Ocular Tilt Reaction Research Articles

The Tullio phenomenon: a neurologically neglected presentation

The Tullio phenomenon refers to sound-induced disequilibrium or oscillopsia. Patients with this condition frequently present to neurologists, many of whom are unfamiliar with the condition and its diagnostic criteria. Indeed, due to the unusual nature of the symptoms patients are often misdiagnosed as having psychiatric disturbances. Tullio patients describe disequilibrium, auditory and visual symptoms, which are recurrent, brief, and often triggered by loud noises or middle ear pressure changes, e.g. the Valsalva manoeuvre. Many cases are associated with superior semicircular canal dehiscence (SCCD). Early work suggested that the presence of sound-induced torsional eye movements and visual field tilts were consequent upon a utricular-mediated ocular tilt reaction. However, more recent evidence from imaging and oculographic research, as well as data from our patient series indicates that these ocular abnormalities are usually the result of superior semicircular canal stimulation. The clinical history and a focussed examination are often sufficient to make the diagnosis, which can be confirmed with high resolution CT imaging of the temporal bones. In some patients, surgical occlusion or resurfacing of the affected canal can ameliorate symptoms and signs. The aim of this paper is two-fold: Firstly, to review the clinical features of the Tullio phenomenon, and secondly, to highlight our own observations in three cases with a new clinical syndrome consisting of Tullio's phenomenon with bilateral vestibular failure, a pure horizontal nystagmus in response to sound, and no evidence of canal dehiscence.

Acute audiovestibular deficit with complete ocular tilt reaction and absent VEMPs

A patient presenting with vertical diplopia along with ocular tilt reaction (OTR) due to peripheral vestibular dysfunction is a rare occurrence. OTR is an eye-head postural reaction consisting of head tilt, skew deviation, conjugated eye cyclotorsion, and alteration of vertical perception, and is thought to occur with central lesions, mainly brainstem lesions. Here, we report a case of a patient who was suffering from left acute peripheral cochleovestibular loss. He had profound deafness and absence of caloric response on the left side. No central lesion was observed on magnetic resonance images. Neuro-ophthalmological examination showed OTR consisting of head tilt, skew deviation with left hypotropia, excyclotorsion, and tilt of the static visual vertical directed to the left side. Both utricular and saccular dysfunctions were identified by the absence of cervical vestibular evoked myogenic potential (cVEMP) and ocular VEMP (oVEMP) on the left side. Within a month, the OTR disappeared completely probably because of vestibular compensation. This is the first report to state that the peripheral otolith dysfunction causing reversible vertical diplopia was identified by objective examinations (VEMP).

Impaired Sacculocollic Reflex in Lateral Medullary Infarction

Objective: The aim of this study was to determine saccular dysfunction by measuring cervical vestibular-evoked myogenic potentials (cVEMP) and to correlate abnormality of cVEMP with results of other vestibular function tests in lateral medullary infarction (LMI). Methods: We recorded cVEMP in 21 patients with LMI documented on MRI. cVEMP was induced by a short tone burst and was recorded in contracting sternocleidomastoid muscle while patients turned their heads forcefully to the contralateral side against resistance. Patients also underwent video-oculographic recording of spontaneous, gaze-evoked and head shaking nystagmus (HSN), evaluation of ocular tilt reaction (OTR), measurement of the subjective visual vertical (SVV) tilt, bithermal caloric tests, and audiometry. Results: Nine patients (43%) showed abnormal cVEMP, unilateral in seven and bilateral in two. The cVEMP abnormalities included decreased p13–n23 amplitude in four, delayed p13/n23 responses in five, and both decreased and delayed responses in two. The abnormal cVEMP was ipsilesional in five, contralesional in two, and bilateral in two. The prevalence of OTR/SVV tilt, spontaneous nystagmus, and HSN did not differ between the patients with normal and abnormal cVEMP. Conclusion: cVEMP was abnormal in approximately half of the patients with LMI. The abnormal cVEMP indicates damage to the descending sacculocollic reflex pathway or disruption of commissural modulation between the vestibular nuclei.

Muscimol inactivation caudal to the interstitial nucleus of Cajal induces hemi-seesaw nystagmus.

Hemi-seesaw nystagmus (hemi-SSN) is a jerk-waveform nystagmus with conjugate torsional and disjunctive vertical components. Halmagyi et al. in Brain 117(Pt 4):789-803 (1994), reported hemi-SSN in patients with unilateral lesions in the vicinity of the Interstitial Nucleus of Cajal (INC) and suggested that an imbalance in projections from the vestibular nuclei to the INC was the source of the nystagmus. However, this hypothesis was called into question by Helmchen et al. in Exp Brain Res 119(4):436-452 (1998), who inactivated INC in monkeys with muscimol (a GABA(A) agonist) and induced failure of vertical gaze-holding (neural integrator) function but not hemi-SSN. We injected 0.1-0.2 microl of 2% muscimol into the supraoculomotor area, 1-2 mm dorso-lateral to the right oculomotor nucleus and caudal to the right INC. A total of seven injections in two juvenile rhesus monkeys were performed. Hemi-SSN was noted within 5-10 min after injection for six of the injections. Around the time the hemi-SSN began, a small skew deviation also developed. However, there was no limitation of horizontal or vertical eye movements, suggesting that the nearby oculomotor nucleus was not initially compromised. Limitations in eye movement range developed about (1/2)-1 h following the injections. Clinical signs that were observed after the animal was released to his cage included a moderate to marked head tilt toward the left (contralesional) side, consistent with an ocular tilt reaction. We conclude that hemi-SSN can be caused by lesions just caudal to the INC, whereas lesions of the INC itself cause down-beat nystagmus and vertical gaze-holding failure, as demonstrated by Helmchen et al. Combined deficits may be encountered with lesions that involve several midbrain structures.

Vestibular function after acute vestibular neuritis

To review the extent and mechanism of the recovery of vestibular function after sudden, isolated, spontaneous, unilateral loss of most or all peripheral vestibular function - usually called acute vestibular neuritis. Critical review of published literature and personal experience. The symptoms and signs of acute vestibular neuritis are vertigo, vomiting, nystagmus with ipsiversive slow-phases, ipsiversive lateropulsion and ocular tilt reaction (the static symptoms) and impairment of vestibulo-ocular reflexes from the ipsilesional semicircular canals on impulsive testing (the dynamic symptoms). Peripheral vestibular function might not improve and while static symptoms invariably resolve, albeit often not totally, dynamic symptoms only improve slightly if at all. The persistent loss of balance that some patients experience after acute vestibular neuritis can be due to inadequate central compensation or to incomplete peripheral recovery and vestibular rehabilitation has a role in the treatment of both.

Ocular Tilt Reaction as a Delayed Complication of Deep Brain Stimulation for Parkinson Disease

A 57-year-old-man treated with deep brain stimulation (DBS) of both subthalamic nuclei for advanced Parkinson disease developed a brain hemorrhage near the site of one of the DBS electrodes 9 months after implantation. The hemorrhage caused vertical diplopia from skew deviation. Examination also disclosed evidence of ipsiversive binocular torsion and a right head tilt, constituting an ocular tilt reaction (OTR). Fourteen months later, he was still symptomatic from diplopia. An OTR has not previously been reported as a delayed complication of DBS.

Synchronised Paroxysmal Ocular Tilt Reaction and Limb Dystonia

ABSTRACTA case of paroxysmal ocular tilt reaction due to a midbrain cavernoma showing co-existing limb dystonia is described with particular reference to the synchronisation of eye and limb paroxysms. The single case has been observed over a 20-year period with video documentation of eye and limb movements. The function of the relevant neuro-anatomical circuits have been investigated. The now 51-year-old female patient suffered from a right midbrain haemorrhage 20 years earlier. She developed a synchronized paroxysmal ocular tilt reaction and focal limb dystonia, associated with a unilateral startle-like response. Video-oculography and electromyography during paroxysms demonstrated left forearm electromyography spikes time locked with bursts of ocular torsion. Whilst the ocular tilt reaction may be explained by a single irritative lesion of the right interstitial nucleus of Cajal, additional involvement of the right rostral interstitial nucleus of the medial longitudinal fasciculus was suggested by abnormal binocular scleral coil recordings of the torsional vestibulo ocular response. There was a marked unilateral acoustic startle-like response, not explained by damage to either nucleus. Treatment with acetazolamide abolished the attacks. The observations suggest that the coexistence of skew deviation, conjugate ocular torsion and limb dystonia in meso-diencephalic lesions may occur because neural centres controlling these eye and limb movements are closely related anatomically, rather than through the activation of a “hard wired” physiological synkinesis. We propose that the paroxysms are generated within the zona incerta where excitatory auditory, somatosensory and motor neurons are densely packed and extensively project to centres of gaze and motor control.

Reacción ocular de inclinación en infarto talámico

Ocular tilt reaction (OTR) includes skew deviation, eye torsion and head tilt. It is usually accompanied by a tilt in the subjective visual vertical. OTR seems to reflect an otolithic dysfunction. This case report shows an OTR of central origin as a result of simultaneous paramedial thalamic and mesencephalon rostral infarcts.

Pearls & Oy-sters: Vestibular neuritis or not?

The ocular tilt reaction (OTR) is a central vestibular disorder that is characterized by the triad of head tilt, skew deviation, and ocular torsion. As the OTR frequently shows an incomplete manifestation, a head tilt can be the only apparent clinical sign of significant brainstem disorders. Hence, even a slight head tilt should be thoroughly investigated. A 48-year-old man presented after sudden onset of a severe, persistent rotatory vertigo associated with nausea, vomiting, and postural imbalance. Examination revealed a horizontal-torsional spontaneous nystagmus with the quick phases beating to the right ear. The nystagmus was attenuated by fixation as examined by Frenzel goggles. Head impulse test was positive to the left. When attempting to walk and during Romberg testing he fell to his left side. Except for a head tilt to the right (see video on the Neurology ® Web site at www.neurology.org), no other neurologic deficit was observed. The patient's medical history revealed no neurologic disorders. Quantitative caloric testing showed a canal paresis of the left vestibular organ. Taken together, the initial clinical presentation was highly suggestive of a left vestibular neuritis. However, due to the head tilt, an extended neuroophthalmologic examination was performed that revealed a discrete but complete OTR to the right with head tilt, mild skew deviation …

Pearls & Oy-sters: Paroxysmal ocular tilt reaction

In skew deviation, one eye is higher than the other, causing vertical diplopia. Skew deviation is a prenuclear disorder resulting from imbalance in otolith input to the oculomotor system, produced by unilateral lesions anywhere from the otoliths to the interstitial nucleus of Cajal in the rostral midbrain,1 and as such is one component of the ocular tilt reaction.2,3 We report a woman with a history of fluctuating diplopia after resection of a left thalamic angioma. She was shown to have intermittent paroxysmal ocular tilt reaction episodes superimposed on a tonic ocular tilt reaction. A 64-year-old woman initially presented at age 55 with malaise, headache, and imbalance, leading to the discovery on neuroimaging of a vascular malformation in the left thalamus. Following resection, she had persistent vertical diplopia on downgaze, and episodes several times a day of vertical diplopia in other gaze positions, associated with a sense of twisting of her eyes. For 9 years she had been on both gabapentin and carbamazepine, with uncertain effect: she reported some modest reduction in episodes with increasing the dose of gabapentin from 600 mg/day to 1,200 mg/day. Visual acuity, tests of color vision with pseudo-isochromatic plates, visual fields by confrontation, pupils, …

OCULAR TILT REACTION: A CLINICAL SIGN OF CEREBELLAR INFARCTIONS?

Ocular tilt reaction (OTR) consists of head tilt, ocular torsion (OT), and skew deviation (SKD) combined with perceptual tilts such as deviations of the subjective visual vertical (SVV). Few case reports have shown that OTR also occurs in patients with cerebellar infarctions.1–4 However, no systematic clinical studies are available on the frequency of signs of OTR in patients with cerebellar lesions. Therefore, the questions arose as to whether OTR is a common clinical sign of an acute cerebellar lesion and whether the time course of its components is similar to those from brainstem infarctions. The cerebellar structures involved in 31 patients were studied in detail elsewhere.5 ### Methods. All 56 patients with acute unilateral cerebellar infarctions (51 ischemic; 5 hemorrhagic) admitted at our department were included in the study (median age 65 years, range 37–84 years; 37 men and 19 women). Forty-three patients had unilateral, and 13 patients with unilateral cerebellar infarctions had additional brainstem lesions (27 patients had right-sided, 29 left-sided lesions). All patients underwent a neuro-ophthalmologic examination including testing tilt of SVV, OT, SKD (for details, see the video5), electronystagmography including caloric irrigation, and MRI scanning. Only two of the patients with infarctions in the territory of the anterior inferior cerebellar artery showed a unilateral hyporesponsiveness in …

Jerky hemi-seesaw nystagmus and head tilt reaction combined with internuclear ophthalmoplegia from a pontine infarction

Internuclear ophthalmoplegia (INO) is a complex ocular motility disorder caused by damage to the medial longitudinal fasciculus. The occurrence of hemi-seesaw nystagmus in an INO patient has been reported rarely. This nystagmus may be caused by damage to the pathway from the contralateral vertical semicircular canal. The ocular tilt reaction is characterized by ipsilateral head and neck tilt, skew deviation, and ocular torsion. We report a patient who presented with hemi-seesaw nystagmus, ocular tilt reaction, and limb ataxia combined with an INO from a right focal pontine infarction. INO may be accompanied by a variety of ocular findings associated with the disruption of the medial longitudinal fasciculus.

Ocular tilt reaction in thalamic infarct

Ocular tilt reaction (OTR) includes skew deviation, eye torsion, and head tilt. It is usually accompanied by a tilt in the subjective visual vertical. OTR seems to reflect an otolithic dysfunction. This case report shows an OTR of central origin as a result of simultaneous paramedial thalamic and mesencephalon rostral infarcts.

33. Ocular tilt reaction – a clinical sign in cerebellar infarctions?

33. Ocular tilt reaction – a clinical sign in cerebellar infarctions?

Paroxysmal ocular tilt reactions after mesodiencephalic lesions: Report of two cases and review of the literature

BackgroundTo elucidate the mechanisms of paroxysmal ocular tilt reaction (OTR) from mesodiencephalic lesions by analyzing the associated ocular motor findings. MethodsTwo patients with paroxysmal ipsiversive OTR due to mesodiencephalic lesions underwent evaluation of associated ocular motor abnormalities and one of them had three-dimensional recording of eye motion. We also reviewed previously reported six patients with paroxysmal OTR. ResultsOne patient showed contraversive torsional nystagmus during the paroxysms in association with vertical gaze limitation and vertical gaze-evoked nystagmus, which are consistent with baseline dysfunction and paroxysmal irritation of the interstitial nucleus of Cajal (INC) during the attacks. The other patient exhibited ipsiversive torsional nystagmus during the attacks, along with slowed vertical saccades and decreased amplitude of ipsitorsional nystagmus during the torsional vestibulo-ocular reflex between the attacks. These findings are best explained by underlying dysfunction and paroxysmal irritation of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF). ConclusionsThe associated ocular motor findings in our patients indicate that paroxysmal ipsiversive OTR in mesodiencephalic lesions is caused by intermittent hyperactivity of partially damaged ipsilesional riMLF or INC.

Teaching Video Neuro Image : Isolated medial rectus palsy in midbrain infarction

A 76-year-old man with hypertension suddenly developed horizontal diplopia. Examination disclosed adduction deficit of left eye without dissociated abducting nystagmus and ocular tilt reaction (OTR) (video). …

Are signs of ocular tilt reaction in patients with cerebellar lesions mediated by the dentate nucleus?

A sensitive clinical sign of a vestibular tone imbalance in the roll plane is the ocular tilt reaction (OTR), a combination of skew deviation, ocular torsion and head and perceptual tilts such as tilts of the subjective visual vertical (SVV). Of these OTR components tilts of SVV are the most frequent. While these signs are regularly seen in patients with unilateral brainstem lesions, only a few case studies are available on their occurrence in patients with cerebellar lesions. Thus, the question arises whether cerebellar structures may be involved in contra- and/or ipsiversive tilts of the perceived vertical and other signs of OTR. We used lesion-mapping techniques in a total of 31 patients with acute cerebellar strokes, all showing at least a significant tilt of SVV. Twenty-three patients had a contraversive tilt of the SVV; they were compared with eight patients with ipsiversive tilts. MRI/CT lesion mapping revealed that in patients showing contraversive signs of OTR in general and contraversive SVV tilts in particular the dentate nucleus was the commonly damaged structure. In contrast, in ipsiversive signs of OTR, the dentate nucleus was spared and lesions were located in the biventer lobule, the middle cerebellar peduncle, the tonsil and the inferior semilunar lobule. These data suggest that the dentate nucleus is a critical anatomical structure within the cerebellum, belonging to a network involved in vestibular processing such as the perception of verticality. Therefore, a lesion of the dentate nucleus can lead to tilts of the SVV in the contraversive direction, i.e. a vestibular tone imbalance to the contralateral side, whereas cerebellar lesions excluding the dentate nucleus can induce a tone imbalance to the ipsilesional side.

Unilateral INO is associated with ocular tilt reaction in pontomesencephalic lesions

Correlation of internuclear ophthalmoplegia (INO) with components of the ocular tilt reaction (OTR) in order to localize graviceptive (specifically otolithic) pathways in the brainstem. We retrospectively analyzed data of 120 patients with INO (87 unilateral [9 of whom had one-and-a-half syndrome], 33 bilateral) for OTR (subjective visual vertical [SVV], ocular torsion, skew deviation) to localize causative brainstem lesions in MRI. Unilateral INO was accompanied by at least one component of OTR: SVV tilt in 96%, ocular torsion in 79%, and skew deviation in 50%. All components were directed to the contralesional side. Contralateral OTR occurred in 89% of patients with one-and-a-half syndrome. Only 9% of patients with bilateral INO exhibited OTR. MRI showed distinct lesions in 68%, which almost exclusively projected onto the pontomesencephalic medial longitudinal fascicle (96%). Follow-up measurements revealed SVV and ocular torsion normalized faster than the adduction deficit in INO. First, unilateral internuclear ophthalmoplegia (INO) is regularly associated with contraversive ocular tilt reaction (OTR): INO plus. Thus, graviceptive pathways join the medial longitudinal fascicle after crossing between the vestibular and abducens nuclei. Second, the different time course and degree of recovery of OTR components and INO signs can be explained by the hypothesis that vestibular tone imbalance is compensated by central vestibular adaptation mechanisms (probably driven mainly by cerebellar-vestibular projections), whereas impaired adduction is less susceptible to compensation according to Hering's law and can only be overcome by lesion repair. Third, bilateral INO is seldom associated with OTR, confirming that bilateral impairment of graviceptive pathways does not cause imbalance in roll plane.

How to differentiate acute peripheral from central vertigo and new findings on the ocular tilt reaction

How to differentiate acute peripheral from central vertigo and new findings on the ocular tilt reaction

Ocular tilt reaction: A clinical sign in cerebellar infarctions?

Objectives: The ocular tilt reaction (OTR) consists of the characteristic triad of head tilt, ocular torsion (OT), and skew deviation (SD). In cerebellar lesions there are only a few case reports available reporting about OTR. Thus, we examined systematically OTR in patients with cerebellar lesions to answer the question whether OTR is a frequent clinical sign in patients with cerebellar lesions or not.