,respectively),with no evidence of organ dysfunction or further complications.CONCLUSION This case identifies WFS1 c.986T>C(p.Phe329Ser)as a novel pathogenic variant causing Wolfram syndrome.It highlights the importance of early genetic testing in pediatric patients with atypical diabetes presentations to enable timely diagnosis,individualized therapy,and comprehensive family support.
文摘100 cases of nerve deafness were treated with Injectio Radix Salviae Miltiorrhizae(丹参注射注)by i.v.drip,and additional drugs that promoted blood circulation were used accordingto symptom differentiation.Results were 28 cases cured,45 cases improved,and 27 casesfailed,for a total effective rate of 73%.
文摘In this paper, the auricular reaction spots of 40 affected children with ner-vous and drug-induced deafness, and 20 healthy children were observed. The result of the re-gular distribution of these spots, which is very significantly different between deafness group andcontrol group, suggests that otopoints Kidney, Brain and Ear where the reaction spots concen-trate play a role in diagnosis, treatment and prevention of child’s deafness.
文摘Profound congenital sensorineural hearing loss(SNHL) is not so infrequent,affecting 1 to 2 of every 1000 newborns in western countries.Nevertheless,universal hearing screening programs have not been widely applied,although such programs are already established for metabolic diseases.The acquisition of spoken language is a time-dependent process,and some form linguistic input should be present before the first 6 mo of life for a child to become linguistically competent.Therefore,profoundly deaf children should be detected early,and referred timely for the process of auditory rehabilitation to be initiated.Hearing assessment methods should reflect the behavioural audiogram in an accurate manner.Additional disabilities also need to be taken into account.Profound congenital SNHL is managed by a multidisciplinary team.Affected infants should be bilaterally fitted with hearing aids,no later than 3 mo after birth.They should be monitored until the first year of age.If they are not progressing linguistically,cochlear implantation can be considered after thorough preoperative assessment.Prelingually deaf children develop significant speech perception and production abilities,and speech intelligibility over time,following cochlear implantation.Age at intervention and oral communication,are the most important determi-nants of outcomes.Realistic parental expectations are also essential.Cochlear implant programs deserve the strong support of community members,professional bodies,and political authorities in order to be successful,and maximize the future earnings of pediatric cochlear implantation for human societies.
文摘Objective To compare the difference in the clinical efficacy on the different frequency hearing damages for the patients with sudden deafness between the comprehensive therapy of electroacupuncture and western medicine and the simple western medicine comprehensive therapy. Methods Fifty-eight cases were randomized into an acupuncture plus medication group （28 cases, 32 damaged ears） and a western medicine group （30 cases, 34 damaged ears）. In the acupuncture plus medication group, the comprehensive therapy of electroacupuncture and western medicine was used. The main points were ěrmén （耳门 TE 21）, Tīnggōng （听宫 SI 19）, Tīnghuì （听会 GB 2）, Yìfēng （翳风 TE 17）, etc. The western medicine therapy was the comprehensive treatment with vasodilators, the medicines for promoting nerve function and releasing inner ear edema and hyperbaric oxygen. In the western medicine group, the western medicine comprehensive therapy was adopted. 10-day treatment made one session. Two sessions of treatment were required. In 1 or 2 sessions of treatment, the efficacy was observed, the electric audiometry was evaluated. Additionally, the blood routine and liver and kidney functions were detected. Results The total effective rate was 50.0% （16/32） in the acupuncture ＋ medication group and was 55.9% （19/34） in the western medicine group. The efficacies were similar between the two groups （P〉0.05）. In 1 and 2 sessions of treatment, at 250 Hz, 500 Hz and 1 kHz, the electric audiometry values in either group were recovered significantly as compared with those before treatment （all P〈0.01）. At 2 kHz, in 1 and 2 sessions of treatment, the improvements of the electric audiometry values were significant as compared with those before treatment in the acupuncture plus medication group separately （P〈0.05, P〈0.01）. At 4 kHz, the differences were not significant statistically before and after treatment in either group （all P〉0.05）. There were no abnormalities in the blood routine and liver and kidney function detections before and after treatment in either group. Conclusion Either the comprehensive therapy of electroacupuncture and western medicine or the simple western medicine comprehensive therapy is effective in the treatment of low frequency hearing damage of sudden deafness. Either of them has no effect on high frequency hearing damage. But for the middle frequency hearing damage, the efficacy of the comprehensive therapy of electroacupuncture and western medicine is superior to that of the simple western medicine comprehensive therapy.
文摘Objective:To analyze the necessity and effectiveness of vestibular rehabilitation training instruction in the treatment of sudden deafness accompanied by vertigo.Methods:Sixty medical staff who learned the treatment methods for sudden deafness accompanied by vertigo(vestibular rehabilitation training)from January 2024 to February 2025 were selected as the research subjects.They were divided into two groups according to the time of study:the control group,who participated in the training teaching first,and the observation group,who participated later.Each group had 30 people.The control group received conventional training,while the observation group underwent continuous quality improvement.The effects and assessment results of different teaching methods were compared.Results:The theoretical and practical assessment scores of medical staff in the observation group were higher than those in the control group(p<0.05);the satisfaction of medical staff in the observation group with the teaching mode was higher than that in the control group(p<0.05).Conclusion:Vestibular rehabilitation training has significant effects on sudden deafness accompanied by vertigo.Training medical staff not only enhances their understanding of training methods but also enables them to provide specialized guidance to patients,improving their vertigo condition.
基金Project supported by the Science Research Foundation of the Ministry of Health of China(No.WKJ2010-2-009)the Science and Technology Department of Zhejiang Province(No.2007C33007)+1 种基金the Key Project of Education Department of Zhejiang Province(No.Z2008 05185)the Natural Science Foundation of Zhejiang Province(No. Y2080132),China
文摘Extrapontine myelinolysis and pure word deafness are very uncommon disorders.Here,we report a case of a 19-year-old woman who suffered from osmotic demyelination syndrome with coincidence of typical pure word deafness.As a consequence of rapid correction of hyponatremia,the patient demonstrated an initial onset of cortical deafness,and then progressed to generalized auditory agnosia,which eventually developed into confined verbal auditory agnosia(pure word deafness) .Bilateral extrapontine myelinolysis was confirmed using brain magnetic resonance imaging.This case suggests that verbal and nonverbal stimuli may involve separate thalamocortical pathways.
文摘The present study was undertaken to measure a-b, a-d and td ridge counts of deaf patients and to compare the dermatoglyphic differences among deaf individuals. The data consists of 208 heredo-familial Punjabi Khatri and Arora deaf subjects, including 118 males and 90 femeles. The control sample consists of 228 Punjabis, comprising of 128 males and 100 females. The present study indicates lower incidence of ’a-b’, ’a-d’ and ’td’ ridge counts in deaf groups when compared to the corresponding normal groups.
文摘Objective: Observing the expression changes of serum proteome in model rats after intervention of the Granules of Eliminating Phlegm and Removing Blood Stasis (豁痰祛瘀颗粒 also known as GEPRB), screening out and identifying the differentially expressed proteins by mass spectrometry and bioinformatics analysis, discussing the molecular mechanism of control the Diabetes deafness by GEPRB. Methods: By use of proteomics technology, the serum protein serum proteome of the control group, model control group, Duxil and each observation group were observed for 2-DE gel pattern matching, and the difference in the relative content of 2 times was chosen for the differentially expressed proteins. Identification of differentially expressed proteins by MALDI-TOF MS/MS, the authors further analysis the phosphorylation, subcellular localization, interaction, direct regulation, and transmembrane of the differences proteins by the way of bioinformatics analysis. Sixty SPF level SD rats elected in diabetic rats model group (abbreviated as DM group) were be randomly divided into 5 groups based on random number sheet, namely model control group, positive drug control group (Du-ke-xi group) and Mai-tong-fang high, medium and low dose group respectively. In addition, set of normal control group. 10 rats in each group. Results: By Coomassie brilliant blue staining, identified 51 differential protein spots dug from 2-D gel by mass spectrometry, successfully identified 13 non-redundant proteins. Most of the identified proteins were secreted protein and belong to different protein families. There were about 12 proteins have the transmembrane region from the authors’ result, ten of them were plasma membrane proteins. Conclusion: It’s suggesting that 13 differential proteins is most likely the protein response to GEPRB in vivo, these proteins may play key role for the treatment of GEPRB to Diabetes deafness. The two highly differentially expressed proteins Apolipoprotein E (apoE) and C3 may be a potential drug target of GEPRB.
基金Supported by The Education Department of Jilin Province,"12th Five-year Plan" science and technology research planning project:J.J.K.H.Z.[2015]No.33~~
文摘Objective： To observe the effect of acupuncture at Jǐngjiājǐ（颈夹脊）on the repair and regeneration of cochlear hair cells of guinea pigs with sensorineural deafness.Methods： Sixty healthy guinea pigs were selected, 20 guinea pigs were randomly assigned to the normal control group（group A）, and other guinea pigs were randomly divided into model control group（group B） and acupuncture treatment group（group C） after injection with gentamicin sulfate in order to induce deafness. No intervention was given to the guinea pigs in group A and group B, and acupuncture at Jǐngjiājǐ was given to the guinea pigs in group C for 30 days. ABR threshold, DPOAE amplitudes and hair cells counting of guinea pigs in each group were recorded after intervention for 30 days.Results： After intervention for 30 days, ABR threshold in group C was significantly lower than that in group B（38.46 ±7.36 vs 82.94 ±6.47, P〈0.01）. and the DPOAE amplitudes in group C were obviously higher than that in group B（28.06 ±5.64 vs 25.23 ±5.38, P〈0.01）. The number of cochlear hair cells in group C increased significantly, over 50% of the hair cells survived, accounting for 66.67% of the observation cases. The number of cochlear hair cells in the 3 rd and 4 th gyri was close to the normal level, and plenty of proliferous sustentacular cells can be seen. Compared with group B, the number of cochlear outer hair cells in each gyrus in group C significantly increased（36.76 ±1.97 vs 28.59±2.24, P〈0.01）, indicating that acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells.Conclusion： Acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells, thus improving the hearing of guinea pigs with deafness.
基金supported by grants from The National Natural Science Foundation of China(81070794 and 31100903)The Natural Science Foundation of Zhejiang Province(Y2110399)The China Postdoctoral Science Foundation(2013M531472)
文摘Mutations in mitochondrial tRNA genes have been shown to be associated with maternally inherited syn-dromic and non-syndromic deafness. Among those, mutations such as tRNALeu(UUR) 3243A>G associated with syndromic deafness are often present in heteroplasmy, and the non-syndromic deafness-associated tRNA mu-tations including tRNASer(UCN) 7445A>G are often in homoplasmy or in high levels of heteroplasmy. These tRNA mutations are the primary factors underlying the development of hearing loss. However, other tRNA mutations such as tRNAThr 15927G>A and tRNASer(UCN) 7444G>A are insufficient to produce a deafness phe-notype, but always act in synergy with the primary mitochondrial DNA mutations, and can modulate their phenotypic manifestation. These tRNA mutations may alter the structure and function of the corresponding mitochondrial tRNAs and cause failures in tRNAs metabolism. Thereby, the impairment of mitochondrial protein synthesis and subsequent defects in respiration caused by these tRNA mutations, results in mitochon-drial dysfunctions and eventually leads to the development of hearing loss. Here, we summarized the deaf-ness-associated mitochondrial tRNA mutations and discussed the pathophysiology of these mitochondrial tRNA mutations, and we hope these data will provide a foundation for the early diagnosis, management, and treatment of maternally inherited deafness.
文摘Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatly different.Traditional Chinese medicine(TCM)believes that the onset of sudden deafness is related to the liver,gallbladder,heart and triple energizer meridians.This article mainly explored the key points of common syndromes,TCM nursing methods and health guidance of sudden deafness.
文摘Led by four generations of leadership from late Prof.JIANG Sichang(academician,Chinese Academy of Engineering),Prof.YANG Weiyan(Honorary President,Division of Otolaryngology Head and Neck Surgery,Chinese Medical Association),Prof.HAN Dongyi(President Elected,Division of Otolaryngology Head and Neck Surgery,Chinese Medical Association)to now Prof.YANG Shiming(President,Division of Otolaryngologists,
基金supported by grants from the National Institutes of Health/National Institute on Deafness and Other Communication Disorders to Xuezhong Liu (R01 DC05575,R01 DC01246,2P50DC000422-Sub-Project 6432,and R01 DC012115)the University of Pretoria RDP grant and the South African ENT Society Research Grant to RI Kabahuma
文摘Hearing loss is the most common sensorineural disorder worldwide and is associated with more than1000 mutations in more than 90 genes. While mutations in genes such as GJB2(gap-junction protein β 2)and GJB6(gap-junction protein β 6) are highly prevalent in Caucasian, Asian, and Middle Eastern populations, they are rare in both native African populations and those of African descent. The objective of this paper is to review the current knowledge regarding the epidemiology and genetics of hearing loss in African populations with a focus on native sub-Saharan African populations. Environmental etiologies related to poor access to healthcare and perinatal care account for the majority of cases. Syndromic etiologies including Waardenburg, Pendred and Usher syndromes are uncommon causes of hearing loss in these populations. Of the non-syndromic causes, common mutations in GJB2 and GJB6 are rarely implicated in populations of African descent. Recent use of next-generation sequencing(NGS) has identified several candidate deafness genes in African populations from Nigeria and South Africa that are unique when compared to common causative mutations worldwide. Researchers also recently described a dominant mutation in MYO3α in an African American family with non-syndromic hearing loss. The use of NGS and specialized panels will aid in identifying rare and novel mutations in a more cost-and timeeffective manner. The identification of common hearing loss mutations in indigenous African populations will pave the way for translation into genetic deafness research in populations of African descent worldwide.
