Objective:To evaluate the audiologists’attitudes and practice towards teleaudiology,as well as to assess the audiological services provided in Egypt and Saudi Arabia during the COVID-19 pandemic.Methods:A cross secti...Objective:To evaluate the audiologists’attitudes and practice towards teleaudiology,as well as to assess the audiological services provided in Egypt and Saudi Arabia during the COVID-19 pandemic.Methods:A cross sectional study was conducted among 112 audiologists who were recruited through convenience sampling.Multinomial logistic regression was used to test the association between practice of tele audiology as a dependent variable and some independent variables.Results:25.4%of the studied sample were practicing tele audiology.Participants’age and attitude toward telemedicine were the independent predictors of tele audiology practice at p value≤0.05.Conclusion:The tele audiology practice is essential.Therefore,raising the knowledge of audiologist about the great value of practicing tele audiology is very important,infrastructure,equipment,and technology especially telecommunication should be improved and facilitated for both audiologist and patients.展开更多
This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a con...This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a condition characterized by damage to the cochlea or the neural pathways that transmit auditory information to the brain.The etiology of SNHL is often complex,involving genetic mutations,prenatal factors,or perinatal insults.Reflexology,an alternative therapy involving the application of pressure to specific points on the feet,is based on the hypothesis that these points correspond to different organs and systems in the body,including the auditory system.However,the biological plausibility and clinical efficacy of foot reflexology in addressing SNHL lack empirical support.This editorial examines the pathophysiology of SNHL,assesses the clinical claims of reflexology practitioners,and emphasizes the necessity of evidence-based approaches in treating infant hearing loss.While complementary therapies may provide ancillary benefits,they should not supplant validated medical treatments in managing SNHL in infants.Further research is needed to evaluate the safety and efficacy of foot reflexology and other alternative therapies in pediatric audiology.展开更多
Purpose Artificial intelligence(AI)is developing across the world in every domain,such as finance,manufacturing,entertainment,agriculture,retail,healthcare,and law.Its use in the education sector has exponentially inc...Purpose Artificial intelligence(AI)is developing across the world in every domain,such as finance,manufacturing,entertainment,agriculture,retail,healthcare,and law.Its use in the education sector has exponentially increased.The present global survey explored the utilization of AI among academicians in audiology and speech-language therapy(ASLT)and their willingness to use AI tools in their academic work.Method This study used a cross-sectional survey design.One hundred and six ASLT academicians participated in the survey(February 2024 to April 2024).The questionnaire contains 18 items,which included a five-point rating scale,yes-no,and open-ended questions.Descriptive statistics were used for analysis.Results Most of the participants were from Asia and North America,followed by Africa.Nearly sixty-eight percent of the academicians used AI tools in their practice.The major concerns reported by the participants were the authenticity of the data,security,the addition of irrelevant information,and incorrect citations.The participants also mentioned that the frequent use of AI tools can reduce a person’s ability to devise novel ideas.AI tools such as ChatGPT,Canva,Grammarly AI,Mentimeter,QuillBot,ResearchRabbit,and Scribd were reported by participants.Conclusions The present study highlights the use of AI tools among ASLT academicians.However,only a few academicians have prior experience in AI courses.This indicates the pressing need for training concerning the appropriate use of AI in academia and support from universities.Furthermore,AI should be incorporated into academia with appropriate monitoring and ethical considerations.展开更多
Despite the increasing investigation into misophonia,its classification remains debated due to symptom overlap with audiological and psychological conditions,as well as methodological limitations in existing studies.I...Despite the increasing investigation into misophonia,its classification remains debated due to symptom overlap with audiological and psychological conditions,as well as methodological limitations in existing studies.In the present study,we compared a clinical sample of adults seeking treatment for misophonia with a non-treatment-seeking sample of age-and gender-matched healthy controls.In these two samples,we examined the psychological and audiological features of misophonia by assessing key psychological processes(anger,disgust,OCD symptoms,anxiety,depression,stress,wellbeing,and psychological inflexibility)and audiological features(hearing,hyperacusis,and tinnitus).We found that individuals with misophonia exhibit higher levels of psychological inflexibility and stress compared to healthy controls.Audiological comparisons indicated that hyperacusis is more prevalent in the misophonia group,with significantly more impairment in social and occupational functioning.There were no differences between groups on an objective assessment of hearing,although self-report measures indicated that individuals with misophonia may have greater difficulty with auditory processing.These findings suggest that misophonia is a complex disorder marked by transdiagnostic psychological characteristics and sound sensitivities.Our results underscore the need for interdisciplinary assessment and treatment approaches that incorporate psychosocial and audiological perspectives.展开更多
The hearing status of children should be examined throughout early childhood,even if they have passed the newborn hearing loss because hearing loss can occur at any time and may affect their ability to learn.Preschool...The hearing status of children should be examined throughout early childhood,even if they have passed the newborn hearing loss because hearing loss can occur at any time and may affect their ability to learn.Preschool hearing screening(PHS)is vital to continue screening throughout early childhood.The current practice of PHS in the primary healthcare centers(PHCs)in Saudi Arabia is unknown.The purpose of this study was to investigate PHS in the PHCs.This cross-sectional descriptive study used an in-person-administered questionnaire to collect data.A total of 106 out of 120 participants(male=61;female=45)representing the PHCs in Riyadh were interviewed.Most of the participants were aged 31–40 years and held a bachelor’s degree as the highest academic qualification with limited years of experience.PHS was mostly performed through subjective measures by asking the parents(71.7%)and the child(65.1%).The audiometric evaluation was conducted for preschool children in only half of the PHCs.General practitioners and nurses usually perform PHS.Substantial gaps in the practice of PHS were identified.The lack of training and appropriate instruments and the need for audiological services were the main challenges.Incorporating robust and objective protocols for PHS into the educational system is a valuable strategy for identifying hearing loss early and reducing its impact through the establishment of effective intervention plans.展开更多
Objective:To investigate if routine audiometry in Bell’s palsy patients has prognostic value.Methods:Retrospective case review was conducted on all Bell’s palsy patients(n紏191)seen at the tertiary otolaryngology sp...Objective:To investigate if routine audiometry in Bell’s palsy patients has prognostic value.Methods:Retrospective case review was conducted on all Bell’s palsy patients(n紏191)seen at the tertiary otolaryngology specialist outpatient clinic from 2015 to 2017.Correlation of ipsilesional audiometric thresholds with patients’time-to-recovery and initial clinical severity(measured by House-Brackmann(HB)scoring)were used for the prognostic outcome measure.Audiometry results were analyzed using three contiguous frequency pure-tone average(1kHz,2kHz,4kHz).Statistical analysis was done via Stata(v13.1),significance tests were 2-sided at 5%significance level.Results:There was no significant difference between audiometric thresholds between the ipsilesional ear and the contralateral ear(p=0.87).Time-to-recovery was significantly longer for patients with severe initial presentation as compared to mild and moderate severity(p<0.01).There was no correlation found between the audiometry results and HB score at presentation(p=0.39).There was no correlation found between ipsilesional audiometric thresholds and time-to-recovery(p=0.58).Conclusion:Our study suggests that routine audiometry has limited prognostic value in Bell’s palsy patients.展开更多
Introduction:Auditory symptoms in individuals with Autistic Spectrum Disorder(ASD)are well described within the neurodevelopmental literature,yet there is minimal mention of ASD in Otolaryngology literature.This is su...Introduction:Auditory symptoms in individuals with Autistic Spectrum Disorder(ASD)are well described within the neurodevelopmental literature,yet there is minimal mention of ASD in Otolaryngology literature.This is surprising considering the potential clinical and diagnostic implications of this link,and the potential for ASD to present to Otolaryngologists in the form of unexplained auditory symptoms.The aims of this literature review were to explore the intersection of auditory symptoms and ASD from the perspective of clinical Otolaryngology,and to outline a clinically focused research agenda based on emerging themes relevant to Otolaryngology.Methods:We searched Pubmed,Embase,Ovid and Cochrane library for studies until November 2021.Four authors independently reviewed 227 publications identified.39 were filtered into the final analysis.The PRISMA 2020 guidelines were followed.The heterogeneity of literature meant that a Systematic Review was not feasible.Included studies were therefore classified thematically,forming the basis of the scoping review.Results:Diagnostic theories for auditory symptoms in ASD include the entire auditory pathway and brain.There is a growing body of literature on auditory symptoms in ASD,suggesting that a primary diagnosis of ASD should be considered in patients presenting with otherwise unexplained auditory symptoms,and indicating a learning need for Otolaryngologists and audiologists,to whom these patients may present.Conclusion:We recommend a research agenda focusing on multidisciplinary collaboration,stakeholder engagement,responsible clinical screening,and clarification of pathophysiological mechanisms and terminology.展开更多
Purpose:Misophonia is not investigated much from an audiological perspective.Our study aims to examine the processing of the auditory retro-cochlear pathways in individuals with misophonia.Methods:A cross-sectional st...Purpose:Misophonia is not investigated much from an audiological perspective.Our study aims to examine the processing of the auditory retro-cochlear pathways in individuals with misophonia.Methods:A cross-sectional study was conducted among university students who had misophonia.The revised Amsterdam Misophonia Scale was used to determine the severity of misophonia.Participants were divided into mild and moderate-severe misophonia and compared with the healthy control group.Auditory Brainstem Response testing was recorded from all the individuals with misophonia.The absolute latency,amplitude,inter-peak latency difference,and inter-rate latency difference were compared between the groups.Results:One-way ANOVA result showed no significant difference in all the parameters of auditory brainstem response between the groups.These results are suggestive of normal brainstem processing in individuals with misophonia.Conclusions:The study concludes that the auditory pathway up to brainstem areas is intact in individuals with misophonia.Further studies are essential on a larger population for generalizing the results.展开更多
Objectives: Auditory neuropathy (AN) is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic ...Objectives: Auditory neuropathy (AN) is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and pathophysiology. Three Chinese pedigrees with familial AN are presented herein to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the Department of Otolaryngology Head and Neck Surgery, China PLA General Hospital. Their family members were studied and the pedigree diagrams were established. History of illness, physical examination,pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion- product otoacoustic emissions (TEOAEs and DPOAEs) were obtained from members of these families. DPOAE changes under the influence of contralateral sound stimuli were observed by presenting a set of continuous white noise to the non - recording ear to exam the function of auditory efferent system. Some subjects received vestibular caloric test, computed tomography (CT)scan of the temporal bone and electrocardiography (ECG) to exclude other possible neuropathy disorders. Results: In most affected subjects, hearing loss of various degrees and speech discrimination difficulties started at 10 to16 years of age. Their audiological evaluation showed absence of acoustic reflex and ABRs. As expected in AN, these subjects exhibited near normal cochlear outer hair cell function as shown in TEOAE & DPOAE recordings. Pure- tone audiometry revealed hearing loss ranging from mild to severe in these patients. Autosomal recessive inheritance patterns were observed in the three families. In Pedigree Ⅰ and Ⅱ, two affected brothers were found respectively, while in pedigree Ⅲ, 2 sisters were affected. All the patients were otherwise normal without evidence of peripheral neuropathy at the time of this writing. Conclusions: In this study, patients with feature of non- syndromic hereditary auditory neuropathy were identified in three Chinese families.Pedigree analysis indicates autosomal recessive inheritances in the pedigrees. The observed inheritance and clinical audiologic findings are different from those previously described for non-syndromic low-frequency sensorineural hearing loss. This information should facilitate future molecular candidate genes screening for understanding the mechanism of AN.展开更多
The objective of this study is to determine the auditory gain, quality of life, audiological benefits, in bone-anchored hearing device users (BAHA). It is a retrospective and concurrent evaluation of thirty patients f...The objective of this study is to determine the auditory gain, quality of life, audiological benefits, in bone-anchored hearing device users (BAHA). It is a retrospective and concurrent evaluation of thirty patients fitted unilaterally and seven fitted bilaterally for at least six months. Patients were assessed with audiometric testing and application of Glasgow Benefit Inventory (GBI) and Abbreviated Profile of Hearing Aid Benefit (APHAB). Regarding sound-field pure audiometry results, we found a statistically significant gain in all frequencies using the bone-anchored device. APHAB scores showed statistically significant subjective audiological gains in all subscales except for the aversiveness subscale. GBI mean scores for all items in both groups were all above 3, suggesting quality of life improvement in conductive and mixed hearing loss patients. BP100 users showed a greater clinical gain in the APHAB global score and subscales compared with Divino users. In conclusion the BAHA provides significant auditory gain, subjective audiological benefits and improves quality of life in all BAHA users. This study shows a significant clinical and statistical benefit of BAHA measured by audiometric testing and by the APHAB and GBI questionnaires.展开更多
Purpose It is essential to investigate the audiological profiles of Williams syndrome in a multicultural context.This study aims to examine the characteristics and management of hearing loss in Chinese children with W...Purpose It is essential to investigate the audiological profiles of Williams syndrome in a multicultural context.This study aims to examine the characteristics and management of hearing loss in Chinese children with Williams syndrome and provide references for future clinical management.Method Between January 2007 and March 2022,families with at least 1 WS patient was recruited from the Newborn Cohort Study of Hearing Loss.Audiological tests were performed,and then appropriate medical management was offered.Furthermore,an overview of the hearing loss phenotype in Williams syndrome in different locations was reviewed.Results A total of two families with at least 1 Williams syndrome patient were recruited from the Newborn Cohort Study of Hearing Loss(ChiCTR2100049765).We identified moderately severe sensorineural or conductive hearing loss that emerged as early as the infancy period in Williams syndrome subjects in Chinese children.Our results extended the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period.We also found that with early diagnosis,proper management,and regular monitoring,children with Williams syndrome could return to a normal or near-normal school life.Conclusions Our study demonstrated the distinct hearing profile in Chinese children with Williams syndrome for the first time.This cohort of WS subjects extends the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period,indicating the importance of clinicians screening and monitoring the hearing status of individuals with WS as early as possible.These data provide references for otolaryngologists and paediatricians to inform the clinical understanding and management of hearing loss in Williams syndrome.展开更多
文摘Objective:To evaluate the audiologists’attitudes and practice towards teleaudiology,as well as to assess the audiological services provided in Egypt and Saudi Arabia during the COVID-19 pandemic.Methods:A cross sectional study was conducted among 112 audiologists who were recruited through convenience sampling.Multinomial logistic regression was used to test the association between practice of tele audiology as a dependent variable and some independent variables.Results:25.4%of the studied sample were practicing tele audiology.Participants’age and attitude toward telemedicine were the independent predictors of tele audiology practice at p value≤0.05.Conclusion:The tele audiology practice is essential.Therefore,raising the knowledge of audiologist about the great value of practicing tele audiology is very important,infrastructure,equipment,and technology especially telecommunication should be improved and facilitated for both audiologist and patients.
文摘This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a condition characterized by damage to the cochlea or the neural pathways that transmit auditory information to the brain.The etiology of SNHL is often complex,involving genetic mutations,prenatal factors,or perinatal insults.Reflexology,an alternative therapy involving the application of pressure to specific points on the feet,is based on the hypothesis that these points correspond to different organs and systems in the body,including the auditory system.However,the biological plausibility and clinical efficacy of foot reflexology in addressing SNHL lack empirical support.This editorial examines the pathophysiology of SNHL,assesses the clinical claims of reflexology practitioners,and emphasizes the necessity of evidence-based approaches in treating infant hearing loss.While complementary therapies may provide ancillary benefits,they should not supplant validated medical treatments in managing SNHL in infants.Further research is needed to evaluate the safety and efficacy of foot reflexology and other alternative therapies in pediatric audiology.
文摘Purpose Artificial intelligence(AI)is developing across the world in every domain,such as finance,manufacturing,entertainment,agriculture,retail,healthcare,and law.Its use in the education sector has exponentially increased.The present global survey explored the utilization of AI among academicians in audiology and speech-language therapy(ASLT)and their willingness to use AI tools in their academic work.Method This study used a cross-sectional survey design.One hundred and six ASLT academicians participated in the survey(February 2024 to April 2024).The questionnaire contains 18 items,which included a five-point rating scale,yes-no,and open-ended questions.Descriptive statistics were used for analysis.Results Most of the participants were from Asia and North America,followed by Africa.Nearly sixty-eight percent of the academicians used AI tools in their practice.The major concerns reported by the participants were the authenticity of the data,security,the addition of irrelevant information,and incorrect citations.The participants also mentioned that the frequent use of AI tools can reduce a person’s ability to devise novel ideas.AI tools such as ChatGPT,Canva,Grammarly AI,Mentimeter,QuillBot,ResearchRabbit,and Scribd were reported by participants.Conclusions The present study highlights the use of AI tools among ASLT academicians.However,only a few academicians have prior experience in AI courses.This indicates the pressing need for training concerning the appropriate use of AI in academia and support from universities.Furthermore,AI should be incorporated into academia with appropriate monitoring and ethical considerations.
基金funded by a grant from the Misophonia Research Fundso Quiet Foundation。
文摘Despite the increasing investigation into misophonia,its classification remains debated due to symptom overlap with audiological and psychological conditions,as well as methodological limitations in existing studies.In the present study,we compared a clinical sample of adults seeking treatment for misophonia with a non-treatment-seeking sample of age-and gender-matched healthy controls.In these two samples,we examined the psychological and audiological features of misophonia by assessing key psychological processes(anger,disgust,OCD symptoms,anxiety,depression,stress,wellbeing,and psychological inflexibility)and audiological features(hearing,hyperacusis,and tinnitus).We found that individuals with misophonia exhibit higher levels of psychological inflexibility and stress compared to healthy controls.Audiological comparisons indicated that hyperacusis is more prevalent in the misophonia group,with significantly more impairment in social and occupational functioning.There were no differences between groups on an objective assessment of hearing,although self-report measures indicated that individuals with misophonia may have greater difficulty with auditory processing.These findings suggest that misophonia is a complex disorder marked by transdiagnostic psychological characteristics and sound sensitivities.Our results underscore the need for interdisciplinary assessment and treatment approaches that incorporate psychosocial and audiological perspectives.
文摘The hearing status of children should be examined throughout early childhood,even if they have passed the newborn hearing loss because hearing loss can occur at any time and may affect their ability to learn.Preschool hearing screening(PHS)is vital to continue screening throughout early childhood.The current practice of PHS in the primary healthcare centers(PHCs)in Saudi Arabia is unknown.The purpose of this study was to investigate PHS in the PHCs.This cross-sectional descriptive study used an in-person-administered questionnaire to collect data.A total of 106 out of 120 participants(male=61;female=45)representing the PHCs in Riyadh were interviewed.Most of the participants were aged 31–40 years and held a bachelor’s degree as the highest academic qualification with limited years of experience.PHS was mostly performed through subjective measures by asking the parents(71.7%)and the child(65.1%).The audiometric evaluation was conducted for preschool children in only half of the PHCs.General practitioners and nurses usually perform PHS.Substantial gaps in the practice of PHS were identified.The lack of training and appropriate instruments and the need for audiological services were the main challenges.Incorporating robust and objective protocols for PHS into the educational system is a valuable strategy for identifying hearing loss early and reducing its impact through the establishment of effective intervention plans.
文摘Objective:To investigate if routine audiometry in Bell’s palsy patients has prognostic value.Methods:Retrospective case review was conducted on all Bell’s palsy patients(n紏191)seen at the tertiary otolaryngology specialist outpatient clinic from 2015 to 2017.Correlation of ipsilesional audiometric thresholds with patients’time-to-recovery and initial clinical severity(measured by House-Brackmann(HB)scoring)were used for the prognostic outcome measure.Audiometry results were analyzed using three contiguous frequency pure-tone average(1kHz,2kHz,4kHz).Statistical analysis was done via Stata(v13.1),significance tests were 2-sided at 5%significance level.Results:There was no significant difference between audiometric thresholds between the ipsilesional ear and the contralateral ear(p=0.87).Time-to-recovery was significantly longer for patients with severe initial presentation as compared to mild and moderate severity(p<0.01).There was no correlation found between the audiometry results and HB score at presentation(p=0.39).There was no correlation found between ipsilesional audiometric thresholds and time-to-recovery(p=0.58).Conclusion:Our study suggests that routine audiometry has limited prognostic value in Bell’s palsy patients.
基金supported by the National Institute for Health ResearchManchester Biomedical Research Centre.
文摘Introduction:Auditory symptoms in individuals with Autistic Spectrum Disorder(ASD)are well described within the neurodevelopmental literature,yet there is minimal mention of ASD in Otolaryngology literature.This is surprising considering the potential clinical and diagnostic implications of this link,and the potential for ASD to present to Otolaryngologists in the form of unexplained auditory symptoms.The aims of this literature review were to explore the intersection of auditory symptoms and ASD from the perspective of clinical Otolaryngology,and to outline a clinically focused research agenda based on emerging themes relevant to Otolaryngology.Methods:We searched Pubmed,Embase,Ovid and Cochrane library for studies until November 2021.Four authors independently reviewed 227 publications identified.39 were filtered into the final analysis.The PRISMA 2020 guidelines were followed.The heterogeneity of literature meant that a Systematic Review was not feasible.Included studies were therefore classified thematically,forming the basis of the scoping review.Results:Diagnostic theories for auditory symptoms in ASD include the entire auditory pathway and brain.There is a growing body of literature on auditory symptoms in ASD,suggesting that a primary diagnosis of ASD should be considered in patients presenting with otherwise unexplained auditory symptoms,and indicating a learning need for Otolaryngologists and audiologists,to whom these patients may present.Conclusion:We recommend a research agenda focusing on multidisciplinary collaboration,stakeholder engagement,responsible clinical screening,and clarification of pathophysiological mechanisms and terminology.
文摘Purpose:Misophonia is not investigated much from an audiological perspective.Our study aims to examine the processing of the auditory retro-cochlear pathways in individuals with misophonia.Methods:A cross-sectional study was conducted among university students who had misophonia.The revised Amsterdam Misophonia Scale was used to determine the severity of misophonia.Participants were divided into mild and moderate-severe misophonia and compared with the healthy control group.Auditory Brainstem Response testing was recorded from all the individuals with misophonia.The absolute latency,amplitude,inter-peak latency difference,and inter-rate latency difference were compared between the groups.Results:One-way ANOVA result showed no significant difference in all the parameters of auditory brainstem response between the groups.These results are suggestive of normal brainstem processing in individuals with misophonia.Conclusions:The study concludes that the auditory pathway up to brainstem areas is intact in individuals with misophonia.Further studies are essential on a larger population for generalizing the results.
基金a grant from the National High Tech Development Project(2001AA221092)and by Beijing Natural Science Foundation(No.7011004)and Beijing Science and Technology Innovation Project(No.H010210160119)grants
文摘Objectives: Auditory neuropathy (AN) is a sensorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and pathophysiology. Three Chinese pedigrees with familial AN are presented herein to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the Department of Otolaryngology Head and Neck Surgery, China PLA General Hospital. Their family members were studied and the pedigree diagrams were established. History of illness, physical examination,pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion- product otoacoustic emissions (TEOAEs and DPOAEs) were obtained from members of these families. DPOAE changes under the influence of contralateral sound stimuli were observed by presenting a set of continuous white noise to the non - recording ear to exam the function of auditory efferent system. Some subjects received vestibular caloric test, computed tomography (CT)scan of the temporal bone and electrocardiography (ECG) to exclude other possible neuropathy disorders. Results: In most affected subjects, hearing loss of various degrees and speech discrimination difficulties started at 10 to16 years of age. Their audiological evaluation showed absence of acoustic reflex and ABRs. As expected in AN, these subjects exhibited near normal cochlear outer hair cell function as shown in TEOAE & DPOAE recordings. Pure- tone audiometry revealed hearing loss ranging from mild to severe in these patients. Autosomal recessive inheritance patterns were observed in the three families. In Pedigree Ⅰ and Ⅱ, two affected brothers were found respectively, while in pedigree Ⅲ, 2 sisters were affected. All the patients were otherwise normal without evidence of peripheral neuropathy at the time of this writing. Conclusions: In this study, patients with feature of non- syndromic hereditary auditory neuropathy were identified in three Chinese families.Pedigree analysis indicates autosomal recessive inheritances in the pedigrees. The observed inheritance and clinical audiologic findings are different from those previously described for non-syndromic low-frequency sensorineural hearing loss. This information should facilitate future molecular candidate genes screening for understanding the mechanism of AN.
文摘The objective of this study is to determine the auditory gain, quality of life, audiological benefits, in bone-anchored hearing device users (BAHA). It is a retrospective and concurrent evaluation of thirty patients fitted unilaterally and seven fitted bilaterally for at least six months. Patients were assessed with audiometric testing and application of Glasgow Benefit Inventory (GBI) and Abbreviated Profile of Hearing Aid Benefit (APHAB). Regarding sound-field pure audiometry results, we found a statistically significant gain in all frequencies using the bone-anchored device. APHAB scores showed statistically significant subjective audiological gains in all subscales except for the aversiveness subscale. GBI mean scores for all items in both groups were all above 3, suggesting quality of life improvement in conductive and mixed hearing loss patients. BP100 users showed a greater clinical gain in the APHAB global score and subscales compared with Divino users. In conclusion the BAHA provides significant auditory gain, subjective audiological benefits and improves quality of life in all BAHA users. This study shows a significant clinical and statistical benefit of BAHA measured by audiometric testing and by the APHAB and GBI questionnaires.
基金supported by the grants of the National Key Research and Development Program of China(Grant No.2023YFC2508400)the National Natural Science Foundation of China(Grant No.82350005).
文摘Purpose It is essential to investigate the audiological profiles of Williams syndrome in a multicultural context.This study aims to examine the characteristics and management of hearing loss in Chinese children with Williams syndrome and provide references for future clinical management.Method Between January 2007 and March 2022,families with at least 1 WS patient was recruited from the Newborn Cohort Study of Hearing Loss.Audiological tests were performed,and then appropriate medical management was offered.Furthermore,an overview of the hearing loss phenotype in Williams syndrome in different locations was reviewed.Results A total of two families with at least 1 Williams syndrome patient were recruited from the Newborn Cohort Study of Hearing Loss(ChiCTR2100049765).We identified moderately severe sensorineural or conductive hearing loss that emerged as early as the infancy period in Williams syndrome subjects in Chinese children.Our results extended the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period.We also found that with early diagnosis,proper management,and regular monitoring,children with Williams syndrome could return to a normal or near-normal school life.Conclusions Our study demonstrated the distinct hearing profile in Chinese children with Williams syndrome for the first time.This cohort of WS subjects extends the reported onset ages of hearing loss in WS from late childhood or early adulthood to the infancy period,indicating the importance of clinicians screening and monitoring the hearing status of individuals with WS as early as possible.These data provide references for otolaryngologists and paediatricians to inform the clinical understanding and management of hearing loss in Williams syndrome.
