Pub Date : 2012-08-21DOI: 10.3109/1651386X.2012.709352
A. Ciorba, C. Aimoni, R. Bovo
Abstract Background: Associations have been reported between diabetes mellitus and sensorineural hearing loss. Nonetheless, at the present time, the pathophysiological relationship between these two conditions is still unclear. Diabetes mellitus is a metabolic disease that produces complications of vascular and neurological malfunction in those with the disorder; possible microvascular involvement of the inner ear has been proposed in order to explain this pathophysiological relationship. Objective and Methods: The purpose of this paper is to examine the relationship between diabetes mellitus and sensorineural hearing loss, through a PubMed database systematic review. Results and Conclusion: The association between diabetes and hearing loss still remains controversial. Large cohort studies are necessary in order to evaluate if diabetes mellitus causes specific changes in the inner ear, due to microvascular disease.
{"title":"Hearing loss and diabetes mellitus: Evidences of cochlear microangiopathy?","authors":"A. Ciorba, C. Aimoni, R. Bovo","doi":"10.3109/1651386X.2012.709352","DOIUrl":"https://doi.org/10.3109/1651386X.2012.709352","url":null,"abstract":"Abstract Background: Associations have been reported between diabetes mellitus and sensorineural hearing loss. Nonetheless, at the present time, the pathophysiological relationship between these two conditions is still unclear. Diabetes mellitus is a metabolic disease that produces complications of vascular and neurological malfunction in those with the disorder; possible microvascular involvement of the inner ear has been proposed in order to explain this pathophysiological relationship. Objective and Methods: The purpose of this paper is to examine the relationship between diabetes mellitus and sensorineural hearing loss, through a PubMed database systematic review. Results and Conclusion: The association between diabetes and hearing loss still remains controversial. Large cohort studies are necessary in order to evaluate if diabetes mellitus causes specific changes in the inner ear, due to microvascular disease.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"132 1","pages":"105 - 108"},"PeriodicalIF":0.0,"publicationDate":"2012-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74207183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-08-21DOI: 10.3109/1651386X.2012.707352
W. Neary, G. Lightfoot
Abstract The majority of babies with auditory neuropathy spectrum disorder (ANSD) spend 48 h or more in the Special Care Baby Unit (SCBU), and the current UK recommendations for the Newborn Hearing Screening Programme state that babies admitted to the SCBU should undergo hearing screening with transient evoked otoacoustic emissions together with automated auditory brainstem response audiometry, in order to identify babies affected with the condition. Current recommendations propose that individuals affected with ANSD should be considered candidates for cochlear implantation. It has been suggested that in patients with ANSD, the presence or absence of cortical electric response audiometry (CERA) responses can help to predict favourable or unfavourable prognosis in auditory language development and comprehension post implantation. We describe two individuals who had not been in SCBU, but came from the well baby population yet satisfied the diagnostic criteria for ANSD. These two patients underwent cochlear implantation, but made poor progress in auditory language development and speech understanding on subsequent follow-up. We suggest that ANSD should be considered in all cases of infants who undergo cochlear implantation but do not make subsequent good progress in auditory language development and speech understanding. We further suggest that CERA be performed prior to cochlear implantation in patients satisfying the diagnostic criteria for ANSD, as the results can assist in predicting post implantation progress in auditory language development and speech understanding.
{"title":"Auditory neuropathy spectrum disorder: Examples of poor progress following cochlear implantation","authors":"W. Neary, G. Lightfoot","doi":"10.3109/1651386X.2012.707352","DOIUrl":"https://doi.org/10.3109/1651386X.2012.707352","url":null,"abstract":"Abstract The majority of babies with auditory neuropathy spectrum disorder (ANSD) spend 48 h or more in the Special Care Baby Unit (SCBU), and the current UK recommendations for the Newborn Hearing Screening Programme state that babies admitted to the SCBU should undergo hearing screening with transient evoked otoacoustic emissions together with automated auditory brainstem response audiometry, in order to identify babies affected with the condition. Current recommendations propose that individuals affected with ANSD should be considered candidates for cochlear implantation. It has been suggested that in patients with ANSD, the presence or absence of cortical electric response audiometry (CERA) responses can help to predict favourable or unfavourable prognosis in auditory language development and comprehension post implantation. We describe two individuals who had not been in SCBU, but came from the well baby population yet satisfied the diagnostic criteria for ANSD. These two patients underwent cochlear implantation, but made poor progress in auditory language development and speech understanding on subsequent follow-up. We suggest that ANSD should be considered in all cases of infants who undergo cochlear implantation but do not make subsequent good progress in auditory language development and speech understanding. We further suggest that CERA be performed prior to cochlear implantation in patients satisfying the diagnostic criteria for ANSD, as the results can assist in predicting post implantation progress in auditory language development and speech understanding.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"29 1","pages":"143 - 150"},"PeriodicalIF":0.0,"publicationDate":"2012-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81143638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-08-21DOI: 10.3109/1651386X.2012.707354
Jennifer McCullagh, F. Musiek, J. Shinn
Abstract Objective: The ability to hear in background noise is related to the processing of the incoming acoustic signal in the peripheral auditory system as well as the central auditory nervous system (CANS). Electrophysiological tests have the ability to demonstrate the underlying neural integrity of the CANS, but to date a lack of literature exists demonstrating the effects of background noise on auditory cortical potentials. Therefore, the purpose of this investigation was to systematically investigate the effects of white noise on tone burst-evoked late auditory evoked potentials (N1, P2, and P3) in normal hearing young adults. Study Design: Twenty young-adult normal-hearing individuals served as subjects. A comparison of the late auditory evoked potentials (N1, P2, and P3) was made at multiple signal-to-noise ratios (SNRs) (quiet, + 20, + 10, 0). N1, P2, and P3 were elicited and both amplitude and latency were measured for each of the potentials. A standard oddball paradigm with binaural stimulation was used to evoke the potentials. Repeated Measures Analyses of Variance (ANOVA) were conducted for both the experimental factors of amplitude and latency with within subjects factors of condition (quiet, + 20, + 10, 0). Results: Results indicated no significant differences in N1, P2, or P3 amplitude or latency between the quiet and + 20 SNR condition; however, at poorer SNRs significant N1, P2, and P3 amplitude and/or latency differences were observed. Conclusion: The results indicate a change in higher-order neural function related to the presence of increased noise in the environment.
{"title":"Auditory Cortical Processing in Noise in Normal-Hearing Young Adults","authors":"Jennifer McCullagh, F. Musiek, J. Shinn","doi":"10.3109/1651386X.2012.707354","DOIUrl":"https://doi.org/10.3109/1651386X.2012.707354","url":null,"abstract":"Abstract Objective: The ability to hear in background noise is related to the processing of the incoming acoustic signal in the peripheral auditory system as well as the central auditory nervous system (CANS). Electrophysiological tests have the ability to demonstrate the underlying neural integrity of the CANS, but to date a lack of literature exists demonstrating the effects of background noise on auditory cortical potentials. Therefore, the purpose of this investigation was to systematically investigate the effects of white noise on tone burst-evoked late auditory evoked potentials (N1, P2, and P3) in normal hearing young adults. Study Design: Twenty young-adult normal-hearing individuals served as subjects. A comparison of the late auditory evoked potentials (N1, P2, and P3) was made at multiple signal-to-noise ratios (SNRs) (quiet, + 20, + 10, 0). N1, P2, and P3 were elicited and both amplitude and latency were measured for each of the potentials. A standard oddball paradigm with binaural stimulation was used to evoke the potentials. Repeated Measures Analyses of Variance (ANOVA) were conducted for both the experimental factors of amplitude and latency with within subjects factors of condition (quiet, + 20, + 10, 0). Results: Results indicated no significant differences in N1, P2, or P3 amplitude or latency between the quiet and + 20 SNR condition; however, at poorer SNRs significant N1, P2, and P3 amplitude and/or latency differences were observed. Conclusion: The results indicate a change in higher-order neural function related to the presence of increased noise in the environment.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"46 1","pages":"114 - 121"},"PeriodicalIF":0.0,"publicationDate":"2012-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76151613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-08-21DOI: 10.3109/1651386X.2012.708986
A. Dabbous
Abstract Background: Hypersensitivity to loud sounds is commonly noticed in children with autism spectrum disorders. Objective and methods: This was a cross-sectional study that included 50 children, ages ranging from 1.5 to 3.33 years, divided into a study group of 25 autistic children with normal hearing and a control group of 25 normal hearing healthy children. The aim of this research was to the assess any abnormality in the loudness growth function objectively using auditory brainstem response (ABR), as well as to detect any ABR abnormalities in normal hearing autistic children with delayed language development. Results: Forty-four percent of normal hearing autistic children showed a lower threshold compared to healthy controls. They also showed significantly delayed wave III, but within-normal wave V, and consequently a longer inter-peak interval (IPI): I–III and shorter IPI: III–V, reflecting retro-cochlear dysfunction that may be related to their difficulty in communication. The mean slope of wave V latency intensity curve did not differ between autism and their controls, reflecting normal loudness growth. Male autistic children showed statistically significant longer latencies of wave V than females except at high intensities, but there was no statistically significant difference between them with regard to the mean slope of wave V latency intensity curve. Conclusion: Autistic children with normal hearing showed a within-normal loudness growth indicating that their abnormal reactions to sounds may either be phonophobia, an efferent system affection or a more central pathology that needs further evaluation. They also showed a retro-cochlear dysfunction that may be related to their difficulty in communication.
{"title":"Characteristics of auditory brainstem response latencies in children with autism spectrum disorders","authors":"A. Dabbous","doi":"10.3109/1651386X.2012.708986","DOIUrl":"https://doi.org/10.3109/1651386X.2012.708986","url":null,"abstract":"Abstract Background: Hypersensitivity to loud sounds is commonly noticed in children with autism spectrum disorders. Objective and methods: This was a cross-sectional study that included 50 children, ages ranging from 1.5 to 3.33 years, divided into a study group of 25 autistic children with normal hearing and a control group of 25 normal hearing healthy children. The aim of this research was to the assess any abnormality in the loudness growth function objectively using auditory brainstem response (ABR), as well as to detect any ABR abnormalities in normal hearing autistic children with delayed language development. Results: Forty-four percent of normal hearing autistic children showed a lower threshold compared to healthy controls. They also showed significantly delayed wave III, but within-normal wave V, and consequently a longer inter-peak interval (IPI): I–III and shorter IPI: III–V, reflecting retro-cochlear dysfunction that may be related to their difficulty in communication. The mean slope of wave V latency intensity curve did not differ between autism and their controls, reflecting normal loudness growth. Male autistic children showed statistically significant longer latencies of wave V than females except at high intensities, but there was no statistically significant difference between them with regard to the mean slope of wave V latency intensity curve. Conclusion: Autistic children with normal hearing showed a within-normal loudness growth indicating that their abnormal reactions to sounds may either be phonophobia, an efferent system affection or a more central pathology that needs further evaluation. They also showed a retro-cochlear dysfunction that may be related to their difficulty in communication.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"19 1","pages":"122 - 131"},"PeriodicalIF":0.0,"publicationDate":"2012-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84242659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-08-21DOI: 10.3109/1651386X.2012.707356
V. Manchaiah, D. Stephens, T. Lunner
Abstract Objective: The aim of this short paper is to highlight the implications of information provision about prognosis given to sudden sensorineural hearing loss (SSNHL) patients. Method: Semi-structured interviews were conducted with four SSNHL patients to develop the patient journey model that is published in our previous paper (12). In this study the implications from general, ethical and legal perspectives about the information provision were considered (i.e. discussion with experts and the use of relevant literature). Results: Three out of four patients interviewed reported that their doctors (both general practitioners and ENT specialists) gave false hopes about prognosis. From the preliminary data it appears that there is considerable variability in the views expressed by patients about preference in information provision. However, this issue needs consideration as the information provided by professionals may have a serious impact on service provision and outcome. Conclusions: It is our view that even though, in some instances, it may appear that false hope provides short-term psychological benefit to patients, providing full and honest information is necessary for general (i.e. to facilitate patient journey process), ethical and legal reasons.
{"title":"Information about the prognosis given to sudden sensorineural hearing loss patients: Implications to ‘patient journey’ process","authors":"V. Manchaiah, D. Stephens, T. Lunner","doi":"10.3109/1651386X.2012.707356","DOIUrl":"https://doi.org/10.3109/1651386X.2012.707356","url":null,"abstract":"Abstract Objective: The aim of this short paper is to highlight the implications of information provision about prognosis given to sudden sensorineural hearing loss (SSNHL) patients. Method: Semi-structured interviews were conducted with four SSNHL patients to develop the patient journey model that is published in our previous paper (12). In this study the implications from general, ethical and legal perspectives about the information provision were considered (i.e. discussion with experts and the use of relevant literature). Results: Three out of four patients interviewed reported that their doctors (both general practitioners and ENT specialists) gave false hopes about prognosis. From the preliminary data it appears that there is considerable variability in the views expressed by patients about preference in information provision. However, this issue needs consideration as the information provided by professionals may have a serious impact on service provision and outcome. Conclusions: It is our view that even though, in some instances, it may appear that false hope provides short-term psychological benefit to patients, providing full and honest information is necessary for general (i.e. to facilitate patient journey process), ethical and legal reasons.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"2 1","pages":"109 - 113"},"PeriodicalIF":0.0,"publicationDate":"2012-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88746371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-06-01DOI: 10.3109/1651386x.2012.672011
submission deadline: August 19, 2012
投稿截止日期:2012年8月19日
{"title":"16th International Symposium in Audiological Medicine of IAPA","authors":"","doi":"10.3109/1651386x.2012.672011","DOIUrl":"https://doi.org/10.3109/1651386x.2012.672011","url":null,"abstract":"submission deadline: August 19, 2012","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"54 1","pages":"104 - 99"},"PeriodicalIF":0.0,"publicationDate":"2012-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86513017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-05-18DOI: 10.3109/1651386X.2012.673755
S. Olsen, Lotte Hagen Hernvig, L. Nielsen
Background: Individuals with unilateral sensorineural hearing loss (USNHL) have difficulties understanding speech from the affected side, perceiving speech in noisy environments and localizing sounds, but the degree of reported participation restrictions varies across the population. Objective: To explore disabilities among adults with USNHL compared to subjects with good hearing, to clarify whether hearing performance differs according to age or gender, whether performance is dependent on the side affected, and if gradual or sudden onset has an effect. Study design: The Speech, Spatial and Qualities of Hearing Scale (SSQ) questionnaire was sent to the subjects together with a dedicated questionnaire on age, gender, handedness, onset, duration and cause of hearing impairment, and hearing instrument (HI) use. Study sample: Ninety-eight subjects with USNHL and a reference group of 89 normal-hearing subjects. Results: No association was found between age and median SSQ ratings. No difference was seen between ratings by female and male subjects. No effect of side of USNHL or onset type was seen. All ratings in the three SSQ sections were higher in the reference group compared to those of the USNHL group. Speech in difficult situations and localization were the factors most affected by USNHL. Conclusions: Age, gender, USNHL onset type and handedness side had no effect on the SSQ scores. Speech recognition in difficult situations, localization, and the estimation of distance from sound sources were rated worse by the USNHL group than by the normal hearing reference group. Subjects from the USNHL group rated speech understanding in easy environments and sound quality no differently from those in the normal hearing reference group.
{"title":"Self-reported hearing performance among subjects with unilateral sensorineural hearing loss","authors":"S. Olsen, Lotte Hagen Hernvig, L. Nielsen","doi":"10.3109/1651386X.2012.673755","DOIUrl":"https://doi.org/10.3109/1651386X.2012.673755","url":null,"abstract":"Background: Individuals with unilateral sensorineural hearing loss (USNHL) have difficulties understanding speech from the affected side, perceiving speech in noisy environments and localizing sounds, but the degree of reported participation restrictions varies across the population. Objective: To explore disabilities among adults with USNHL compared to subjects with good hearing, to clarify whether hearing performance differs according to age or gender, whether performance is dependent on the side affected, and if gradual or sudden onset has an effect. Study design: The Speech, Spatial and Qualities of Hearing Scale (SSQ) questionnaire was sent to the subjects together with a dedicated questionnaire on age, gender, handedness, onset, duration and cause of hearing impairment, and hearing instrument (HI) use. Study sample: Ninety-eight subjects with USNHL and a reference group of 89 normal-hearing subjects. Results: No association was found between age and median SSQ ratings. No difference was seen between ratings by female and male subjects. No effect of side of USNHL or onset type was seen. All ratings in the three SSQ sections were higher in the reference group compared to those of the USNHL group. Speech in difficult situations and localization were the factors most affected by USNHL. Conclusions: Age, gender, USNHL onset type and handedness side had no effect on the SSQ scores. Speech recognition in difficult situations, localization, and the estimation of distance from sound sources were rated worse by the USNHL group than by the normal hearing reference group. Subjects from the USNHL group rated speech understanding in easy environments and sound quality no differently from those in the normal hearing reference group.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"61 1","pages":"83 - 92"},"PeriodicalIF":0.0,"publicationDate":"2012-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73346891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-05-18DOI: 10.3109/1651386X.2012.686165
Alpana Kulkarni, K. Rajput, E. Raglan, D. Abrams, M. Bitner-Glindzicz
Abstract Jervell and Lange-Nielsen syndrome is a rare autosomal recessive condition characterized by bilateral profound sensorineural hearing loss, and prolonged QT interval that may result in ventricular tachycardia leading to syncope, cardiac arrest or sudden death. Treatment of the arrhythmias is often life-saving as this condition has a high mortality if untreated. We describe 3 cases of Jervell and Lange-Nielsen syndrome, who presented with profound sensorineural hearing loss and delayed gross motor milestones and have evidence of bilateral vestibular hypofunction on vestibular testing. We suggest that vestibular hypofunction is associated with this syndrome and evaluation of vestibular function may increase the diagnostic yield in the asymptomatic stage of this rare, but severe form of long QT syndrome. Clinicians should consider Jervell Lange-Nielsen in the differential diagnosis of ‘non-syndromic’ profound sensorineural hearing loss presenting with vestibular failure, along with Usher syndrome type 1, and morphological malformations of the vestibular apparatus. However, more studies are needed to confirm these findings.
{"title":"Is gross motor delay secondary to bilateral vestibular hypofunction in Jervell and Lange-Nielsen syndrome?","authors":"Alpana Kulkarni, K. Rajput, E. Raglan, D. Abrams, M. Bitner-Glindzicz","doi":"10.3109/1651386X.2012.686165","DOIUrl":"https://doi.org/10.3109/1651386X.2012.686165","url":null,"abstract":"Abstract Jervell and Lange-Nielsen syndrome is a rare autosomal recessive condition characterized by bilateral profound sensorineural hearing loss, and prolonged QT interval that may result in ventricular tachycardia leading to syncope, cardiac arrest or sudden death. Treatment of the arrhythmias is often life-saving as this condition has a high mortality if untreated. We describe 3 cases of Jervell and Lange-Nielsen syndrome, who presented with profound sensorineural hearing loss and delayed gross motor milestones and have evidence of bilateral vestibular hypofunction on vestibular testing. We suggest that vestibular hypofunction is associated with this syndrome and evaluation of vestibular function may increase the diagnostic yield in the asymptomatic stage of this rare, but severe form of long QT syndrome. Clinicians should consider Jervell Lange-Nielsen in the differential diagnosis of ‘non-syndromic’ profound sensorineural hearing loss presenting with vestibular failure, along with Usher syndrome type 1, and morphological malformations of the vestibular apparatus. However, more studies are needed to confirm these findings.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"69 1","pages":"93 - 98"},"PeriodicalIF":0.0,"publicationDate":"2012-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76350375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-05-18DOI: 10.3109/1651386X.2012.683291
A. Verrotti, M. Laus, G. Loiacono, D. Crescenzi, A. Croce
Autosomal dominant partial epilepsy with auditory features (ADPEAF) is a rare familial partial epilepsy syndrome with onset in the second or third decades of life characterized by recurrent auditory auras and/or other symptoms suggesting a lateral temporal onset. Mutations in the leucine-rich, glioma inactivated 1 gene (LGI1) on chromosome 10q have been identified in approximately 50% of families with ADPEAF. In the literature a total of 25 LGI1 mutations have been described. The mechanisms by which LGI1 mutations cause epilepsy remain unclear. Among the various diagnostic techniques it is important to obtain an electroencephalogram and long-latency auditory evoked potentials. The prognosis is good and the treatment is based on carbamazepine, phenytoin and valproate.
{"title":"Autosomal dominant partial epilepsy with auditory features","authors":"A. Verrotti, M. Laus, G. Loiacono, D. Crescenzi, A. Croce","doi":"10.3109/1651386X.2012.683291","DOIUrl":"https://doi.org/10.3109/1651386X.2012.683291","url":null,"abstract":"Autosomal dominant partial epilepsy with auditory features (ADPEAF) is a rare familial partial epilepsy syndrome with onset in the second or third decades of life characterized by recurrent auditory auras and/or other symptoms suggesting a lateral temporal onset. Mutations in the leucine-rich, glioma inactivated 1 gene (LGI1) on chromosome 10q have been identified in approximately 50% of families with ADPEAF. In the literature a total of 25 LGI1 mutations have been described. The mechanisms by which LGI1 mutations cause epilepsy remain unclear. Among the various diagnostic techniques it is important to obtain an electroencephalogram and long-latency auditory evoked potentials. The prognosis is good and the treatment is based on carbamazepine, phenytoin and valproate.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"2 1","pages":"71 - 75"},"PeriodicalIF":0.0,"publicationDate":"2012-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.3109/1651386X.2012.683291","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72530161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2012-05-18DOI: 10.3109/1651386X.2012.667998
G. Lovell, A. Refaie
Objective: A broad variation in speech perception results has been observed in patients who have received electrical stimulation of the cochlear nucleus complex from auditory brainstem implants (ABIs). This study aims to investigate the factors following implantation that influence speech perception outcomes in both tumour (T) and non-tumour (NT) patient groups by reviewing the current literature and also help explain the reasons behind the broad variation in speech perception outcomes reported. Study design: A search strategy was devised and applied to databases including PubMed, Medline, Google Scholar, CINAHL, and the Cochrane review library to identify relevant cohort studies available. Relevant citations from current publications were also followed up. Papers investigating speech perception outcomes post ABI implantation were sought. Results: Six longitudinal studies were identified that assessed speech perception outcomes in ABI recipients. Meta-analysis was not possible due to variations in methodologies and outcome measures. Qualitative analysis was completed and suggests that speech perception improves for at least the first year post implantation. Conclusions: There is currently limited evidence for the factors post implantation that effect speech perception outcomes, although current literature would suggest that speech perception improves for at least the first year after implantation. Current research also implies that the choice of speech processing strategy may also affect outcomes although further research in this area is required.
{"title":"Speech perception outcomes following auditory brainstem implantation in both tumour and non-tumour patients: A review","authors":"G. Lovell, A. Refaie","doi":"10.3109/1651386X.2012.667998","DOIUrl":"https://doi.org/10.3109/1651386X.2012.667998","url":null,"abstract":"Objective: A broad variation in speech perception results has been observed in patients who have received electrical stimulation of the cochlear nucleus complex from auditory brainstem implants (ABIs). This study aims to investigate the factors following implantation that influence speech perception outcomes in both tumour (T) and non-tumour (NT) patient groups by reviewing the current literature and also help explain the reasons behind the broad variation in speech perception outcomes reported. Study design: A search strategy was devised and applied to databases including PubMed, Medline, Google Scholar, CINAHL, and the Cochrane review library to identify relevant cohort studies available. Relevant citations from current publications were also followed up. Papers investigating speech perception outcomes post ABI implantation were sought. Results: Six longitudinal studies were identified that assessed speech perception outcomes in ABI recipients. Meta-analysis was not possible due to variations in methodologies and outcome measures. Qualitative analysis was completed and suggests that speech perception improves for at least the first year post implantation. Conclusions: There is currently limited evidence for the factors post implantation that effect speech perception outcomes, although current literature would suggest that speech perception improves for at least the first year after implantation. Current research also implies that the choice of speech processing strategy may also affect outcomes although further research in this area is required.","PeriodicalId":88223,"journal":{"name":"Audiological medicine","volume":"33 1","pages":"55 - 63"},"PeriodicalIF":0.0,"publicationDate":"2012-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80156727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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