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10.5005/jp-journals-10003-1093
Nystagmus
REVIEW ARTICLE
Nystagmus
Bachi T Hathiram, Vicky S Khattar
ABSTRACT
Nystagmus is an important clinical sign. Often it may be the
harbinger of an intracranial pathology, and at other times may
just be a silent manifestation of a short lived vestibular imbalance;
yet at other times it may be an intermittently visible sign that may
have to be induced, and again, at others it may be the only
evidence to support an ongoing pathology. Given its numerous
causes and presentations, it becomes important for the clinician
dealing with balance disorder patients to understand the basic
physiology and mechanisms that surround it.
Presented below is a brief outline of the physiology, the
causes and their respective interpretations of nystagmus.
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Fig. 2: Schematic diagram of the caloric irrigation procedure and the resultant nystagmus response
Fig. 3: The tracing for the caloric test performedright (red and Fig. 5: The nystagmus seen due to rotational stimulation
blue) and left (green and pink)
Fig. 6: The Frenzels goggles Fig. 9: The nystagmus beats superimposed upon the smooth
pursuit tracing (pink) with the stimulus seen in green
Fig. 7: A patient wearing Frenzels goggles. The lights are turned Fig. 10: The nystagmus beats superimposed upon the saccadic
down, as the glasses are illuminated with small bulbs, which are tracing (pink) with the stimulus seen in green
seen through the magnifying lens. The patient perceives blurred
images and bright lights, and hence cannot make use of peripheral
fixation
large moving field, e.g. looking out of the window of a
moving vehicle. The oscillations are in the plane of the
moving field and are generally 3 to 4 in amplitude and 2 to
3 Hz in frequency. Both the cortical and subcortical
mechanisms are involved in this nystagmus which is driven
by the retinal image slip velocity. Smooth pursuit inputs
are of particular importance.7,8,15-17
On far eccentric gaze (>40), normal individuals will
show a small amplitude (<2) conjugate, jerk nystagmus.
This is termed as the end-point nystagmus and is
physiological. It is thought to reflect the time constant of
the gaze-holding control system and in particular, the
cerebellar neural integrator.7-9,15,18-20
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nystagmus.10,12 The oscillations in both are conjugate, nystagmus which is caused by a disease of the brainstem
horizontal and jerky, however, in congenital nystagmus and/or cerebellum, is not attenuated by visual fixation and
(CN) the slow phase velocity increases exponentially may be bidirectional.
whereas in manifest latent nystagmus (MLN), the slow
phase velocity decreases or remains the same. Also, the fast Clinical Significance and Management
phase of the nystagmus in MLN always beats toward the Oculomotor disturbances and nystagmus are seen in many
viewing eye. MLN is usually associated with the presence diseases of the vestibular apparatus, the nervous system,
of strabismus and dissociated vertical divergence, is strongly and the eyes, as well as in toxic and metabolic disorders.
visually driven and largely dependent on the attentional state They often indicate a specific underlying cause. The key to
of the patient.20 CN may occur without any ocular or central diagnosis is systematic clinical examination of the patients
nervous system anomalies, i.e. idiopathic CN. However, eye movements. A thorough knowledge of the charac-
both MLN and CN are usually associated with various teristics of the nystagmus can often help to determine the
systemic disorders, such as albinism, optic nerve hypoplasia site of the lesion, pathogenesis and underlying mechanisms.
and congenital cataracts.21-25 There are various theories In acute unilateral peripheral vestibular deficit,
proposed for the etiopathogenesis of infantile and congenital
horizontal spontaneous nystagmus (SN) increases when
nystagmus, however none have been proven so far.
patients lie on their affected ear. This phenomenon indicates
an ipsilesional reduction of otolith function that normally
Acquired Nystagmus
suppresses asymmetric semicircular canal signals. In the
The three mechanisms that normally ensure steady gaze course of vestibular compensation, the velocity of
include; visual fixation, the vestibulo-ocular reflex and the spontaneous eye drift gradually decreases. Patients with
mechanism that makes it possible to hold the eyes at an chronic unilateral vestibular deficit may yet show some SN
eccentric eye position. Disturbances in these mechanisms in darkness, but not during ocular fixation in the light.26
give rise to acquired nystagmus. Nystagmus can still be detected, however, after shaking the
Diseases affecting the visual system causing loss of head rapidly over 20 to 30 cycles in the horizontal plane27
vision will cause nystagmus due to loss of visual fixation. or after whole body oscillation on a turn table about an Earth-
Diseases affecting the vestibular organ in the inner ear will vertical axis.28,29 This so-called head-shaking nystagmus
result in a horizontal torsional nystagmus usually associated (HSN) is considered to be a sensitive symptom for detecting
with vertigo. Diseases affecting the central connections of asymmetries in the vestibular system.28,30,31 The presence
the vestibular system may result in down-beating nystagmus, of HSN reflects a directional imbalance of the vestibulo-
torsional nystagmus, periodic alternation nystagmus or see- ocular reflex (VOR) in the high frequency range after
saw nystagmus. unilateral vestibular lesions. During high-acceleration head
The gaze-evoked nystagmus is one of the most studied rotations, the VOR is mainly generated by the excited side,32
and frequently-seen acquired nystagmus. It is elicited when because the nonlinear pathway of the inhibited side is driven
the patient attempts to maintain an eccentric eye position. into inhibitory cutoff.33
The oscillations are jerky with a decreasing velocity slow The pseudospontaneous nystagmus (PSN) is a new
phase taking the eyes away from the desired position, clinical sign to diagnose the impaired side of lateral
followed by the corrective fast phase. It is thought to occur semicircular canal-BPPV described, for the first time, in
due to a failure of the step or tonic eye position command 2005.34 In a study by Asprella-Libonatti (2008),35 293
from the gaze holding network (the neural integrator).8,13,19 patients affected by lateral semicircular canal benign
It is very difficult to differentiate a physiological end-point paroxysmal positional vertigo (197 geotropic and 96
nystagmus from an acquired gaze-evoked nystagmus by apogeotropic forms) were examined. Pseudospontaneous
viewing eye movements alone.18,19,22 nystagmus was observed in 222 patients (76%). After a very
Pathologies affecting the vestibular labyrinth or nerve slow, repeated horizontal rotation of the head, in the seated
cause a jerk nystagmus with a linear or constant slow phase position, this percentage increased to 96% (281 patients).
velocity. Typically, the nystagmus increases when the eyes The pseudospontaneous nystagmus and the seated supine
are turned in the direction of the fast phase keeping with positioning nystagmus always beat in the same direction
the principle of Alexanders law and it is suppressed by and both were in accordance in identifying the affected side
visual fixation. The direction of the nystagmus is opposite with the nystagmus evoked by the head yaw test. The
to the side of the lesion. A change in head position may differential diagnosis between spontaneous nystagmus and
exacerbate this nystagmus. In contrast, a central vestibular pseudospontaneous nystagmus is easily achieved with the
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24. DellOsso LF. Congenital latent and manifest latent nystagmus: 35. Asprella-Libonatti G. Pseudospontaneous nystagmus: A new
similarities, differences and relation to strabismus. Jpn J sign to diagnose the affected side in lateral semicircular canal
Ophthalmol 1985;29:351-63. benign paroxysmal positional vertigo. Acta Otorhinolaryngo-
25. Gage JE, Abadi RV, Lloyd IC, Thompson CM. Fixation stability logical Italica 2008;28:73-78.
and infantile cataract. Invest Ophtholmol Vis Sci 2001;42:4S-164. 36. Strupp M, Brandt T. Pharmacological advances in the treatment
26. Baloh RW, Honrubia V. Clinical Neurophysiology of the vestibular of neuro-otological and eye movement disorders. Curr Opin
system (3rd ed). Oxford University Press, New York, 2001. Neurol 2006;19:33-40.
37. Straube A, Leigh RJ, Bronstein A, et al. EFNS task force-therapy
27. Kamei T, Kimura K, Kaneko H. Re-evaluation of the head-
of nystagmus and oscillopsia. Eur J Neurol 2004;11:83-89.
shaking test as a method of nystagmus provocation. Jpn J Otol
38. Leigh RJ, Tomsak RL. Drug treatments for eye movement
1964;67:1530-34.
disorders. J Neurol Neurosurg Psychiatry 2003;74:1-4.
28. Fetter M, Zee DS, Koenig E, Dichgans J. Head-shaking 39. Strupp M, Brandt T. Current treatment of vestibular, ocular motor
nystagmus during vestibular compensation in humans and rhesus disorders and nystagmus. Ther Adv Neurol Disord
monkeys. Acta Otolaryngol 1990;110:175-81. 2009;2(4):223-39.
29. Katsarkas A, Smith H, Galiana H. Head-shaking nystagmus
(HSN): The theoretical explanation and the experimental proof. ABOUT THE AUTHORS
Acta Otolaryngol 2000;120:177-81.
30. Hain TC, Fetter M, Zee DS. Head-shaking nystagmus in patients Bachi T Hathiram
with unilateral peripheral vestibular lesions. Am J Otolaryngol
1987;8:36-47. Professor and Head, Department of ENT and Head and Neck Surgery
31. Hain TC, Splindler J. Head-shaking nystagmus. In: Sharde JA, Topiwala National Medical College and BYL Nair Charitable Hospital
Barbo HO (Eds) The vestibulo-ocular reflex and vertigo. Raven Mumbai, Maharashtra, India
Press, New York, 1993. Correspondence Address: Flat No. 2, Ground Floor, MN Banaji
32. Ewald EJ. Physiologische Untersuchungenuber das Endorgan Building, Forjett Street Cross Lane, Mumbai-400036
desNervus Octavus. Bergmann, Wiesbaden, Germany, 1892. Maharashtra, India, Phone: 91-9323699192, 91-9920499192
33. Lasker DM, Hullar TE, Minor LB. Horizontal vestibulo-ocular e-mail: bachi.hathiram@rediffmail.com, orlclinics@gmail.com
reflex evoked by high-acceleration rotations in the squirrel
monkey. III. Responses after labyrinthectomy. J Neurophysiol
Vicky S Khattar
2000;83:2482-96.
34. Asprella-Libonati G. Diagnostic and treatment strategy of the Assistant Professor, Department of ENT and Head and Neck Surgery
lateral semicircular canal canalolithiasis. Acta Otorhino- Topiwala National Medical College and BYL Nair Charitable Hospital
laryngological Italica 2005;25:277-83. Mumbai, Maharashtra, India