Serial Auditory-Evoked Potentials in the Diagnosis and Monitoring of a Child with Landau-Kleffner Syndrome

2013 ◽  
Vol 24 (07) ◽  
pp. 564-571 ◽  
Author(s):  
Erin Plyler ◽  
Ashley W. Harkrider

Background: A boy, aged 2 1/2 yr, experienced sudden deterioration of speech and language abilities. He saw multiple medical professionals across 2 yr. By almost 5 yr, his vocabulary diminished from 50 words to 4, and he was referred to our speech and hearing center. Purpose: The purpose of this study was to heighten awareness of Landau-Kleffner syndrome (LKS) and emphasize the importance of an objective test battery that includes serial auditory-evoked potentials (AEPs) to audiologists who often are on the front lines of diagnosis and treatment delivery when faced with a child experiencing unexplained loss of the use of speech and language. Research Design: Clinical report. Results: Interview revealed a family history of seizure disorder. Normal social behaviors were observed. Acoustic reflexes and otoacoustic emissions were consistent with normal peripheral auditory function. The child could not complete behavioral audiometric testing or auditory processing tests, so serial AEPs were used to examine central nervous system function. Normal auditory brainstem responses, a replicable Na and absent Pa of the middle latency responses, and abnormal slow cortical potentials suggested dysfunction of auditory processing at the cortical level. The child was referred to a neurologist, who confirmed LKS. At age 7 1/2 yr, after 2 1/2 yr of antiepileptic medications, electroencephalographic (EEG) and audiometric measures normalized. Presently, the child communicates manually with limited use of oral information. Conclusions: Audiologists often are one of the first professionals to assess children with loss of speech and language of unknown origin. Objective, noninvasive, serial AEPs are a simple and valuable addition to the central audiometric test battery when evaluating a child with speech and language regression. The inclusion of these tests will markedly increase the chance for early and accurate referral, diagnosis, and monitoring of a child with LKS which is imperative for a positive prognosis.

PLoS ONE ◽  
2021 ◽  
Vol 16 (5) ◽  
pp. e0251363
Author(s):  
Dennis McFadden ◽  
Craig A. Champlin ◽  
Michelle H. Pho ◽  
Edward G. Pasanen ◽  
Mindy M. Maloney ◽  
...  

Auditory brainstem responses (ABRs) and auditory middle-latency responses (AMLRs) to a click stimulus were measured in about 100 subjects. Of interest were the sex differences in those auditory evoked potentials (AEPs), the correlations between the various AEP measures, and the correlations between the AEP measures and measures of otoacoustic emissions (OAEs) and behavioral performance also measured on the same subjects. Also of interest was how the menstrual cycle affected the various AEP measures. Most ABR measures and several AMLR measures exhibited sex differences, and many of the former were substantial. The sex differences tended to be larger for latency than for amplitude of the waves, and they tended to be larger for a weak click stimulus than for a strong click. The largest sex difference was for Wave-V latency (effect size ~1.2). When subjects were dichotomized into Non-Whites and Whites, the race differences in AEPs were small within sex. However, sex and race interacted so that the sex differences often were larger for the White subjects than for the Non-White subjects, particularly for the latency measures. Contrary to the literature, no AEP measures differed markedly across the menstrual cycle. Correlations between various AEP measures, and between AEP and OAE measures, were small and showed no consistent patterns across sex or race categories. Performance on seven common psychoacoustical tasks was only weakly correlated with individual AEP measures (just as was true for the OAEs also measured on these subjects). AMLR Wave Pa unexpectedly did not show the decrease in latency and increase in amplitude typically observed for AEPs when click level was varied from 40 to 70 dB nHL (normal Hearing Level). For the majority of the measures, the variability of the distribution of scores was greater for the males than for the females.


2002 ◽  
Vol 13 (07) ◽  
pp. 367-382 ◽  
Author(s):  
Suzanne C. Purdy ◽  
Andrea S. Kelly ◽  
Merren G. Davies

Auditory evoked potentials (AEPs) and behavioral tests were used to evaluate auditory processing in 10 children aged 7 to 11 years who were diagnosed as learning disabled (LD). AEPs included auditory brainstem responses (ABRs), middle latency responses (MLRs), and late cortical responses (P1, N1, P2, P3). Late cortical responses were recorded using an active listening oddball procedure. Auditory processing disorders were suspected in the LD children after a psychologist found phonologic processing and auditory memory problems. A control group of 10 age- and gender-matched children with no hearing or reported learning difficulties was also tested. Teacher ratings of classroom listening and SCAN Competing Words and Staggered Spondaic Word scores were poorer in the LD children. There were minor ABR latency differences between the two groups. Wave Na of the MLR was later and Nb was smaller in the LD group. The main differences in cortical responses were that P1 was earlier and P3 was later and smaller in the LD group.


2009 ◽  
Vol 19 (1) ◽  
pp. 14-21 ◽  
Author(s):  
Suzanne C Purdy ◽  
Kirsty Gardner-Berry

Abstract Auditory evoked potentials can be used to objectively assess hearing sensitivity, central auditory processing, and neural encoding of speech sounds up to the level of the auditory cortex. Evoked potentials have been of interest to clinicians and researchers in the cochlear implant field for a long time because of their potential for objectively predicting cochlear implant outcomes, as well as improving candidacy determination, and implant programming. Neural response telemetry and intra-operative electrical auditory brainstem recording have been routinely performed by implant programs for many years. Recently, there has been great interest in potential clinical applications of cortical auditory evoked potentials in the implant field. Research and clinical applications are reviewed and case studies are presented that illustrate clinical applications of cortical evoked potentials in children before and after implantation.


2017 ◽  
Vol 28 (01) ◽  
pp. 091-101 ◽  
Author(s):  
Vasiliki Vivian Iliadou ◽  
Nikos Eleftheriadis

AbstractClinical importance of auditory processing disorder (APD) testing is often overlooked and regarded with skepticism given the challenging interpretation of results and the current growing debate of its nature and clinical entity.Presentation of this case is highly educational as APD is the single clinical manifestation of a large cerebellopontine and internal auditory canal lesion.A case report.The patient underwent a standard audiological evaluation with normal results. She was referred for APD evaluation. The APD test battery consisted of speech in babble (SinB), dichotic digits (DD), frequency and duration of pattern sequence testing, Random Gap Detection Test, and gaps in noise. These were followed by otoacoustic emissions testing, auditory brainstem responses (ABR) and magnetic resonance imaging (MRI).Her auditory processing results showed deficits in SinB and DD limited to the right ear as well as deficits in temporal processing. Both verbal and nonverbal tests exhibited deficits strictly limited to the right ear, which was in accordance with what she was experiencing as reduced loudness for the incoming sounds on the right ear. This less costly evaluation revealed that there was good reason to assess electrophysiologically the auditory system. ABR showed an abnormal waveform with either missing or severely prolongated wave V (depending on stimulus polarity). Otoacoustic emissions were normal. MRI was then implemented revealing a large cerebellopontine and internal auditory canal lesion.This clinical case stresses the importance of testing for APD with a psychoacoustical test battery despite current debate of lack of a gold standard diagnostic approach to APD. In this case, APD diagnosis led to a cerebellopontine lesion identification with extension to the right internal auditory canal. This rare cause of APD demonstrates the efficiency of the current diagnostic test battery in revealing lesional causes of central APD.


2011 ◽  
Vol 21 (2) ◽  
pp. 50-58
Author(s):  
James W. Hall ◽  
Anuradha R. Bantwal

Early identification and diagnosis of hearing loss in infants and young children is the first step toward appropriate and effective intervention and is critical for optimal communicative and psychosocial development. Limitations of behavioral assessment techniques in pediatric populations necessitate the use of an objective test battery to enable complete and accurate assessment of auditory function. Since the introduction of the cross-check principle 35 years ago, the pediatric diagnostic test battery has expanded to include, in addition to behavioral audiometry, acoustic immittance measures, otoacoustic emissions, and multiple auditory evoked responses (auditory brainstem response, auditory steady state response, and electrocochleography). We offer a concise description of a modern evidence-based audiological test battery that permits early and accurate diagnosis of auditory dysfunction.


Author(s):  
Matt D. Schalles ◽  
Dorian S. Houser ◽  
James J. Finneran ◽  
Peter Tyack ◽  
Barbara Shinn-Cunningham ◽  
...  

AbstractAuditory neuroscience in dolphins has largely focused on auditory brainstem responses; however, such measures reveal little about the cognitive processes dolphins employ during echolocation and acoustic communication. The few previous studies of mid- and long-latency auditory-evoked potentials (AEPs) in dolphins report different latencies, polarities, and magnitudes. These inconsistencies may be due to any number of differences in methodology, but these studies do not make it clear which methodological differences may account for the disparities. The present study evaluates how electrode placement and pre-processing methods affect mid- and long-latency AEPs in (Tursiops truncatus). AEPs were measured when reference electrodes were placed on the skin surface over the forehead, the external auditory meatus, or the dorsal surface anterior to the dorsal fin. Data were pre-processed with or without a digital 50-Hz low-pass filter, and the use of independent component analysis to isolate signal components related to neural processes from other signals. Results suggest that a meatus reference electrode provides the highest quality AEP signals for analyses in sensor space, whereas a dorsal reference yielded nominal improvements in component space. These results provide guidance for measuring cortical AEPs in dolphins, supporting future studies of their cognitive auditory processing.


1997 ◽  
Vol 111 (3) ◽  
pp. 218-222 ◽  
Author(s):  
William W. Qiu ◽  
Shengguang S. Yin ◽  
Fred J. Stucker ◽  
Mardjohan Hardjasudarma

AbstractGlomus tumours involving the middle ear and the cerebellopontine angle are reported with emphasis on audiological findings. Magnetic resonance imaging (MRI), angiographic and pathological results are presented. Audiological tests, including impedance audiometry, evoked otoacoustic emissions and auditory brainstem responses, are valuable in evaluation of the effect of glomus tumours on the auditory system as well as their pathological extent.


2016 ◽  
Author(s):  
Εμμανουήλ Νησωτάκης

Εισαγωγή Η συγγενής βαρηκοΐα είναι ίσως η πιο συχνή αισθητηριακή βλάβη της νεογνικής ηλικίας. Τα νοσηλευόμενα στη ΜΕΝΝ νεογνά θεωρείται ότι διατρέχουν μεγαλύτερο κίνδυνο για εμφάνιση είτε νευροαισθητήριας βαρηκοΐας είτε ακουστικής νευροπάθειας. Η σοβαρότητα της απώλειας ακοής κατά τη διάρκεια της πρώιμης κρίσιμης περιόδου ανάπτυξης του λόγου καθορίζει την απόκτηση επαρκούς γλωσσικής ικανότητας γι’ αυτό και είναι σημαντική η έγκαιρη διάγνωση και αντιμετώπιση κάθε υποκείμενης ακουστικής διαταραχής.Στη μελέτη αυτή εστιάζουμε στην παρουσία ακοολογικών παραγόντων κινδύνου και προσπαθούμε να διευκρινίσουμε την συσχέτισή τους με την εμφάνιση συγγενούς βαρηκοΐας, συμπεριλαμβανομένης και της ακουστικής νευροπάθειας, στον πληθυσμό των νεογνών της ΜΕΝΝ του νοσοκομείου μας.Ασθενείς και ΜέθοδοςΣτην παρούσα προοπτική μελέτη κοόρτης μελετήθηκαν 453 νεογνά τα οποία εισήχθησαν στη ΜΕΝΝ για περισσότερο από 24 ώρες μεταξύ 2012 και 2016 και εμφάνιζαν έναν ή περισσότερους παράγοντες κινδύνου. Όλα τα νεογνά υποβλήθηκαν σε ακοολογικό έλεγχο με χρήση αυτοματοποιημένων προκλητών δυναμικών του εγκεφαλικού στελέχους (automated Auditory Brainstem Responses – aABRs) σε συνδυασμό με παροδικά προκλητές ωτοακουστικές εκπομπές (Transiently Evoked OtoAcoustic Emissions – TEOAEs) τουλάχιστον 48 ώρες μετά τη γέννησή τους. Τα νεογνά που απέτυχαν στην αρχική εξέταση επανεξετάζονταν πριν τον πρώτο μήνα ζωής κι επί αποτυχίας παραπέμπονταν στο ιατρείο παιδικής βαρηκοΐας για πλήρη ακοολογικό έλεγχο. ΑποτελέσματαΑπό τον συνολικό αριθμό των 453 νεογνών 71 απέτυχαν στον αρχικό ακοολογικό έλεγχο. Μόνο 49 προσήλθαν για επανεξέταση και τελικά 8 νεογνά (1.8%) απέτυχαν τόσο στα a-ABRs όσο και στις TEOAEs και διαγνώσθηκαν με πιθανή νευροαισθητήρια βαρηκοΐα, ενώ σε επίσης 8 νεογνά (1.8%) οι TEOAEs εκλύθηκαν φυσιολογικά αλλά απέτυχαν στα a-ABRs οπότε διαγνώσθηκαν με πιθανή ακουστική νευροπάθεια. Από τους 13 ανεξάρτητους παράγοντες κινδύνου που ελέγχθηκαν η ωτοτοξικότητα των χορηγούμενων φαρμάκων, η μηχανική υποστήριξη της αναπνοής, το χαμηλό βάρος γέννησης και η υπερχολερυθριναιμία ήταν οι πιο συχνά εμφανιζόμενοι. Η πλειοψηφία των νεογνών (48%) εμφάνιζε ταυτόχρονα δύο παράγοντες κινδύνου. Το χαμηλό βάρος γέννησης βρέθηκε να συσχετίζεται με την εμφάνιση πιθανής νευροαισθητήριας βαρηκοΐας (p=0.002), ενώ οι κρανιοπροσωπικές ανωμαλίες και οι λοιμώξεις της ομάδας TORCH παρουσίασαν στατιστικά σημαντική συμμετοχή (p=0.03 και p=0.05 αντίστοιχα) στην ανάπτυξη πιθανής απώλειας ακοής συμπεριλαμβανομένης και της ακουστικής νευροπάθειας.ΣυμπεράσματαΤα προκλητά δυναμικά του εγκεφαλικού στελέχους (ABRs) και οι ωτοακουστικές εκπομπές (OAEs) αποτελούν τους θεμέλιους λίθους του καθολικού νεογνικού ελέγχου ακοής στη ΜΕΝΝ καθώς σημαντικός αριθμός παιδιών μπορεί να πάσχει από ακουστική νευροπάθεια. Η συνεχής επανεξέταση των παραγόντων κινδύνου και της συμβολής τους στην εμφάνιση βαρηκοΐας κρίνεται απαραίτητη καθώς αυτοί μεταβάλλονται. Απαιτείται ένα αποδοτικό σύστημα γραμματειακής υποστήριξης προκειμένου να ελαττωθεί το ποσοστό των νεογνών που δεν προσέρχονται για επανεξέταση.


Author(s):  
Pamela Papile Lunardelo ◽  
Marisa Tomoe Hebihara Fukuda ◽  
Patricia Aparecida Zuanetti ◽  
Ângela Cristina Pontes-Fernandes ◽  
Marita Iannazzo Ferretti ◽  
...  

2010 ◽  
Vol 68 (4) ◽  
pp. 528-534 ◽  
Author(s):  
Carla Gentile Matas ◽  
Sandro Luiz de Andrade Matas ◽  
Caroline Rondina Salzano de Oliveira ◽  
Isabela Crivellaro Gonçalves

Multiple sclerosis (MS) is an inflammatory, demyelinating disease that can affect several areas of the central nervous system. Damage along the auditory pathway can alter its integrity significantly. Therefore, it is important to investigate the auditory pathway, from the brainstem to the cortex, in individuals with MS. OBJECTIVE: The aim of this study was to characterize auditory evoked potentials in adults with MS of the remittent-recurrent type. METHOD: The study comprised 25 individuals with MS, between 25 and 55 years, and 25 age- and gender-matched healthy controls (research and control groups). Subjects underwent audiological and electrophysiological evaluations. RESULTS: Statistically significant differences were observed between the groups regarding the results of the auditory brainstem response and the latency of the Na and P300 waves. CONCLUSION: Individuals with MS present abnormalities in auditory evoked potentials indicating dysfunction of different regions of the central auditory nervous system.


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