Auditory brain-stem responses in the fragile X syndrome.

Auditory brain-stem responses (ABRs) were recorded from a group of 12 mentally retarded males with the fragile X (fra[X]). The responses were analyzed in terms of ABR thresholds, absolute latencies, and interpeak latencies. One patient had increased ABR thresholds, indicating hearing impairment. Five fra(X) subjects had prolonged I-V interpeak latencies. Comparisons between the fra(X) group (excluding one possible hard-of-hearing subject) and a control group of age-matched males with normal intelligence showed that the fra(X) group's interpeak latencies were significantly prolonged for the III-V and I-V but not for the I-III. This pattern of prolongation of interpeak latencies suggests that central, as opposed to peripheral, nervous-system dysfunction predominates in many patients having this syndrome. In addition, frequently observed prolongation of the transmission time may indicate that brain-stem white-matter functioning is also apt to be involved in this syndrome.     

Chronic effects of phenytoin on brain-stem auditory evoked potentials in man

The effects of phenytoin (PHT) on brain-stem auditory evoked potentials (BAEPs) were studied in 65 epileptic patients who received long-term PHT monotherapy at therapeutic and supra-therapeutic levels with no clinical evidence of brain-stem toxicity. Abnormal BAEPs were found in 7.5% and 33.3% of patients with therapeutic and supra-therapeutic PHT levels respectively. Serum PHT levels had a trend towards a positive relationship with the I–V interpeak latency (IPL), and a significant negative relationship with the amplitudes of waves I and V. at supra-therapeutic levels, both I–V and I–III IPLs were significantly prolonged while at therapeutic evels onl I–III IPLs were prolonged. The absolute latency of wave I was prolonged in both the therapeutic and the supra-therapeutic groups. These results suggest that PHT acts both peripherally on either the auditory nerve or the cochlea, and centrally on brain-stem conduction.     

Evoked potential findings in Behçet's disease. Brain-stem auditory,visual, and somatosensory evoked potentials in 44 patients

We studied 54 patients with Behçet's disease, 41 males and 13 females, mean age 28 years. Forty-four patients had auditory brain-stem evoked potential (BAEP) recordings, 39 had pattern reversal visual evoked potentials (VEP), 27 had median nerve somatosensory evoked potential (SEP) recordings, and 25 tibial nerve SEPs. BAEPs were abnormal in 16 patients (52%) with neurological manifestations and in 4 (31%) without, because of decreased amplitude of wave V, prolonged I–III or III–V interpeak latencies, or uncertain/absent waves III and/or V. Eleven patients (40%) with neurological symptoms and 3 patients (25%) without, had abnormal VEPs. Absent potentials, decreased amplitude, with or without prolonged P100 latency, were found in 75% of the cases, the rest had prolonged P100 latency only. Median SEPs were abnormal in 8 patients (38%) with neurological manifestations. Four patients (21%) had abnormal tibial SEPs. Decreased amplitude with or without mild slowing in central conduction was the predominant SEP abnormality. SEPs were normal in all patients without neurological symptoms. In total, 84% of patients with, and 38% of patients without, neurological symptoms had abnormalities of one or more EP modality.When used cautiously, EP studies in Behçet's disease might be helpful to separate neuro-Behçet from other disorders with similar symptomatology, to disclose subclinical CNS involvement, to evaluate and monitor CNS disease activity, and to provide objective measures of treatment response.     

Diagnostic role of brain-stem auditory evoked potentials in neurobrucellosis

Evoked potential audiometry and brain-stem auditory evoked potentials were evaluated in 15 patients with systemic brucellosis in whom brucella meningitis was suspected clinically. In 8 patients cerebrospinal fluid (CSF) was abnormal with high brucella titre, and evoked potentials were abnormal in all of them. In 7 patients the CSF was normal and evoked potentials were also normal. Brain-stem auditory evoked potential abnormalities were categorised into 4 types: (1) abnormal wave I, (2) abnormal wave V, both irreversible, (3) prolonged I–III interpeak latencies, and (4) prolonged I–V interpeak latencies, both reversible. These findings are of important diagnostic value and correlate well with the clinical features, aetiopathogenesis and final outcome.     

Clinical correlates of brain-stem auditory evoked potential variables in multiple sclerosis. Relation to click polarity

The correlations between clinical signs and BAEP latency, amplitude and dispersion variables were investigated in 98 multiple sclerosis patients. A new dispersion variable, the wave IV–V “shape ratio” (SR IV–V), correlated most strongly with brain-stem signs (i.e., nystagmus). Severely reduced wave IV–V amplitude was frequently found in patients with vertical nystagmus or internuclear ophthalmoplegia, and interpeak latency (IPL) III–V correlated most strongly with cerebellar dysfunction (i.e., ataxia). The results may reflect different localizing ability among the various BAEP variables.The association between ataxia and increased IPL III–V was significantly stronger for BAEP to C clicks than to R clicks. Patients with abnormal BAEPs to one polarity (C or R) but not to the other, had significantly more clinical dysfunction than patients with normal BAEPs to both C and R clicks. Hence, C vs. R discordance may be interpreted to indicate possible brain-stem dysfunction.     

No effects of mobile phone electromagnetic field on auditory brainstem response

The present study investigated the possible effects of the electromagnetic field (EMF) emitted by an ordinary GSM mobile phone (902.4 MHz pulsed at 217 Hz) on brainstem auditory processing. Auditory brainstem responses (ABR) were recorded in 17 healthy young adults, without a mobile phone at baseline, and then with a mobile phone on the ear under EMF‐off and EMF‐on conditions. The amplitudes, latencies, and interwave intervals of the main ABR components (waves I, III, V) were compared among the three conditions. ABR waveforms showed no significant differences due to exposure, suggesting that short‐term exposure to mobile phone EMF did not affect the transmission of sensory stimuli from the cochlea up to the midbrain along the auditory nerve and brainstem auditory pathways. Bioelectromagnetics 31:48–55, 2010. © 2009 Wiley‐Liss, Inc.     

Effects of hypothermia on auditory brain-stem and somatosensory evoked responses. A model of a synaptic and axonal lesion

Auditory nerve brain-stem (ABR) and somatosensory evoked responses (SER) were recorded in cats as body temperature was uniformly lowered from 37 to 27°C. Analysis of the results showed that the alterations in the evoked responses were due to disturbances induced both in axonal propagation and synaptic transmission by the hypothermia. By studying the first wave of the SER, which is solely an axonal event, and by assuming reasonable values for the total synaptic delay and axonal propagation times along the ABR pathway, it was concluded that this lesion model induced an effect on synaptic transmission 1.3–1.7 times greater than that on axonal propagation. There was a strong inverse correlation between wave latency and body temperature, with slightly steeper slopes for the longer latency waves. Wave amplitudes were not correlated with temperature. Furthermore, the wave latencies and amplitudes were generally not dependent on stimulus rate.     

The effect of click rate on latency and interpeak interval of the brain-stem auditory evoked potentials in children from birth to 6 years

Latency and interpeak interval of the brain-stem auditory evoked potentials at different click rates were measured in 80 healthy children from birth to 6 years, and 21 adults. Clicks were presented at 10, 30, 50, 70 and 90/sec, and 70, 40 and 20 db HL. At high stimulus intensity (70 dB SL), all latencies of waves I, III and V and the I–V, I–III and III–V intervals showed a progressive prolongation with increasing repetition rate. The latency- and the interval-rate functions were similar for all age groups but their slopes were slightly steeper in younger than in older. As click rate increased from 10/sec to 90/sec, the latencies of waves I, III and V at different age groups were prolonged by 4–10%, 9–13% and 12–15% respectively, and the intervals of I–V, I–III and III–V were prolonged by 15–16%, 8–16% and 14–24% respectively. The mean increments of wave V latency and I–V interval in different age groups were 0.404–0.575 and 0.332–0.526 msec respectively with increasing click rate from 10 to 50/sec, and 0.697–1.009 and 0.629–0.776 msec respectively with increasing click rate from 10 to 90/sec. The younger the age the larger the absolute increments for all these BAEP parameters, but the increasing rates for a BAEP measure were similar among different age groups, exhibiting no age-dependent differences. The III–V/I–III interval ration in most age groups was increased by 3–10% with increasing click rate from 10 to 90/sec, suggesting that the III–V interval was affected by stimulus rate slightly more than I–III interval.At moderate (40 dB HL) and low (20 dB SL) intensity, all waves and intervals showed similar latency- and interval-rate functions to those at high intensity. This demonstrates that the shifting latencies and interpeak intervals with increasing click rate appeared to be independent of the stimulus intensities.     

Auditory brain-stem evoked potentials in cat after kainic acid induced neuronal loss. I. Superior olivary complex

Auditory brain-stem potentials (ABRs) were studied in cats for up to 45 days after kainic acid had been injected unilaterally or bilaterally into the superior olivary complex (SOC) to produce neuronal destruction while sparing fibers of passage and the terminals of axons of extrinsic origin connecting to SOC neurons. The components of the ABR in cat were labeled by their polarity at the vertex (P, for positive) and their order of appearance (the arabic numerals 1, 2, etc.). Component P1 can be further subdivided into 2 subcomponents labeled P1a and P1b. The correspondences we have assumed between the ABR components in cat and man are indicated by providing a Roman numeral designation for the human component in parentheses following the feline notation, e.g., P4 (V). With bilateral SOC destruction, there was a significant and marked attenuation of waves P2 (III), P3 (IV), P4 (V), P5 (VI), and the sustained potential shift (SPS) amounting to as much as 80% of preoperative values. Following unilateral SOC destruction the attenuation of many of these same ABR components, in response to stimulation of either ear, was up to 50%. No component of the ABR was totally abolished even when the SOC was lesioned 100% bilaterally. In unilaterally lesioned cats with extensive neuronal loss (> 75%) the latencies of the components beginnign at P3 (IV) were delayed to stimulation of the ear ipsilateral to the injection site but not to stimulation of the ear contralateral to the injection. Binaural interaction components of the ABR were affected in proportion to the attenuation of the ABR. These results are compatible with multiple brain regions contributing to the generation of the components of the ABR beginning with P2 (III) and that components P3 (IV), P4 (V), and P5 (VI) and the sustained potential shift depend particularly on the integrity of the neurons of the SOC bilaterally. The neurons of the lateral subdivision (LSO) and the medial nucleus of the trapezoid body (MNTB) of the SOC have a major role in generating waves P3 (IV) and P4 (V).     

Auditory brain-stem (ABss) and middle latency auditory responses (MLRs) in the prognosis of severely head-injured patients

Auditory brain-stem responses (ABRs) were studied in 66 subjects with severe head trauma. Middle latency responses (MLRs) were also recorded in 22 of them. Patients were carefully selected to avoid conditions such as pre-existing or acute deafness, hypothermia or ethanol intoxication. In order to evaluate the usefulness of potentials in predicting recorery, patients were classified according to the Glasgow Coma Scale (GCS). ABR tracings were classified into 5 groups and MLR into 2 groups. The recovery was good in the presence of a type 1 ABR, poor in the presence of types 3,4 and 5. Concerning type 2 ABR, the outcome is related to the MLR type, and to the presence of an electrophysiological improvement within the first 3 months following trauma. The reliability of ABR and MLR in predicting the outcome of severe head injury appears to be greater than other usually considered clinical and instrumental data (age, GCS, CT scan, EEG).     

Auditory brain-stem evoked potentials to clicks at different presentation rates: estimating maturation of pre-term and full-term neonates

Auditory brain-stem evoked potentials ABEPs were recorded from 57 neonates ranging in gestational age between 27 and 43 weeks. Averages and standard deviations of I, III and V peak latencies, I–V, I–III and III–V inter-peak latency differences (IPLDs), for 10/sec and 55/sec clicks were calculated for each age group. An additional measure, the net effect of increasing stimulus rate (ISR), was calculated by subtracting 10/sec measures from their 55/sec counterparts. Correlations between ABEP measures and subject age were determined.The results of this study demonstrate a significant correlation between gestational age and electrophysiological measures of peripheral, as well as central, conduction: an inverse correlation between age and peak latencies as well as IPLDs. The slope of this correlation was steeper for the higher stimulus rate. The slope of 55/sec measures vs. age was the sum of the respective slopes of 10/sec measures and of ISR.The maturation of 10/sec measures may reflect white matter development, while ISR changes with gestational age represent maturation of synaptic efficacy. Thus, the maturation of 55/sec measures reflects the combined maturation of nerve conduction velocity and synaptic efficacy along the neonatal auditory nerve and brain-stem. This differential evaluation may enable more accurate determination of developmental age of neonates, with respect to total maturation as well as its constituents.     

Electroencephalography in the diagnosis of hydrocephalus in golden hamsters (Mesocricetus auratus)

The golden hamster (Mesocricetus auratus) is a popular laboratory animal and is used in a multitude of behavioural studies. However, it has been shown that it suffers from different forms of hereditary hydrocephalus, which may result in behavioural changes. This prospective study was designed to look into the usefulness of electroencephalography (EEG) measurements in the diagnosis of hydrocephalus in hamsters. The EEGs of the hydrocephalic hamsters were evaluated double-blind and showed a high-voltage slow wave activity, with a fast activity superimposed onto it. This pattern has already been well described in other hydrocephalic species and differed significantly from the EEGs that were obtained from the normal hamsters. It was concluded from our study that a background activity with an amplitude over 50 muV in combination with a frequency of < or =5 Hz was highly indicative of hydrocephalus in young hamsters. We believe that the EEG could be a very useful diagnostic tool in the screening for hydrocephalus in hamsters.     

The assessment of severe head injury by short-latency somatosensory and brain-stem auditory evoked potentials

The relative prognostic value of short-latency somatosensory evoked potentials (SEPs) and brain-stem auditory evoked potentials (BAEPs) was assessed in 35 patients with post-traumatic coma. Analysis of the evoked potentials was restricted to those recorded within the first 4 days following head injury. Abnormal SEPs were defined as an increase in central somatosensory conduction time or an absence of the initial cortical potential following stimulation of either median nerve. Abnormal BAEPs were classified as an increase in the wave I–V interval or the loss of any or all of its 3 most stable components (waves I, III and V) following stimulation of either ear. SEPs reliably both good and bad outcomes. All 17 patients in whom SEPs were graded as normal had a favourable outcome and 15 of 18 patients in whom SEPs were abnormal had an unfavourable outcome. Although abnormal BAEPs were associated with an unfavourable outcome in almost all patients (6 of 7), only 19 of 28 patients with normal BAEPs had a favourable outcome. The finding of normal BAEPs was therefore of little prognostic significance. These results confirm the superiority and greater sensitivity of the SEP in detecting abnormalities of brain function shortly after severe head trauma.     

Auditory brain-stem evoked potentials in cat after kainic acid induced neuronal loss. II. Cochlear nucleus

Auditory brain-stem potentials (ABRs) were studied in cats for up to 6 weeks after kainic acid had been injected unilaterally into the cochlear nucleus (CN) producing extensive neuronal destruction. The ABRcomponents were labeled by the polarity at the vertex (P, for positive) and their order of appearance (the arabic numerals 1,2, etc.). Component P1 can be further subdivided into 2 subcomponents, P1a and P1b. The assumed correspondence between the ABR components in cat and man is indicated by providing human Roman numeral designations in parentheses following the feline notation, e.g., P2 (III). To stimulation of the ear ipsilateral to the injection, the ABR changes consisted of a loss of components P2 (III) and P3 (IV), and an attenuation and prolongation of latency of components P4 (V) and P5 (VI). The sustained potential shift from which the components arose was not affected. Wave P1a (I) was also slightly but significantly attenuated compatible with changes of excitability of nerve VIII in the cochlea secondary to cochlear nucleus destruction. Unexpectedly, to stimulation of the ear contralateral to the injection side, waves P2 (III), P3 (IV), and P4 (V) were also attenuated and delayed in latency but to a lesser degree than to stimulation of the ear ipsilateral to the injection. Changes in binaural interaction of the ABR following cochlear nucleus lesions were similar to those produced in normal animals by introducing a temporal delay of the input to one ear. The results of the present set of studies using kainic acid to induce neuronla loss in auditory pathway when combined with prior lesion and recording experiments suggest that each of the components of the ABR requires the integrity of an anatomically diffuse system comprising a set of neurons, their axons, and the neurons on which they terminate. Disruption of any portion of the system will alter the amplitude and/or the latency of that component.     

Effects of stimulus repetition rate on slow and fast components of auditory brain-stem responses

Effects of stimulus repetition rate on the slow and fast components of the auditory brain-stem response (ABR) were investigated in 10 adult subjects with normal hearing. The ABRs were recorded with click stimuli at repetition rates of 8, 13.3, 23.8, 40 and 90.9/sec and at an intensity level of 55 dB nHL. Power spectral analysis of the averaged responses was performed. Then the responses were divided into a slow component (0–400Hz) and a fast component (400–1500 Hz) by using digital filtering technique. The magnitude of the slow component was little affected with increasing stimulus rate from 8/sec to 90.9/sec, while successive waves of the fast component, including wave V, decreased in amplitude as stimulus rate was increased. The latency of the slow component and each wave of the fast component was prolonged with increasing click rates. The shift of latency became longer in the later waves than in the earlier waves.     

Electroencephalogram and evoked potentials in the primate model of viral encephalitis

Squirrel monkeys with induced canine distemper virus (CDV) encephalitis showed characteristic clinical signs such as seizures or myoclonus, with EEGs showing periodic synchronized discharge (PSD). Histopathologically, there was gliosis and neuronal degeneration diffusely distributed in both the gray and white matters in the subacute phase, the lesions resembling those found in childhood acute viral encephalitis and subacute sclerosing panencephalitis (SSPE). The auditory brain-stem response (ABR) changes were remarkable in the subacute phase, and the recovery of latency was correlated with survival. The visual evoked potential (VEP) abnormalities disappeared in the acute phase and appeared again with delayed latency in the subacute stage. One monkey which did not show clinical signs but had the pathological changes showed the VEP abnormality. These data suggest that the evoked potentials are helpful in judging the prognosis and that this model is useful for the analysis of the pathogenesis of viral encephalitis.     

Inconsistency of auditory middle latency and steady-state responses in infants

Auditory middle latency and steady-state responses (MLR/SSRs) were recorded in normal infants (aged 3 weeks to 28 months) and adults. SSR amplitudes were maximum using stimulus presentation rates near 40 Hz in adults. By contrast, the infant data showed no consistent amplitude maximum across the rates tested (9–59 Hz). With the exception of the brain-stem response wave V to MLR Na deflection, MLR components in infant's responses to 10.85 Hz clicks did not show any consistent pattern. To investigate the hypothesis that the 40 Hz SSR is derived from overlapping of the 10 Hz MLR components, 43.4 Hz SSRs were synthesized from the responses recorded at 10.85 Hz and compared with those recorded at 43.4 Hz. The predictive accuracy of the synthesized 43.4 Hz SSRs was significantly better in adults than in infants. The results of these studies indicate the presence of large age-related differences in the auditory MLR and SSR, and in the relationship between the two responses.     

Change in glucose metabolism with time in hydrocephalic rats

Energy metabolism was studied by measurements of the activity and isozyme pattern of lactate dehydrogenase (LDH) in rats with kaolin-induced hydrocephalus at 1, 2, 4, and 6 weeks after the induction, as well as in normal, control rats. The total LDH activity in hydrocephalic rats was 1.5 times that in controls throughout the examination period. The main LDH isozyme in hydrocephalic rats was LDH 5(M4) at 2 weeks, but the isozyme pattern was the same as in controls at 4 and 6 weeks. These results suggest that the ratio of anaerobic to aerobic glycolysis increases in the acute stage of hydrocephalus and gradually lowers to become similar to that in controls with time.     

Auditory brain-stem responses in Marcus Gunn ptosis

The auditory brain-stem response (ABR) can detect anomalies both in the auditory pathways and in structures adjacent to these pathways. Patients with Duaneś retraction syndrome, associated with hypoplasia of the abducens nerve in the brain-stem, and patients with hemifacial spasm, due to compression of the facial nerve in the brain-stem, have been found to have abnormal ABRs. Marcus Gunn ptosis with ‘jaw winking’ is considered to be due to misconnection of oculomotor, trigeminal and other cranial nerves. Suspecting that perhaps some ‘jaw-winking’ phenomena may be due to detectable brain-stem anomalies we tested 7 patients with Marcus Gunn ptosis. Three of the patients demonstrated abnormal ABRs indicative of pontine pathology.     

Methods for determining auditory evoked brain-stem response thresholds in human newborns

Previous investigators have reported that newborn auditory evoked brain-stem responses (ABRs) are 20–30 dB higher than adult psychophysical thresholds to the same stimuli. These investigators reduced the intensity of the stimulus until they no longer reported an ABR to the stimulus. We adapted 2 widely used psychophysical methods, the up-down-transformed response (UDTR) method and the method of constant stimuli, for ABR threshold determination of human newborns. Response judgments were made blindly. ABR thresholds of healthy normal newborns by both procedures were no more than 10–15 dB higher than adult psychophysical thresholds. The differences between the newborn ABR thresholds we reported and those in the literature were probably explained by different procedures including the method used to estimate adult psychophysical thresholds. The correlations between ABR thresholds and suprathreshold ABR latencies and amplitudes and latency and amplitude/intensity functions were modest at best. In normal newborns suprathreshold ABR measurements are of little value in predicting ABR thresholds.