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Objective: Patients with superior canal dehiscence (SCD) have large sound-evoked vestibular reflexes with pathologically low threshold. We wished to determine whether a recently discovered measure of the vestibulo-ocular reflex-the ocular vestibular evoked myogenic potential (OVEMP)-produced similar high-amplitude, low-threshold responses in SCD, and could differentiate patients with SCD from normal control patients., Methods: Nine patients with CT-confirmed SCD and 10 normal controls were stimulated with 500 Hz, 2 ms tone bursts and 0.1 ms clicks at intensities up to 142 dB peak SPL. Conventional VEMPs were recorded from the ipsilateral sternocleidomastoid muscle to determine threshold, and OVEMPs were recorded from electrode pairs placed superior and inferior to the eyes. Three-dimensional eye movements were measured with scleral dual-search coils., Results: In patients with SCD, OVEMP amplitudes were significantly larger than normal (p<0.001) and thresholds were pathologically low. The n10 OVEMP in the contralateral inferior electrode became particularly large with increasing stimulus intensity (up to 25 microV) and with up-gaze (up to 40 microV). Sound-evoked (slow-phase) eye movements were present in all patients with SCD (vertical: upward; torsional: upper pole away from the affected side; and horizontal: towards or away from the affected side), but began only as the OVEMP response became maximal, which is consistent with the surface potentials being produced by activation of the extraocular muscles that generated the eye movements., Conclusions: OVEMP amplitude and threshold (particularly the contralateral inferior n10 response) differentiated patients with SCD from normal controls. Our findings suggest that both the OVEMPs and induced eye movements in SCD are a result of intense saccular activation in addition to superior canal stimulation.

Objective: To examine the properties and potential clinical uses of myogenic potentials to bone conducted sound., Methods: Myogenic potentials were recorded from normal volunteers, using bone conducted tone bursts of 7 ms duration and 250-2000 Hz frequencies delivered over the mastoid processes by a B 71 clinical bone vibrator. Biphasic positive-negative (p1n1) responses were recorded from both sternocleidomastoid (SCM) muscles using averaged unrectified EMG. The best location for stimulus delivery, optimum stimulus frequency, stimulus thresholds, and the effect of aging on evoked response amplitudes and thresholds were systematically examined. Subjects with specific lesions were studied. Vestibular evoked myogenic potentials (VEMP) to air conducted 0.1 ms clicks, 7 ms/250-2000 Hz tones, and forehead taps were measured for comparison., Results: Bone conducted sound evoked short latency p1n1 responses in both SCM muscles. Ipsilateral responses occurred earlier and were usually larger. Mean (SD) p1 and n1 latencies were 13.6 (1.8) and 22.3 (1.2) ms ipsilaterally and 14.9 (2.1) and 23.7 (2.7) ms contralaterally. Stimuli of 250 Hz delivered over the mastoid process, posterosuperior to the external acoustic meatus, yielded the largest amplitude responses. Like VEMP in response to air conducted clicks and tones, p1n1 responses were absent ipsilaterally in subjects with selective vestibular neurectomy and preserved in those with severe sensorineural hearing loss. However, p1n1 responses were preserved in conductive hearing loss, whereas VEMP to air conducted sound were abolished or attenuated. Bone conducted response thresholds were 97.5 (3.9) dB SPL/30.5 dB HL, significantly lower than thresholds to air conducted clicks (131.7 (4.9) dB SPL/86.7 dB HL) and tones (114.0 (5.3) dB SPL/106 dB HL)., Conclusions: Bone conducted sound evokes p1n1 responses (bone conducted VEMP) which are a useful measure of vestibular function, especially in the presence of conductive hearing loss. For a given perceptual intensity, bone conducted sound activates the vestibular apparatus more effectively than air conducted sound.

A woman developed brain stem encephalopathy in association with serum anti-Ma2 antibodies and left upper lobe lung mass. T2 weighted MRI of the brain showed abnormalities involving the pons, left middle and superior cerebellar peduncles, and bilateral basal ganglia. Immunohistochemical analysis for serum antineuronal antibodies was confounded by the presence of a non-neuronal specific antinuclear antibody. Immunoblot studies showed the presence of anti-Ma2 antibodies. A premortem tissue diagnosis of the lung mass could not be established despite two CT guided needle biopsies, and the patient died as a result of rapid neurological deterioration. The necropsy showed that the lung lesion was an adenocarcinoma which expressed Ma2 immunoreactive protein. Neuropathological findings included prominent perivascular inflammatory infiltrates, glial nodules, and neuronophagia involving the brain stem, basal ganglia, hippocampus and the dentate nucleus of the cerebellum. Ma2 is an autoantigen previously identified in patients with germ cell tumours of the testis and paraneoplastic brain stem and limbic encephalitis. Our patient's clinical and immunopathological findings indicate that this disorder can affect women with lung adenocarcinoma, and that the encephalitic changes predominate in those regions of the brain known to express high concentrations of Ma proteins.

Three cases are reported of the pseudotumour cerebri syndrome-that is, intracranial hypertension without mass lesion or enlarged ventricles, due to cryptococcal meningitis. In these patients the papilloedema was successfully treated with optic nerve sheath decompression, and the intracranial hypertension with lumboperitoneal CSF shunting. These cases support the concept that pseudotumour cerebri is a syndrome of intracranial hypertension that can be due to any disorder producing obstruction of the CSF pathways at the level of the arachnoid villi. This concept is important because it directs therapy to normalise the intracranial pressure and preserve vision.

A 64 year old woman with a predominantly midline pontine tegmental haemorrhage presented with bilateral total deafness. One week later reasonable pure-tone thresholds appeared but she still had total bilateral loss of speech discrimination. At that time contralateral acoustic reflexes were bilaterally absent, whereas ipsilateral acoustic reflexes and waves IV and V of the brainstem auditory evoked potential were bilaterally preserved. It is proposed that this patient's hearing deficit was due to inactivation of the ventral acoustic striae decussating in the trapezoid body. This case supports the contention that in humans the ventral pontine acoustic decussation carries most of the neural signals required for hearing and perhaps all the neural signals required for speech perception.

A patient was seen with secondary cluster headache whose acute pain responded promptly to sumatriptan. The headaches started after injury to the vertebral artery. This finding provides clinical affirmation of the existence of the trigeminal/cervical nuclear overlap that is central to this condition.

Electromyograms (EMGs) were recorded from surface electrodes over the sternomastoid muscles and averaged in response to brief (0.1 ms) clicks played through headphones. In normal subjects, clicks 85 to 100 dB above our reference (45 dB SPL: close to perceptual threshold for normal subjects for such clicks) evoked reproducible changes in the averaged EMG beginning at a mean latency of 8.2 ms. The earliest potential change, a biphasic positive-negativity (p13-n23), occurred in all subjects and the response recorded from over the muscle on each side was predominantly generated by afferents originating from the ipsilateral ear. Later potentials (n34, p44), present in most but not all subjects, were generated bilaterally after unilateral ear stimulation. The amplitude of the averaged responses increased in direct proportion to the mean level of tonic muscle activation during the recording period. The p13-n23 response was abolished in patients who had undergone selective section of the vestibular nerve but was preserved in subjects with severe sensorineural hearing loss. It is proposed that the p13-n23 response is generated by activation of vestibular afferents, possibly those arising from the saccule, and transmitted via a rapidly conducting oligosynaptic pathway to anterior neck muscles. Conversely, the n34 and p44 potentials do not depend on the integrity of the vestibular nerve and probably originate from cochlear afferents.

Resting and postural tremor, intention and action tremor, clonus and the cogwheel phenomenon in Parkinson's disease have been characterised in terms of frequency content using spectral analysis. Typical resting tremor ranged in peak frequency from 4 to 5.3 HZ with tremor in each individual varying only by 0.2 to 0.3 HZ. The peak frequency of postural tremor ranged between 6 and 6.2 HZ. Intention tremor appeared to be an exaggeration of postural tremor. Clonus evoked by active or passive stretch at the wrist had a frequency of 6 HZ and appeared to be a continuation of postural tremor. The cogwheel phenomenon was found at frequencies between 6 and 6.5 HZ and between 7.5 to 9 HZ. Action tremor was indistinguishable from the cogwheel phenomenon. Some patients had either a symptomatic resting tremor with a concurrent 6 HZ component of smaller amplitude or a symptomatic postural tremor with a 4-5 HZ component of smaller amplitude. These combinations would produce two peaks in the power spectrum. When this occurred EMG studies showed that individual muscles had two types of rhythmical activation suggesting that the tremors have separate mechanisms. Likewise some patients had a symptomatic 6 HZ tremor on posture with a second peak at 8-10 HZ in the physiological band. Therefore, the 6 HZ postural tremor is not an exaggeration of physiological tremor. On the basis of wave form and frequency similarities postural tremor, the low frequency type of active or passive cogwheeling, intention tremor and clonus possibly involve a common spinal mechanism. Higher frequency cogwheel phenomenon and action tremor may be an exaggeration of physiological tremor. More than 80% of patients with Parkinson's disease manifest tremors at both 4-5 HZ and 6 HZ. This combination would appear to be the strongest objective criterion for the diagnosis of basal ganglia disease.

Abnormal head movements have been studied in a variety of diseases using objective recording techniques and the data analysed with respect to the frequency content of the movement. Flopping, nodding, tic, chorea, myoclonic jerks, and most head tremors involve frequencies of approximately 2 and 4 Hz which correspond to the natural fundamental and second harmonic resonances of the head as determined by the mechanical properties of the head/neck system. These findings provide a basis for classification of abnormal head movements as well as an explanation of the characteristics of those arising from hypotonia of the neck muscles. The similarities between tremor frequencies and natural resonances suggest that in the case of the head, tremor arises from disorders of neural mechanisms normally responsible for the fine control of voluntary head movement and for stabilisation of the head during disturbance of posture. Head movements in cases of congenital nystagmus were found to be of two types. Some were of bizarre waveform, in no way assisted vision, and were taken to be of primarily pathological origin and classified as tremors. Others were learned adaptive responses which assisted vision either by interrupting the nystagmus, as in the case of spasmus nutans, or by compensating for the nystagmus with an inverse waveform and were called nodding. A prerequisite for true compensatory nodding is modified vestibulo-ocular reflex.

Visual suppression of vestibulo-ocular reflexes (induced vestibular nystagmus) differs between normal subjects and patients with various neurological disorders. Abnormalities of VOR suppression were associated consistently with abnormalities of the visual and/or vesitibular oculomotor reflexes and in particular with abnormal smooth pursuit eye movements in the direction of abnormal suppression. Absent VOR suppression in a gaze position of, and in the same direction as, a spontaneous nystagmus was found exclusively in patients with spontaneous nystagmus of central nervous system origin. Conversely, ability to suppress in these circumstances was found only in patients with spontaneous nystagmus of peripheral labyrinthine origin. Suppression of VOR was abnormal ipsilaterally in patients with unilateral cerebral hemisphere lesions and abnormal in both the horizontal and vertical planes in patients with basal ganglia lesions. Failure of VOR suppression in the absence of spontaneous nystagmus indicates a supratentorial lesion.

Conventional neuro-otological tests measure only semi-circular canal function and not otolith function. A clinically acceptable test of otolith function was developed. Previous studies suggested that muscle responses which occur less than 100 ms after release into free-fall are part of a startle reflex originating in the otoliths. With a couch capable of producing sudden, safe, comfortable free-falls it was shown that in normal subjects orbicularis oculi muscle responses (that is eyeblinks) invariably began within 45 ms of this stimulus and confirmed that these eyeblink reflexes are part of a generalised startle reflex, as they cross-habituate with eyeblink reflexes elicited by supraorbital nerve stimulation. Eyeblink reflexes to free-fall may prove a useful clinical test of otolith function.