Optic nerve sheath fenestration for treating papilloedema in the era of cerebral venous sinus stenting.

BACKGROUND: Pseudotumour cerebri (PTC) is the syndrome of intracranial hypertension without intracranial mass or hydrocephalus and is the commonest cause of papilloedema seen in many eye clinics. In the last 10 years, we have increasingly used TSS in patients whose papilloedema was not well controlled with medical treatment and have done fewer ONSFs. Here, we review our experience at Royal Prince Alfred Hospital Sydney with ONSF in 35 patients over the period 2002-2021. METHODS: Retrospective case series of 35 patients, 30 of whom had primary PTC [i.e., idiopathic intracranial hypertension (IIH)] and 5 with secondary PTC. RESULTS: Eighteen patients had bilateral ONSF and 17 patients unilateral ONSF, in each case of the worse eye. Thirteen patients then underwent transverse sinus stenting (TSS), in each case following ONSF. The primary outcome measures were visual acuity (VA) and mean deviation (MD) on visual field (VF) testing. MD improved by 5 dB or more in 34 of 70 total eyes (48.6%); VA improved by 0.2 logMAR (two lines on Snellen chart) or more in 21 eyes (30%), and by both in 15 eyes (21.4%). Final MD was -10 dB or better in 38 eyes (54.3%); final VA was 0.3 (6/12) or better in 54 eyes (77.1%), and both in 39 eyes (55.7%). CONCLUSIONS: The results confirm that ONSF can relieve papilloedoema in both eyes and improve both VF and VA, even in cases of fulminant PTC with severe acute visual impairment.

Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects.

PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

ACR Appropriateness Criteria® Routine Chest Imaging.

Routine chest imaging has been used to identify unknown or subclinical cardiothoracic abnormalities in the absence of symptoms. Various imaging modalities have been suggested for routine chest imaging. We review the evidence for or against the use of routine chest imaging in different clinical scenarios. This document aims to determine guidelines for the use of routine chest imaging as initial imaging for hospital admission, initial imaging prior to noncardiothoracic surgery, and surveillance imaging for chronic cardiopulmonary disease. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Vestibular, saccadic and fixation abnormalities in genetically confirmed Friedreich ataxia.

Friedreich ataxia (FRDA), the commonest of the inherited ataxias, is a multisystem neurodegenerative condition that affects ocular motor function. We assessed eye movement abnormalities in 20 individuals with genetically confirmed FRDA and compared these results to clinical measures. All subjects were assessed with infrared oculography. Fifteen individuals underwent a full protocol of eye movement recordings. Ten subjects were analysed using two-dimensional scleral coil equipment and five using three-dimensional scleral coil recording equipment. We also recorded visual quality of life, Sloan low contrast letter acuity and Friedreich Ataxia Rating Scale scores to compare to the visual measures. Whilst saccadic velocity was essentially normal, saccadic latency was prolonged. The latency correlated with clinical measures of disease severity, including the scores for the Friedreich Ataxia Rating Scale and the Sloan low contrast letter acuity tests. Fixation abnormalities consisting of square wave jerks and ocular flutter were common, and included rare examples of vertical square wave jerks. Vestibular abnormalities were also evident in the group, with markedly reduced vestibulo-ocular reflex gain and prolonged latency. The range of eye movement abnormalities suggest that neurological dysfunction in FRDA includes brainstem, cortical and vestibular pathways. Severe vestibulopathy with essentially normal saccadic velocity are hallmarks of FRDA and differentiate it from a number of the dominant spinocerebellar ataxias. The correlation of saccadic latency with FARS score raises the possibility of its use as a biomarker for FRDA clinical trials.

Vestibulo-ocular reflex deficits with medial longitudinal fasciculus lesions.

The medial longitudinal fasciculus (MLF) is the final common pathway for all conjugate adducting horizontal eye movements, as well as for the vertical-torsional vestibulo-ocular reflex (VOR). MLF lesion causes adduction paresis of ipsilesional (adducting) eye with dissociated nystagmus of contralesional (abducting) eye-the well-known clinical syndrome of internuclear ophthalmoplegia (INO). We measured the VOR stimulation and also any catch-up saccades, from individual semicircular canal (SCC) evoked by the head impulse test (HIT), using head and binocular 3-dimensional scleral search coils in 27 multiple sclerosis (MS) patients, 8 with unilateral, 19 with bilateral INO. In unilateral INO, VOR gain (normal >0.90) from ipsilesional lateral SCC stimulation was 0.48 for the adducting eye and 0.81 for the abducting eye; 0.61 from contralesional anterior SCC stimulation and only 0.29 from contralesional posterior SCC stimulation. In bilateral INO, there were VOR gain deficits from all six SCCs: lateral SCC gains were asymmetrically reduced to 0.45 in the adducting eye and 0.66 in the abducting eye; anterior SCC gain was 0.48 and posterior SCC gain was only 0.19. Horizontal VOR versional dysconjugacy between adducting and abducting eyes at 0.66 was less severe than horizontal catch-up saccade versional dysconjugacy (0.44); normal >0.80. Unexpected partial preservation of horizontal VOR with greater catch-up saccade impairment from the adducting than abducting eye suggests that the ascending tract of Deiters (ATD), an extra-MLF pathway, also mediates the horizontal VOR, but not adducting horizontal saccades. Vertical VOR deficits will produce vertical oscillopsia with any vertical head movement and measurement of the vertical VOR could help with the diagnosis and quantitative evaluation of MLF lesions in suspected MS. Horizontal VOR deficits and catch-up saccade versional dysconjugacy in INO will cause gaze instability and horizontal oscillopsia during active horizontal head movements.

MRI of the vestibular nerve after selective vestibular neurectomy.

CONCLUSION: In patients with Ménière's disease and persisting vertigo attacks after vestibular neurectomy (VNx) MRI of the vestibulocochlear nerve can identify residual vestibular nerve fibres that could be responsible for the vertigo attacks. OBJECTIVE: To test if MRI of the vestibulocochlear nerve can corroborate the presence of residual vestibular nerve fibres in patients with persisting vertigo attacks and residual vestibular function after VNx. MATERIALS AND METHODS: Vestibulocochlear nerve bundles of seven post-VNx unilateral Ménière's patients were imaged using 1.5 Tesla MRI with steady state free precession (SSFP) sequences. Reformatted MR images orthogonal to the vestibulocochlear nerve axis in internal auditory canal were compared on the VNx and intact sides. Vestibular function was assessed with caloric tests, three-dimensional head impulse tests and vestibular evoked myogenic potentials. Of the seven patients only one was asymptomatic (totally free of vertigo); six had continued to experience vertigo attacks, albeit not as long or as severe as before VNx. RESULTS: On the VNx side, MRI showed intact facial and cochlear nerves in all seven patients. In the six symptomatic patients, although superior and inferior vestibular nerve bulk and signal were reduced, residual bulk suggestive of inferior vestibular nerve was evident, correlating with evidence of residual posterior canal function on impulsive testing in all six symptomatic patients. In the asymptomatic patient, superior and inferior vestibular nerves were absent on MRI and impulsive testing revealed no residual posterior canal function.

The active head-impulse test in unilateral peripheral vestibulopathy.

BACKGROUND: The head-impulse test, which is sensitive and specific for detecting severe unilateral peripheral vestibulopathy, is an accepted part of the neurological examination, especially in patients with vertigo and balance disorders. OBJECTIVE: To discover if the head-impulse test is just as useful diagnostically when patients are asked to rotate their own heads, the active head-impulse test, rather than when the clinician does so as in the standard passive head-impulse test. METHODS: Clinical observation of compensatory saccades and search coil measurement of compensatory eye rotations, during active and passive horizontal head-impulses in 6 patients with total unilateral vestibular deafferentation. RESULTS: Clinical observation showed the expected compensatory saccades with rotations toward the side with the lesion with passive head-impulses but not with active head-impulses. Search coil recordings revealed 2 reasons for this. With active head-impulses not only was vestibulo-ocular reflex gain higher, but compensatory saccade latency was shorter resulting in an occult saccade that occurred during, rather than after, head rotation. CONCLUSIONS: Passive head-impulses are necessary to detect a severe unilateral peripheral vestibulopathy; active head-impulses will produce a false-negative result.

Benign paroxysmal positional vertigo can interfere with the cardiac response to head-down tilt.

OBJECTIVE: To assess the pulse rate and the respiratory rate responses to head-down tilt of the whole body in the plane of the posterior canals in healthy subjects and in patients with benign paroxysmal positional vertigo (BPPV). BACKGROUND: Although BPPV attacks are usually accompanied by autonomic symptoms, there are no studies assessing autonomic responses during triggering maneuvers for BPPV, neither in healthy subjects nor in patients. METHODS: We evaluated nine healthy subjects and four BPPV patients (3 unilateral and 1 bilateral). Using a two-axis rotator, from an upright position they were rotated 135 degrees backwards to head-down tilt, either in the plane of the right or the left posterior canal. RESULTS: In healthy subjects, head-down tilt always induced a significant decrease of the pulse rate, which was similar after rotation to the right and to the left posterior canals. This response was observed in patients with unilateral BPPV only when they were rotated toward the nonaffected side, and it was not evident when they were rotated toward the affected side (p < 0.025). In the patient with bilateral BPPV, no change of the pulse rate was observed after rotation toward the right or to the left posterior canal. Although, in all the patients, the respiratory rate increased during the tilt, a similar increase was observed in two healthy subjects. CONCLUSION: After rotation in the plane of the affected semicircular canal, BPPV can interfere with the cardiac response to head-down tilt of the whole body.

Superior semicircular canal dehiscence simulating otosclerosis.

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

Vestibular and Saccadic Abnormalities in Gaucher's Disease.

Gaucher's disease (GD) is a hereditary lysosomal storage disease characterized by abnormal deposition of glucocerebroside due to the enzyme glucocerebrosidase deficiency, resulting in multi-organ pathology. GD type III has a progressive neurological involvement. We studied the vestibular and saccadic abnormalities in GD type III to determine if these parameters may be useful for assessing neurological involvement. We evaluated the vestibular and saccadic responses of two siblings with genetically identified GD type III on enzyme replacement therapy. Vestibular functions were assessed with the head impulse test (HIT), vestibular evoked myogenic potentials (VEMPs), and electrical vestibular stimulation (EVS). Saccadic functions were investigated with volitional horizontal and vertical saccades to ±20°. Three-dimensional head and eye movements were recorded with dual-search coils and VEMP with surface electrodes. HIT showed impaired individual semicircular canal function with halved angular vestibulo-ocular reflex (VOR) gains and absent horizontal refixation saccade. Ocular and cervical VEMPs to air-conducted clicks were absent in the older sibling, and only cervical VEMP was present in the younger sibling indicating otolithic dysfunction. EVS showed prolonged onset latency and attenuated tonic and phasic responses suggesting impaired neural conduction and vestibular function. Horizontal saccadic velocity was miniscule (<30°/s) and multiple back-to-back saccades with saccade-vergence interaction were utilized to minimize eye position error in the older sibling. Vertical saccades were slightly abnormal, but vergence and smooth pursuit were normal in both siblings. Our findings suggest that GD affected the vestibular nuclei in addition to the paramedian pontine reticular formation. These vestibular and saccadic abnormalities may be useful biomarkers to monitor neurological deterioration.

Enhanced vestibulo-ocular reflex to electrical vestibular stimulation in Meniere's disease.

Meniere's disease is characterized by sporadic episodes of vertigo, nystagmus, fluctuating sensorineural hearing loss, tinnitus and aural pressure. Since Meniere's disease can affect different regions of the vestibular labyrinth, we investigated if electrical vestibular stimulation (EVS) which excites the entire vestibular labyrinth may be useful to reveal patchy endorgan pathology. We recorded three-dimensional electrically evoked vestibulo-ocular reflex (eVOR) to transient EVS using bilateral, bipolar 100-ms current steps at intensities of 0.9, 2.5, 5.0, 7.5 and 10.0 mA with dual-search coils in 12 unilateral Meniere's patients. Their results were compared to 17 normal subjects. Normal eVOR had tonic and phasic spatiotemporal properties best described by the torsional component, which was four times larger than horizontal and vertical components. At EVS onset and offset of 8.9 ms latency, there were phasic eVOR initiation (M = 1,267 °/s(2)) and cessation (M = -1,675 °/s(2)) acceleration pulses, whereas during the constant portion of the EVS, there was a maintained tonic eVOR (M = 9.1 °/s) at 10 mA. However in Meniere's disease, whilst latency of EVS onset and offset was normal at 9.0 ms, phasic eVOR initiation (M = 1,720 °/s(2)) and cessation (M = -2,523 °/s(2)) were enlarged at 10 mA. The initiation profile was a bimodal response, whilst the cessation profile frequently did not return to baseline. The tonic eVOR (M = 20.5 °/s) exhibited a ramped enhancement of about twice normal at 10 mA. Tonic eVOR enhancement was present for EVS >0.9 mA and disproportionately enhanced the torsional, vertical and horizontal components. These eVOR abnormalities may be a diagnostic indicator of Meniere's disease and may explain the vertigo attacks in the presence of declining mechanically evoked vestibular responses.

Augmented active surface model for the recovery of small structures in CT.

This paper devises an augmented active surface model for the recovery of small structures in a low resolution and high noise setting, where the role of regularization is especially important. The emphasis here is on evaluating performance using real clinical computed tomography (CT) data with comparisons made to an objective ground truth acquired using micro-CT. In this paper, we show that the application of conventional active contour methods to small objects leads to non-optimal results because of the inherent properties of the energy terms and their interactions with one another. We show that the blind use of a gradient magnitude based energy performs poorly at these object scales and that the point spread function (PSF) is a critical factor that needs to be accounted for. We propose a new model that augments the external energy with prior knowledge by incorporating the PSF and the assumption of reasonably constant underlying CT numbers.

Weighted Distance Weighted Discrimination and Its Asymptotic Properties.

While Distance Weighted Discrimination (DWD) is an appealing approach to classification in high dimensions, it was designed for balanced datasets. In the case of unequal costs, biased sampling, or unbalanced data, there are major improvements available, using appropriately weighted versions of DWD (wDWD). A major contribution of this paper is the development of optimal weighting schemes for various nonstandard classification problems. In addition, we discuss several alternative criteria and propose an adaptive weighting scheme (awDWD) and demonstrate its advantages over nonadaptive weighting schemes under some situations. The second major contribution is a theoretical study of weighted DWD. Both high-dimensional low sample-size asymptotics and Fisher consistency of DWD are studied. The performance of weighted DWD is evaluated using simulated examples and two real data examples. The theoretical results are also confirmed by simulations.

A mathematical model of human semicircular canal geometry: a new basis for interpreting vestibular physiology.

We report a precise, simple, and accessible method of mathematically measuring and modeling the three-dimensional (3D) geometry of semicircular canals (SCCs) in living humans. Knowledge of this geometry helps understand the development and physiology of SCC stimulation. We developed a framework of robust techniques that automatically and accurately reconstruct SCC geometry from computed tomography (CT) images and are directly validated using micro-CT as ground truth. This framework measures the 3D centroid paths of the bony SCCs allowing direct comparison and analysis between ears within and between subjects. An average set of SCC morphology is calculated from 34 human ears, within which other geometrical attributes such as nonplanarity, radius of curvature, and inter-SCC angle are examined, with a focus on physiological implications. These measurements have also been used to critically evaluate plane fitting techniques that reconcile many of the discrepancies in current SCC plane studies. Finally, we mathematically model SCC geometry using Fourier series equations. This work has the potential to reinterpret physiology and pathophysiology in terms of real individual 3D morphology.

Impulsive testing of semicircular canal function.

After acute vestibular loss in humans or animals, eye-movement responses to rapid horizontal ipsilesional head rotations ("head impulses") show that there is severe, permanent impairment of the angular vestibulo-ocular reflex. The basis for this appears to be an inhibitory saturation of ipsilesional vestibular nerve, and perhaps vestibular nucleus afferents, in response to high-acceleration, off-direction stimulation of the sole functioning member of any pair of semicircular canals. For the clinician, impulsive testing is an easy and reliable way to identify severe unilateral as well as bilateral impairment of semicircular canal function.

Three-dimensional kinematics of saccadic eye movements in humans with cerebellar degeneration.

During saccades, the orientation of the eye velocity axis varies depending on the instantaneous eye position. The means by which this eye velocity axis tilting arises remains contentious. Some have argued that muscle pulleys in the orbit implement the tilts, since they cause the pulling directions of the extraocular muscles to change in a manner that depends on instantaneous eye position. Others have suggested that the tilting is centrally programmed. In the current study, three-dimensional eye and head rotation data were acquired, using the magnetic search coil technique, to confirm the presence of eye position-dependent eye velocity axis tilting during saccades. We studied normal humans and humans with inherited or sporadic cerebellar degeneration. While the humans with cerebellar degeneration were noted to have abnormalities in the two-dimensional metrics and consistency of their saccades, the eye velocity axis tilts were similar to those observed in the normal subjects. Our findings suggest that the cerebellum does not encode eye velocity axis tilting during saccades, further supporting the notion that these phenomena arise due to the effects of muscle pulleys.

Inter-ocular differences of the horizontal vestibulo-ocular reflex during impulsive testing.

Impulsive testing, which accurately measures the gain of the vestibulo-ocular reflex (VOR) is mostly recorded from only one eye. We measured the horizontal VOR of both eyes in response to graded velocity head impulses in 14 normal subjects. VOR acceleration gains were similar for each eye in both directions at low accelerations, but as head acceleration increased, gains of the adducting eye exceeded gains of the abducting eye. These inter-ocular VOR gain differences were due to longer latencies but higher accelerations of the adducting eye compared to the abducting eye. Consequently, directional VOR gain asymmetry occurred at high accelerations if the same eye was always analysed--with an average inter-ocular difference of 15.3%. If only the abducting eye was analysed, VOR gains were symmetrical in both directions. For accurate measurements of the VOR at high accelerations, the inter-ocular VOR gain differences require binocular recording.

Latency and initiation of the human vestibuloocular reflex to pulsed galvanic stimulation.

Cathodal galvanic currents activate primary vestibular afferents, whereas anodal currents inhibit them. Pulsed galvanic vestibular stimulation (GVS) was used to determine the latency and initiation of the human vestibuloocular reflex. Three-dimensional galvanic vestibuloocular reflex (g-VOR) was recorded with binocular dual-search coils in response to a bilateral bipolar 100-ms rectangular pulse of current at 0.9 (near-threshold), 2.5, 5.0, 7.5, and 10.0 mA in 11 normal subjects. The g-VOR consisted of three components: conjugate torsional eye rotation away from cathode toward anode; vertical divergence (skew deviation) with hypertropia of the eye on the cathodal and hypotropia of the eye on the anodal sides; and conjugate horizontal eye rotation away from cathode toward anode. The g-VOR was repeatable across all subjects, its magnitude a linear function of the current intensity, its latency about 9.0 ms with GVS of >or=2.5 mA, and was not suppressed by visual fixation. At 10-mA stimulation, the g-VOR [x, y, z] on the cathodal side was [0.77 +/- 0.10, -0.05 +/- 0.05, -0.18 +/- 0.06 degrees ] (mean +/- 95% confidence intervals) and on the anodal side was [0.79 +/- 0.10, 0.16 +/- 0.05, -0.19 +/- 0.06 degrees ], with a vertical divergence of 0.20 degrees . Although the horizontal g-VOR could have arisen from activation of the horizontal semicircular canal afferents, the vertical-torsional g-VOR resembled the vestibuloocular reflex in response to roll-plane head rotation about an Earth-horizontal axis and might be a result of both vertical semicircular canal and otolith afferent activations. Pulsed GVS is a promising technique to investigate latency and initiation of the human vestibuloocular reflex because it does not require a large mechanical apparatus nor does it pose problems of head inertia or slippage.

Head impulse test reveals residual semicircular canal function after vestibular neurectomy.

Meniere disease patients sometimes report vertiginous Meniere attacks after vestibular neurectomy that spares hearing. To determine why, the authors compared postsurgical semicircular canal function in nine patients with preserved hearing with that of a control group with no preservation of hearing. The three-dimensional head impulse test revealed residual posterior canal function in all patients with vertigo attacks (eight). The control patients had no residual canal function. Thus, residual vestibular function on the ipsilesional side may cause vertiginous Meniere attacks.