Deepak Prasher

Sensory and cognitive event-related potentials in myalgic encephalomyelitis.

Myalgic Encephalomyelitis (ME) is a form of post viral fatigue syndrome resulting in myalgia and fluctuating fatiguability. Symptoms reflecting central nervous system dysfunction are common and include muscle weakness, headache, sensory disturbances, poor short term memory and impairment of concentration. In view of the fact that sensory and cognitive disturbances are experienced by many patients objective evidence was sought with multi-modality sensory evoked potentials and auditory event-related cognitive potentials in a group of ME patients both with and without the enteroviral antigen, VP1 test positive. The auditory brainstem, median nerve somatosensory and pattern reversal checkerboard visual potentials were normal for all 37 patients tested. In contrast to the sensory potentials significant differences in the mean latencies of the cognitive potential N2 and P3 were found. Reaction times were also significantly prolonged but the performance in terms of error was not significantly affected. No significant difference emerged in any of the parameters for the VP1 test. P3 was abnormal in latency or amplitude in 36% of the VP1 positive patients for the frequency discrimination task and 48% for the more difficult duration discrimination task. The abnormalities indicate attentional deficits in some patients and slower speed of information processing in others. The prolonged latencies observed in these patients have not been observed in patients with depression in many other studies.

Neuro-otological abnormalities in Friedreich's ataxia.

Ten patients with an accepted diagnosis of Friedreichs ataxia have been examined neuro-otologically, and oculomotor, vestibular and auditory function assessed. Brainstem auditory evoked potentials (BAEPs) were also recorded. A high incidence of various eye movement disorders was noted. Some of these were indicative of cerebellar dysfunction. Reduced vestibular function and impaired hearing were common to most of the patients. BAEPs were also abnormal in the majority; reasons underlying these abnormalities are discussed. Neuro-otologically, the patients did not constitute an homogeneous group. The findings cast doubt upon the accuracy and validity of the currently accepted criteria for the diagnosis and classification of the spinocerebellar degenerations.

The value of combining auditory brainstem responses and acoustic reflex threshold measurements in neuro-otological diagnosis.

Acoustic reflex thresholds (ART) and auditory brainstem responses (ABR) were measured in 69 patients referred to the Neuro-otological clinic on the suspicion of a retrocochlear lesion. The pure-tone selection criterion was an average hearing loss of less than 60 dB at 0.5, 1, 2, and 4 kHz, with no thresholds exceeding 70 dB and only one threshold exceeding 60 dB. Both tests proved independently to be most effective in the diagnosis of cerebello-pontine angle (CPA) tumours and to a lesser extent for brainstem lesions. The ABR was positive in 100% of CPA group and 90% in the brainstem group compared with 93% and 70% respectively for the ART. The combination of the two tests provides patterns of abnormality which are more disease specific. In the CPA cases the most common and consistent patterns of abnormality were the unilateral delay of Wave V or III and V of the ABR associated with a vertical ART pattern indicative of an abnormality of the ipsi and contralateral reflexes on stimulating the affected side. Intrinsic brainstem lesions most commonly resulted in bilateral ABR abnormalities combined with the full-house ART pattern indicative of bilateral abnormalities of both ipsi and contralateral reflexes.

Intraoperative Monitoring of the Electrocochleogram and the Preservation of Hearing During Acoustic Neuroma Excision

We have monitored the electrocochleogram (ECochG) of 24 patients, using a transtympanic electrode, during acoustic neuroma excision. All patients had unilateral tumours with good preoperative hearing and complete excision was achieved in each case. Of the 24 patients, seven retained some hearing, however, a further two patients had normal ECochG waveforms at the end of operation but were nevertheless deaf. Thus, there is not an invariable correlation between immediate preservation of the ECochG and hearing. As expected, tumour size was important in hearing preservation. Five of seven patients with tumours less than 1.5 cm in diameter retained some hearing after operation, whereas 15 of 17 patients with tumours greater than 1.5 cm in diameter were deaf.

The Effect of Acoustic Neuroma Removal on Hearing in the Contralateral Ear

22 patients with unilateral acoustic neuromas were assessed audiometrically pre- and post-operatively. A high proportion (36%) were found to have a hearing loss of 10 dB or more (6 frequency average) in the contralateral ear in the post-operative period. The evidence points to a cochlear site of the loss, and serial audiograms suggest that the hearing deteriorates in the immediate post-operative period. Follow-up audiometry at three months or more showed that the hearing recovered to pre-operative levels. The possible mechanism of the hearing loss is discussed.

Effectiveness of Acoustic Reflex Threshold Criteria in the Diagnosis of Retrocochlear Pathology

This study examines the acoustic reflex threshold criteria, derived from a large group of patients with cochlear hearing loss proposed by Cohen & Prasher (1992), in order to evaluate their effectiveness in differentiating between cochlear and retro-cochlear lesions. This criterion was tested on 63 patients with surgically confirmed cerebello-pontine angle (CPA) lesions. The false results obtained with this and other criteria were compared. The best balance between the false positive and negative results in the cochlear and retrocochlear group is provided by the criterion of any two adjacent test frequencies (proposed in the companion paper) having an upper limit of 105 dB for hearing below 60 dB and 110 dB for hearing over 60 dB. This study has also singled out the interaural difference criterion as the best diagnostic indicator with the lowest false results with only 1 false negative from 63 CPA cases and 3 false positive cases from 61 cochlear lesions with hearing thresholds over 55 dB.

Preservation of Cochlear Potentials in a Deaf Patient Fifteen Months After Excision of an Acoustic Neuroma

Intraoperative electrocochleographic monitoring has been used in an attempt to protect cochlear nerve function during acoustic neuroma excision. One patient is presented with an apparently intact cochlear nerve and waves N1 and N2 preserved at the end of surgery, but no hearing on subsequent testing. Fifteen months after operation, cochlear microphonics and the summating potential were still present, but N1 and N2 had disappeared. There had been no improvement in her hearing and there were no consistent brainstem auditory evoked potentials on the affected side. A possible explanation for these findings is given.

Effect of Simulated Bilateral Cochlear Distortion on Speech Discrimination in Normal Subjects

Bilateral sensorineural hearing loss may introduce grossly dissimilar cochlear distortion at the two ears, causing abnormal demands to be made upon the cortical analytical centres which normally receive congruent information. As a result, the prescription of binaural hearing aids may be a handicap rather than a help. In order to explore this possibility, 10 normal subjects were presented with simulated, dissimilar cochlear distortion at the two ears. Discrimination scores with binaural presentation were poorer than the best monaural score and there were clear indications that in the former, subjects selectively attended to one ear and neglected the other. In contrast, binaural presentation of the same simulated distortion resulted in a significant improvement, compared with the monaural discrimination score. Inability of the cortex to contend with discongruent speech input from the two ears may be a factor contributing to the rejection of binaural hearing aids in some individuals.

Defining the Relationship Between Cochlear Hearing Loss and Acoustic Reflex Thresholds

In this study several methods of defining the relationship between the magnitude of cochlear hearing loss and the acoustic reflex threshold (ART) levels were examined, with a view to defining the appropriate upper limits of ART that would ease clinical applicability and reduce the false positive rate. The 90th percentile, two standard deviations (SD) from the mean, the regression method and an empirical method based on the scatter plots of the ART at each activator frequency were all applied to the results of 99 patients with cochlear and/or peripheral vestibular pathology. The upper limits of ART defined on the basis of the scatter plots provided the most appropriate upper limits for different ranges of hearing loss, with relatively few false positives. A clinical criterion based on the ART levels at adjacent frequencies is proposed, which further reduces the false positive rate and could also prove effective in the differential diagnosis of cochlear from retro-cochlear lesions.

The value of acoustic reflex thresholds and brainstem responses in the investigation of cochlear, retrocochlear, and brainstem lesions

Acoustic reflex thresholds (ART) and auditory brainstem responses (ABR) were measured in 69 patients referred to the Neuro-otological clinic on the suspicion of a retro-cochlear lesion. The pure tone selection criterion was an average hearing loss of less than 60 dB at 0.5, 1, 2 and 4 kHz, with no thresholds exceeding 70 dB and only one threshold exceeding 60 dB. Both tests proved independently to be most effective in the diagnosis of cerebello-pontine angle (CPA) tumours and to a lesser extent brainstem lesions. The ABR was positive in 100% of CPA group and 90% in the brainstem group compared to 92.8% and 70% respectively for the ART. The combination of the two tests provides patterns of abnormality which are more disease specific. In the CPA cases the most common and consistent patterns of abnormality were the unilateral delay of wave V or III and V of the ABR associated with a vertical ART pattern indicative of an abnormality of the ipsi and contralateral reflexes on stimulating the affected side. Intrinsic brainstem lesions most commonly resulted in bilateral ABR abnormalities combined with the full house ART pattern indicative of bilateral abnormalities of both ipsi and contralateral reflexes.