This case is a 49-year-old female patient who presented with suspected eustachian tube dysfunction symptoms that were refractory to medical treatment. She presented with conductive hearing loss and aural fullness on t...This case is a 49-year-old female patient who presented with suspected eustachian tube dysfunction symptoms that were refractory to medical treatment. She presented with conductive hearing loss and aural fullness on the left. A physical exam revealed an epitympanic fleshy mass in the middle ear with effusion. Imaging with MRI and CT showed opacification of the epitympanum with surrounding bony demineralization bilaterally left greater than right and significant thinning versus dehiscence of the tegmen mastoideum and tympani on the left with only thinning of the tegmen on the right. An MR temporal bone with and without contrast, demonstrated enhancement of the left middle ear extending to the tegmen and corresponding enhancement of the dura along the floor of the left middle cranial fossa as well as extending along foramen ovale into the infratemporal fossa. There was to a lesser degree similar appearance on the right. A biopsy of the middle ear mass on the left revealed meningioma. This case highlights the need to widen your differential with common symptoms when they are refractory to treatment.展开更多
miRNA-183 family, in normal biology, is expressed in a harmonious and stable manner in the neurosensory organs and cells. Studies have also shown that miRNA-183 family, in different pathways, affects the neurosensory ...miRNA-183 family, in normal biology, is expressed in a harmonious and stable manner in the neurosensory organs and cells. Studies have also shown that miRNA-183 family, in different pathways, affects the neurosensory development, maintenance, survival and function. In addition, it has potential neuroprotective effects in response to neurosensory destructive stimulations. miRNA-96 mutation causes hereditary deafness in humans and mice, and therefore affects the inner ear activity and its maintenance. Certain roles have been identified for mi R-96 in the maintenance and function of the inner ear. The comparison of the target genes of family-183 in transcriptomes of newborn and adult hair cells shows that hundreds of target genes in this family may affect development and maintenance of the ears. Identifying the genes that are regulated by miRNA-183 family provides researchers with important information about the complex development and environmental regulation of the inner ear, and can offer new approaches to the maintenance and regeneration of hair cells and auditory nerve.展开更多
Objectives: Recent studies have introduced middle ear volume(MEV) as a novel determinant of perforation-induced conductive hearing loss(CHL) in a mechanism driven by trans-tympanic membrane pressure differences. The p...Objectives: Recent studies have introduced middle ear volume(MEV) as a novel determinant of perforation-induced conductive hearing loss(CHL) in a mechanism driven by trans-tympanic membrane pressure differences. The primary aims of this preliminary report are to: 1) correlate CHL with perforation size; 2) describe the relationship between CHL and MEV; and 3) compare CHL across a range of cholesteatoma involvement.Design: A retrospective pilot study was performed in 31 subjects with audiometry indicative of conductive hearing loss, temporal bone CT scans,and no prior middle ear surgery. Perforation size and MEV were analyzed with respect to CHL in a cohort of 10 perforated ears with no cholesteatoma. CHLs were compared in 3 groups defined by extent of cholesteatoma involvement.Results: Ears with large and small perforations showed mean ABG values of 32.0 ± 15.7 dB and 16.0 ± 16.4 dB, respectively. A direct relationship was observed between MEV and CHL for ears with large perforations across all frequencies, whereas this relationship for small perforations was frequency-dependent. Finally, a statistically significant increase in CHL was found across ears with increasing cholesteatoma involvement at 1000 Hz(X^2(2) = 9.786, p = 0.008),2000 Hz(x^2(2) = 8.455, p = 0.015),and 4000 Hz(x^2(2)= 8.253, p = 0.016).Conclusions: These pilot data suggest that greater perforation-induced conductive hearing losses may be associated with larger perforation sizes and cholesteatoma. The correlation between MEV and CHL may require additional study.展开更多
Hearing loss is one of the most frequent health issues in industrialized countries. The pathogenesis and molecular mechanisms of hearing loss are still unclear. Histone deacetylases(HDACs) are emerging as key enzymes ...Hearing loss is one of the most frequent health issues in industrialized countries. The pathogenesis and molecular mechanisms of hearing loss are still unclear. Histone deacetylases(HDACs) are emerging as key enzymes in many physiological processes, including chromatin remodeling,regulation of transcription, DNA repair, metabolism, genome stability and protein secretion. Recent studies indicated that HDACs are associated with the development and progression of hearing loss. Dysfunction of HDACs could promote the oxidative stress and aging in the inner ear. In light of considering the current stagnation in the development of therapeutic options, the need for new strategies in the treatment of hearing loss has never been so pressing. In this review, we will summarize the reported literatures for HDACs in hearing loss and discuss how HDAC family members show different performances for the possibility of process of diseases development. The possibility of pharmacological intervention on hearing loss opens a novel path in the treatment of hearing loss.展开更多
Objective: To understand the crucial role of the klotho gene in hearing development in mouse models.Methods: PCR was used to identify CBA mice with different genotypes, i.e. WT, heterozygous(klotho +/-)or homozygous(k...Objective: To understand the crucial role of the klotho gene in hearing development in mouse models.Methods: PCR was used to identify CBA mice with different genotypes, i.e. WT, heterozygous(klotho +/-)or homozygous(klotho-/-). Mice phenotype and weight were recorded postnatal 25 days(P-25) and auditory brainstem responses(ABR) were used to determine auditory function at P-60.Results: klotho-/-mice tended to have smaller size, lighter weight and higher ABR thresholds at P-60,showing early onset age-related hearing loss(ARHL).Conclusion: Heterozygous and homozygous klotho deficient mice exhibit different degrees of hearing loss at young age, with homozygous mice(klotho-/-) showing more severe hearing loss. Our results indicate that persisted expression of klotho protein in the inner ear may potentially delay the onset of ARHL and play an important role in the protection of auditory function.展开更多
We present a very rare case of tophaceous gout of the middle ear causing conductive hearing loss,with special emphasis on Computed Tomography presentation.
Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recover...Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations(right side, incomplete partition type I [IP-I]; left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditoryevestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear.展开更多
In this study,I focused on finding a mean of protecting against hearing loss.By infusing the cochlea with the neurotrophin factor,NT-3 alone or combined treatment with MK 801,a NMDA receptor antagonist I found hearing...In this study,I focused on finding a mean of protecting against hearing loss.By infusing the cochlea with the neurotrophin factor,NT-3 alone or combined treatment with MK 801,a NMDA receptor antagonist I found hearing loss was attenuated and spiral ganglion neuron loss was nearly totally protected indicating that the importance of the combined treatment of NT-3 and NMDA receptor antagonists in the treatment of hearing disorders.展开更多
Hearing plays a vital role in the performance of a soldier and is important for speech processing. Noise-induced hearing loss is a significant impairment in the military and can affect combat performance. Military per...Hearing plays a vital role in the performance of a soldier and is important for speech processing. Noise-induced hearing loss is a significant impairment in the military and can affect combat performance. Military personnel are constantly exposed to high levels of noise and it is not surprising that noise induced hearing loss and tinnitus remain the second most prevalent service-connected disabilities. Unfortunately, unlike civilian personnel, military personnel have little option but to remain in noisy environments in order to complete specific tasks and missions. Use of hearing protection devices and follow-up audiological tests have become the mainstay of prevention of noise-induced hearing loss. This review focuses on sources of noise within the military, pathophysiology and management of patients with noise induced hearing loss.展开更多
<strong>Background:</strong> Preventable otologic conditions have been found to be important health issues among paediatric age group. Poor socioeconomic status and inappropriate management services in dev...<strong>Background:</strong> Preventable otologic conditions have been found to be important health issues among paediatric age group. Poor socioeconomic status and inappropriate management services in developing countries lead to various complications despite the fact that most of these diseases are self-limiting. The aim of this study was to determine the prevalence and pattern of ear diseases in school children of high and low socioeconomic status in Yola, Nigeria. <strong>Methods:</strong> This study was conducted in March 2017 in two primary schools (private and public) in Yola among 6 - 8 years old pupils. Approval for the study was obtained from Federal Medical Centre, Yola and consent was obtained from the parents/guardians and the school authorities. Using a health questionnaire, a brief ENT history was obtained from children in years 1 and 2 of each school. Otoscopic examination, audiometry and tympanometry were then carried out. Data collected was analysed using SPSS version 20. <strong>Results:</strong> One hundred and three children were studied. The age range was 6 - 8 with mean age of 6.8 ± 0.9 and 53 (51.5%) boys. Fifty-two (50.5%) were from public school (low socio-economic class) and 51 (49.5%) from private (high social class). Wax was the most common ear condition encountered affecting 37 (35.9%) children, and the number was similar in both groups. Otitis media with effusion (OME) was encountered in seven children evenly split between the two groups showing a point incidence of 6.8%. There was hearing loss in 13 (12.6%) children, and this was also similar in the two groups. <strong>Conclusion:</strong> Wax impaction was the commonest otologic disease encountered. Our study also showed a point incidence of 6.8% for OME, but there was no statistical difference between the two groups, and neither was there any statistically significant difference between the two groups in any of the other diseases encountered.展开更多
Diabetes mellitus (DM) is a chronic systemic disease characterized by hyperglycemia, with various patho-genic mechanisms. From absolute or relative insulin deficiency, patients with DM often demonstrate vari-ous level...Diabetes mellitus (DM) is a chronic systemic disease characterized by hyperglycemia, with various patho-genic mechanisms. From absolute or relative insulin deficiency, patients with DM often demonstrate vari-ous levels of metabolic disorders. Major clinical manifestations of DM include metabolic disorders, vascu-lar lesions, circulatory disturbances and neurologic complications. Along with advances in DM research, re-ports of DM related tinnitus and hearing impairment have increased continuously. Research on DM related auditory system dysfunction has focused on cochlear microcirculation, cellular homeostasis, genetics and ag-ing. Cochlear microcirculation plays an important role in cochlear physiology and its disorders are associat-ed with many inner ear diseases. Ischemia and subsequent reperfusion seen in cochlear microcirculation dis-orders are important factors in hearing damage. Understanding cochlear microcirculation and structural as well as functional changes in DM patients with hearing loss and their causal factors will help reveal patho-genic mechanisms in diabetic hearing loss and provide new ideas in developing interventions and preventing damages caused by diabetes.展开更多
文摘This case is a 49-year-old female patient who presented with suspected eustachian tube dysfunction symptoms that were refractory to medical treatment. She presented with conductive hearing loss and aural fullness on the left. A physical exam revealed an epitympanic fleshy mass in the middle ear with effusion. Imaging with MRI and CT showed opacification of the epitympanum with surrounding bony demineralization bilaterally left greater than right and significant thinning versus dehiscence of the tegmen mastoideum and tympani on the left with only thinning of the tegmen on the right. An MR temporal bone with and without contrast, demonstrated enhancement of the left middle ear extending to the tegmen and corresponding enhancement of the dura along the floor of the left middle cranial fossa as well as extending along foramen ovale into the infratemporal fossa. There was to a lesser degree similar appearance on the right. A biopsy of the middle ear mass on the left revealed meningioma. This case highlights the need to widen your differential with common symptoms when they are refractory to treatment.
文摘miRNA-183 family, in normal biology, is expressed in a harmonious and stable manner in the neurosensory organs and cells. Studies have also shown that miRNA-183 family, in different pathways, affects the neurosensory development, maintenance, survival and function. In addition, it has potential neuroprotective effects in response to neurosensory destructive stimulations. miRNA-96 mutation causes hereditary deafness in humans and mice, and therefore affects the inner ear activity and its maintenance. Certain roles have been identified for mi R-96 in the maintenance and function of the inner ear. The comparison of the target genes of family-183 in transcriptomes of newborn and adult hair cells shows that hundreds of target genes in this family may affect development and maintenance of the ears. Identifying the genes that are regulated by miRNA-183 family provides researchers with important information about the complex development and environmental regulation of the inner ear, and can offer new approaches to the maintenance and regeneration of hair cells and auditory nerve.
基金supported by the National Institutes of Health under Award Numbers 5T32DC013018-03 and TL1TR001116
文摘Objectives: Recent studies have introduced middle ear volume(MEV) as a novel determinant of perforation-induced conductive hearing loss(CHL) in a mechanism driven by trans-tympanic membrane pressure differences. The primary aims of this preliminary report are to: 1) correlate CHL with perforation size; 2) describe the relationship between CHL and MEV; and 3) compare CHL across a range of cholesteatoma involvement.Design: A retrospective pilot study was performed in 31 subjects with audiometry indicative of conductive hearing loss, temporal bone CT scans,and no prior middle ear surgery. Perforation size and MEV were analyzed with respect to CHL in a cohort of 10 perforated ears with no cholesteatoma. CHLs were compared in 3 groups defined by extent of cholesteatoma involvement.Results: Ears with large and small perforations showed mean ABG values of 32.0 ± 15.7 dB and 16.0 ± 16.4 dB, respectively. A direct relationship was observed between MEV and CHL for ears with large perforations across all frequencies, whereas this relationship for small perforations was frequency-dependent. Finally, a statistically significant increase in CHL was found across ears with increasing cholesteatoma involvement at 1000 Hz(X^2(2) = 9.786, p = 0.008),2000 Hz(x^2(2) = 8.455, p = 0.015),and 4000 Hz(x^2(2)= 8.253, p = 0.016).Conclusions: These pilot data suggest that greater perforation-induced conductive hearing losses may be associated with larger perforation sizes and cholesteatoma. The correlation between MEV and CHL may require additional study.
基金supported by Zhejiang Provincial Medical Technology Fund, China (No. 2015KYB340)Ningbo Municipal Natural Science Grant (No. 2016A610130)
文摘Hearing loss is one of the most frequent health issues in industrialized countries. The pathogenesis and molecular mechanisms of hearing loss are still unclear. Histone deacetylases(HDACs) are emerging as key enzymes in many physiological processes, including chromatin remodeling,regulation of transcription, DNA repair, metabolism, genome stability and protein secretion. Recent studies indicated that HDACs are associated with the development and progression of hearing loss. Dysfunction of HDACs could promote the oxidative stress and aging in the inner ear. In light of considering the current stagnation in the development of therapeutic options, the need for new strategies in the treatment of hearing loss has never been so pressing. In this review, we will summarize the reported literatures for HDACs in hearing loss and discuss how HDAC family members show different performances for the possibility of process of diseases development. The possibility of pharmacological intervention on hearing loss opens a novel path in the treatment of hearing loss.
基金supported by the National Nature Science Foundation of China(81470684,21405130)Postdoctoral Science Foundation of China (2015M571818)+5 种基金Clinical Special Fund of Jiangsu Province (b12014032)Six Major Categories Talent (2014WSN-043,2011-WS-074)Jiangsu Provincial University Fund (16621632)Innovation and Entrepreneurship Training Program for College Students in Jiangsu Province (KYLX14-1455,201610313002Z)Colleges and universities Foundation in Jiangsu Province(16621632,16KJB320016)Nature Science planning Foundation of Xuzhou (KC17087)
文摘Objective: To understand the crucial role of the klotho gene in hearing development in mouse models.Methods: PCR was used to identify CBA mice with different genotypes, i.e. WT, heterozygous(klotho +/-)or homozygous(klotho-/-). Mice phenotype and weight were recorded postnatal 25 days(P-25) and auditory brainstem responses(ABR) were used to determine auditory function at P-60.Results: klotho-/-mice tended to have smaller size, lighter weight and higher ABR thresholds at P-60,showing early onset age-related hearing loss(ARHL).Conclusion: Heterozygous and homozygous klotho deficient mice exhibit different degrees of hearing loss at young age, with homozygous mice(klotho-/-) showing more severe hearing loss. Our results indicate that persisted expression of klotho protein in the inner ear may potentially delay the onset of ARHL and play an important role in the protection of auditory function.
文摘We present a very rare case of tophaceous gout of the middle ear causing conductive hearing loss,with special emphasis on Computed Tomography presentation.
文摘Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations(right side, incomplete partition type I [IP-I]; left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditoryevestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear.
文摘In this study,I focused on finding a mean of protecting against hearing loss.By infusing the cochlea with the neurotrophin factor,NT-3 alone or combined treatment with MK 801,a NMDA receptor antagonist I found hearing loss was attenuated and spiral ganglion neuron loss was nearly totally protected indicating that the importance of the combined treatment of NT-3 and NMDA receptor antagonists in the treatment of hearing disorders.
文摘Hearing plays a vital role in the performance of a soldier and is important for speech processing. Noise-induced hearing loss is a significant impairment in the military and can affect combat performance. Military personnel are constantly exposed to high levels of noise and it is not surprising that noise induced hearing loss and tinnitus remain the second most prevalent service-connected disabilities. Unfortunately, unlike civilian personnel, military personnel have little option but to remain in noisy environments in order to complete specific tasks and missions. Use of hearing protection devices and follow-up audiological tests have become the mainstay of prevention of noise-induced hearing loss. This review focuses on sources of noise within the military, pathophysiology and management of patients with noise induced hearing loss.
文摘<strong>Background:</strong> Preventable otologic conditions have been found to be important health issues among paediatric age group. Poor socioeconomic status and inappropriate management services in developing countries lead to various complications despite the fact that most of these diseases are self-limiting. The aim of this study was to determine the prevalence and pattern of ear diseases in school children of high and low socioeconomic status in Yola, Nigeria. <strong>Methods:</strong> This study was conducted in March 2017 in two primary schools (private and public) in Yola among 6 - 8 years old pupils. Approval for the study was obtained from Federal Medical Centre, Yola and consent was obtained from the parents/guardians and the school authorities. Using a health questionnaire, a brief ENT history was obtained from children in years 1 and 2 of each school. Otoscopic examination, audiometry and tympanometry were then carried out. Data collected was analysed using SPSS version 20. <strong>Results:</strong> One hundred and three children were studied. The age range was 6 - 8 with mean age of 6.8 ± 0.9 and 53 (51.5%) boys. Fifty-two (50.5%) were from public school (low socio-economic class) and 51 (49.5%) from private (high social class). Wax was the most common ear condition encountered affecting 37 (35.9%) children, and the number was similar in both groups. Otitis media with effusion (OME) was encountered in seven children evenly split between the two groups showing a point incidence of 6.8%. There was hearing loss in 13 (12.6%) children, and this was also similar in the two groups. <strong>Conclusion:</strong> Wax impaction was the commonest otologic disease encountered. Our study also showed a point incidence of 6.8% for OME, but there was no statistical difference between the two groups, and neither was there any statistically significant difference between the two groups in any of the other diseases encountered.
基金Projects of Hebei Provincial Administration of Traditional Chinese Medicine,No.2012068
文摘Diabetes mellitus (DM) is a chronic systemic disease characterized by hyperglycemia, with various patho-genic mechanisms. From absolute or relative insulin deficiency, patients with DM often demonstrate vari-ous levels of metabolic disorders. Major clinical manifestations of DM include metabolic disorders, vascu-lar lesions, circulatory disturbances and neurologic complications. Along with advances in DM research, re-ports of DM related tinnitus and hearing impairment have increased continuously. Research on DM related auditory system dysfunction has focused on cochlear microcirculation, cellular homeostasis, genetics and ag-ing. Cochlear microcirculation plays an important role in cochlear physiology and its disorders are associat-ed with many inner ear diseases. Ischemia and subsequent reperfusion seen in cochlear microcirculation dis-orders are important factors in hearing damage. Understanding cochlear microcirculation and structural as well as functional changes in DM patients with hearing loss and their causal factors will help reveal patho-genic mechanisms in diabetic hearing loss and provide new ideas in developing interventions and preventing damages caused by diabetes.