Download Acute Dizziness with Hearing Loss Autoimmune Inner Ear Disease

Acute Dizziness with Hearing Loss
Autoimmune Inner Ear Disease:
• Unilateral or bilateral
• Vertigo, lightheadedness, ataxia, motion intolerance
• unilateral/bilateral/fluctuating/progressive SHNL
• fluctuating tinnitus and aural fullness occ present
• symptoms progress over weeks to months
• diagnosis primarily clinical
• symptoms may improve with steroids
• associated w/ systemic autoimmune diseases (15-30%), rarely endocrine disease
and/or recurrent fevers
Tests: NO DEFINITIVE TESTS. Lymphocyte transformation test (controversial), Mitogen
inhibition test (technical difficulties), Circulating Immune Complexes, ESR, CRP, ANA, RF,
TSH, anti-microsomal antibodies, anti-gliadin antibodies (Celiac disease), HLA testing.
Others: FTA-ABS (syphilis), HIV, Hb A1C, and Lyme titers
Cogan’s Syndrome:
• rare inflammatory disease
• ocular: nonsyphilitic ocular interstitial keratitis, scleritis, episcleritis, iritis or
choroiditis
• audiovestibular: sudden SNHL, vestibular impairment
o fluctuating hearing and vestibular function
o variable vestibular impairment (SCCs, otolith organs)
o may appear before or after ocular symptoms (up to 2 yr delay)
o w/o steroids, SNHL may progress
• systemic: pericarditis, vasculitis, fevers, arthralgias, myalgias
• some theories that vaccinations and infections may trigger
Enlarged Vestibular Aqueduct (EVA):
• hearing loss ranges from mild to profound, fluctuating, step-wise progressive or
sudden and can be either Mixed or SNHL
• ? progression of HL from minor head trauma
• vestibular dysfunction ranges from mild imbalance (clumsiness) to vertigo
• unilateral or bilateral
• diagnosis: axial CT or T2 MRI - mid point of vestibular aqueduct >1.5 mm
(Valvassori and Clemis – traditional definition.); Newer criteria: > 1.0 mm at
midpoint, > 2.0 mm at operculum (Vijayasekaran)
• third window effect?
• Histologically – thin walled endolymphatic sacs which are missing the rugal folds
and perisaccular loose vascular tissue seen in normal endolymphatic sacs
• Possible association b/w EVA and Meniere’s.
Tests: VEMPS – lower thresholds, higher amplitudes;
ETD and Middle ear effusion:
• Most common causes of vestibular disturbances in children
• Parents report clumsiness, awkwardness, unsteadiness, frequent falls
• Fluid visible on exam, pneumatic otoscopy w/ limited TM mobility
• Balance disturbance equally likely w/ unilateral effusions as bilateral effusions
• Hearing loss (Conductive)
Tests: ENG – no abnormalities on OKN and Pursuit testing; Spontaneous unidirectional
horizontal nystagmus in 1/3, positional nystagmus in 17.5%
HIV:
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Congenital infection
Chronic or acute vertigo, ataxia, hearing loss (CHL or SNHL), tinnitus
o Hearing loss can be unilateral or bilateral, sudden or progressive
o ABR: increased I-III latencies
ENG: both central and peripheral findings
o Pursuit abnormalities
o Weakness on calorics
Etiology – unknown
o temporal bone studies have shown viral-like particles and inclusion bodies in
the hair cells of the vestibular end organs
o ? if result of antiviral therapy
Labyrinthine Concussion:
• follows head injury (temporo-parietal and occipito-parietal areas)
• headaches, vertigo, tilting sensation, dizziness, nausea/vomiting, visual disturbances,
unsteadiness (sway to affected side), irritability, personality changes, cognitive
impairments, sleep disturbances
• Symptoms present for several days – subside over 4-6 wks
• Recurrent attack of vertigo (5-10 sec) w/ nausea
• Can have positional nystagmus on Dix-Hallpike testing (BPPV)
• Normal hearing
• May have balance problems for many years as seen w/ tandem gait and Romberg w/
eyes closed
Tests:
Dix-Hallpike – positive test for affected ear
Normal rotational chair
Lyme Disease:
• Spirochete Borrelia burgdorferi (BB) spread by tick bite
• First Stage: Erythema chronica migrans
• Second Stage (acute disseminated stage):
o 3-24 months after infection
o Cardiac abnormalities (eg AV block)
o Arthritis/arthralgias
• Third Stage (“neuroborreliosis” – infection of CNS)
o 6 months – 2yrs after tick bite
o Peripheral neuropathies
o Encephalitis, chronic meningoenchephalitis
o Rheumatoid arthritis
o Chronic dermatologic d/o (acrodermatitis chronica atrophicans)
o Headache, fatigue, arthritis fevers, malaise
o Cranial Nerve abnormalities are seen in 5-10%
ƒ Facial N Paralysis 80% of CN abnormalities; can be bilateral
ƒ CN VIII: Vertigo, Unexplained SNHL, Tinnitus, aural fullness
ƒ CN III (oculomotor) and VI (abducens)
• CT normal; MRI – may be normal or may show focal brain inflammation (w/
predilection for white matter) – in cases of encephalitis.
• IgG Antibodies to BB elevated in CSF and serum (past or present infection); CSF
pleocytosis and elevated protein indicate active infection. “titers may be negative in
up to 1/3 of cases” – Controversial. Current thinking is that “seronegative Lyme” is
very rare;
Tests: From one case report only: ENG – saccadic dysmetria, increased latency ; Smooth
pursuits – normal; Calorics – normal;
Meniere’s Disease/ Endolymphatic Hydrops:
• Meniere’s is rare in childhood
• Recurrent paroxysmal vertigo, SNHL (fluctuating to progressive), aural fullness,
tinnitus, n/v and headache
• HL may be high frequency (unlike adults) initially
• FH may be significant for Meniere’s
• Hydrops may also be seen with congenital CMV and syphilis;
o Congenital CMV – vestibular symptoms may not be present for many years;
alternatively – a child may have delay in walking
o Congenital CMV – vestibular function may be normal, may have unilateral or
bilateral peripheral hypofunction
Tests: Calorics – Unilateral weakness (if one ear affected)
Rotational Chair – asymmetry
ECOG – SP:AP ratio >0.45 (seen in 2/3 of pts w/ Meniere’s)
Otitis Media (acute) and Complications: see Miyamoto paper
Ototoxicity:
• Shown to or alleged to produce vestibular ototoxicity: aminoglycosides, loop
diuretics, salicylates, quinidine, barbiturates, various antibiotics (minocycline,
erythromycin, polymixin, chloramphenicol) and certain chemotherapeutic agents
(cisplatin, nitrogen, mustard.)
• Affects both VOR and VSR
• High-freq SNHL (destroys outer hair cells in basal turn of cochlea)
• Disequilibrium, ataxic gait, stumble easily, lose balance w/ quick turns
• Pts may navigate by holding onto wall or furniture
• Oscillopsia w/ severe damage – secondary to loss of VOR; these pts have balance
problems when trying to walk in darkness or on uneven surfaces
Tests: Rotational Chair (VOR) – reduced gains, reduced time constants; CDP – falls on
SOT 6
Perilymph Fistula:
• direct injury to labyrinth or barotrauma
• imbalance, intermittent dizziness, or vertigo
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progressive SNHL
tinnitus
nystagmus
unilateral or bilateral
Positive fistula test
CT may have congenital abnormalities (eg Mondini)
Definitive diagnosis: MEE
Superior Canal Dehiscence Syndrome:
• Vertigo and oscillopsia induced by loud sounds (Tulio phenomenon), pressure
changes in the EAC (Hennebert’s sing) or Valsalva
• Vertical oscillopsia, unsteadiness
• c/o autophonia, hearing pulse or their own eyes move
• vertical-torsional eye movements evoked by sound or pressure stimuli
• Apparent CHL – mimicking otosclerosis: air-bone gaps are greatest at lower
frequencies and bone conduction thresholds “better” than 0 dB
• HRCT – can see dehiscence
Tests: VEMPS – low threshold, high amplitude
Temporal Bone Fractures:
• Transverse fractures may violate IAC, SCCs, vestibule, cochlea or FN
• Hearing loss (usually immediate and severe but can be progressive if PLF or pt
develops endolymphatic hydrops)
• theoretical mechanisms of injury:
o Avulsion of CN VII
o Disruption of membranous labyrinth
o Vascular vasospasm, thrombosis or hemorrhage
o Disruption of endosteum of round or oval window resulting in PLF
o Fracture across vestibular aqueduct – occlusion and secondary endolymphatic
hydrops
• Vertigo, nausea/vomiting
• Spontaneous nystagmus
• HRCT
Usher’s Syndrome:
• Retinitis pigmentosa, bilateral SNHL, ± Vestibular dysfunction, ataxia
• AR
• High degree of clinic heterogeneity
• Type I: profound congential HL, Vestibular dysfunction, retinal degeneration
beginning in childhood
o Type IB: peripheral vestibular dysfunction, absence of VOR
• Type II: mod to severe congenital HL, normal vestibular function, late onset retinitis
pigmentosa
• Type III: progressive HL
• Ophthalmologists – Electroretinography
Tests: Type I: ENG, Rotational Chair – absence of post-rotary nystagmus
Vestibular Neuronitis and Labyrinthitis:
• Sudden, unilateral, peripheral vestibular deficit
• vertigo – lasts for few days; unsteadiness may persist
• onset frequently after a URI
• acute hearing loss (labyrinthitis only)
• horizontal nystagmus beating towards healthy ear
• viral etiology – HSV, measles, mumps
• predominantly damages superior branch of VN
• utricular dysfunction seen in both
• main difference: saccule loss primarily w/ labyrinthitis
Tests: Calorics – decreased response affected ear
Rotational chair – greater h-VOR asymmetry in labyrinthitis than neuritis
VEMP – absent in approximately 1/3 of pts
SVH – deviated from horizontal by the affected side was down; Three months later, this
deviation was greater if VEMPS were absent
Waardenburg’s Syndrome:
• Vestibular complaints in 72% of Type I and II
• Vertigo, imbalance or dizziness
• AD with variable penetrance
• Type I: Hearing loss, dystopia canthorum (lateral displacement of inner canthi),
hypertrichosis of eyebrows, white forelock, skin pigmentary changes, heterochromia
irides,
• Type II: same as Type I except dystopia canthorum
• Of those subjects w/ vesibular c/o: 77% had abnormal vestibulo-ocular tests
(calorics, vestibular autorotation), 57% had abnormal vestibulo-spinal function tests,
and 59% had abnormal ECOG (SP:AP > .40)
• CT: enlargement of the vestibular aqueduct and the upper vestibule, narrowing of the
internal auditory canal porus, and hypoplasia of the modiolus
Whiplash Injury:
• Vertigo, dizziness
• Tinnitus
• ± Hearing loss
• Visual disturbances
• rapid flexion-extension of head results in cervical muscles spasm, impairing blood
flow through the vertebro-basilar circulation
Tests: Positional nystagmus w/ head hyperextended; COR gains increased (likely secondary
to reduced neck movements.)
ENG findings: spontaneous, positional nystagmus, gaze nystagmus, disturbances of smooth
pursuits, and optokinetics. VOR abnormalities on both rotational chair and calorics. SVV
abnormalities and saccadic impairments may be seen up to 1 yr out.
References:
Black, FO et al. A vestibular phenotype for Waardenburg syndrome? Otol Neurotol, 2001;
22(2): 188-94.
Black FO and Pesznecker SC. Vestibular ototoxicity. Oto Clin N Am, 1993. 26: 713-736.
Bovo R, Ciorba A and Alessandro M. The diagnosis of autoimmune inner ear disease:
evidence and critical pitfalls. Eur Arch Otorhinolaryngol, 2009; 266:37-40.
Choung YH, Park K, Kim CH et al. Rare cases of Meniere’s disease in children. J Laryngol
Otol, 2006; 120: 343-352.
Eviatar L, Bergtraum M and Randel MR. Post-traumatic vertigo in children: a diagnostic
approach. Pediatr Neurol, 1986; 2: 61-6.
Golz A, Netzer A, Batia AY, et al. Effects of middle ear effusion on the vestibular system I
children. Oto Head Neck Surg, 1998; 119: 695-9.
Halperin J. Nervous system Lyme disease. Infect Dis Clin N Am, 2008. 22: 261-74.
Huygen PLM and Admiraal RJC. Audiovestibular sequelae of congenital cytomegalovirus
infection in 3 children presumably representing 3 symptomatically different types of delayed
endolymphatic hydrops. Int J Pediatr Otorhinolaryngol, 1996; 35: 143-154.
Ishizaki H, Pyykko I and Nozue M. Neuroborreliosis in the etiology of vestibular neuronitis.
Acta Otolaryngol (Suppl), 1993; 503: 67-9.
Lempert T and von Brevern M. Episodic vertigo. Current Opinion Neurol, 2005; 18: 5-9.
Lyos A, Marsh M, Jenkins H and Coker N. Progressive hearing loss after transverse
temporal bone fracture. Arch Oto Head Neck Surg, 1995; 121: 795-799.
Madden C, Haltstead MJ, Hopkin RJ et al. Temporal bone abnormalities associated with
hearing loss in Waardenburg syndrome. Laryngoscope, 2003; 113: 2035-41.
Maire R and Melle GV. Horizontal vestibulo-ocular reflex dynamics in acute vestibular
neuritis and viral labyrinthitis: evidence of otolith-canal interaction. Acta Otolaryngol,
2004; 124: 36-40.
Miyamoto RC and Miyamoto RT. Pediatric Neurotology. Seminars in Ped Neurol, 2003;
10: 298-303.
Montfoort I, Van der Geest J, Slijper H et al. Adaptation of the cervico- and vestibulo-ocular
reflex in whiplash injury patients. J Neurotrauma, 2008. 25: 687-93.
Murofushi T, Halmagyi M, Yavor R and Colebatch J. Absent vestibular evoked myogenic
potentials in vestibular neurolabyrinthitis. Arch Oto Head Neck Surg, 1996; 122: 845-848.
Murofushi T, Ushio M, Takai Y et al. Does acute dysfunction of the saccular afferents affect
the subjective visual horizontal in patients with vestibular neurolabyrinthitis? Acta
Otolaryngol, 2007; 127: 61-4.
Ndiaye I, Rassi S, Wiener-Vacher S. Cochleovestibular impairment in pediatric Cogan’s
syndrome. Pediatrics, 2002; 109(2):
Parnes L and McCabe BF. Perilymph fistula: an important cause of deafness and dizziness
in children. Pediatrics, 1987; 80: 524-8.
Palacios G, Montalvo M, Fraire M et al. Audiologic and vestibular findings in a sample of
Human Immunodeficiency Virus type-1-infected Mexican children under highly active
antiretroviral therapy. Int J Ped Otorhinolaryngol, 2008; 72: 1671-81.
Sheykholeslami K, Schmerber S, Kermany M and Kaga K. Vestibular-evoked myogenic
potentials in three patients with large vestibular aqueduct. Hear res, 2004; 190: 161-8.
Soliman A. Immune-mediated inner ear disease. Am J Otol, 1992; 13(6): 575-579.
Spiegel J and Lalwani A. Large vestibular aqueduct syndrome and endolymphatic
hydrops: two presentations of a common primary inner-ear dysfunction? J Laryngol Otol,
2008; 12: 1-3.
Teschner M, Neuburger J, Gockeln R et al. “Minimized rotational vestibular testing” as a
screening procedure detecting vestibular areflexy in deaf children: screening cochlear implant
candidates for Usher syndrome Type I. Eur Arch Otorhinolaryngol, 2008; 265: 759-763.
Vibert D and Hausler R. Acute peripheral vestibular deficits after whiplash injuries. Ann
Otol Rhinol Laryngol, 2003; 112: 246-51
Vijayasekaran S, Halsted M, Boston M et al. When is the vestibular aqueduct enlarged? A
statistical analysis of the normative distribution of vestibular aqueduct size. AJNR, 2007;
28(6): 1133-8.
Zhou G, Gopen Q and Poe D. Clinical and diagnostic characterization of canal dehiscence
syndrome: a great otologic mimicker. Otol Neurotol, 2007; 28: 920-6.
Acute Dizziness without Hearing Loss
Benign Paroxysmal Positional Vertigo (BPPV):
• Classic description is of the posterior canal
• Sudden attacks of vertigo, nausea and vomiting triggered by head movements
• Dix-Hallpike maneuver: latency of few seconds before onset of rotary nystagmus.
Nystagmus is of limited duration and fatigues w/ repeated provocation
• Uncommon in children
Migraines/Migraine Variants:
• Most common etiology of pediatric dizziness
• Paroxysmal ischemia of the vestibular nuclei
• Infants: Paroxysmal Torticollis
o Avg age 18 mo
o Ischemia of Inferior VN
o w/ or w/o pallor, vomiting or behavioral changes
o Episodic torticollis lasts between 4 hrs and 4 days
o Frequency and duration of episodes decline as child gets older
• Young Children: Benign Paroxysmal Vertigo of Childhood (BPV)
o Begins before age 5 and resolves within 2 yrs of onset
o Rotary vertigo lasting seconds to minutes (1-5 min)
o Autonomics signs/symptoms: pallor, nausea, vomiting, sweating
o No headaches, tinnitus or hypoacusis
o Sometimes fear, inconsolable crying
o Not associated w/ head position
o Resolves spontaneously and may occur several times/wk
• Older Children: Paroxymal Vertigo/Migraine Variant:
o Mean age of onset 8 yrs
o Vertigo lasting minutes
o Pallor, nausea, feeling unwell, noise intolerance, light intolerance, anorexia,
and vomiting
o Positive FH of migraines
o Some also have other migraine related symptoms: abdominal migraines,
recurrent limb pain, cyclic vomiting
o Triggers: fatigue, stress, missing a meal, travel, climate, bright lights, lack of
sleep
o symptoms often improve or resolve w/ sleep
• Older Children and Adolescents: Vertebrobasilar migraine/Basilar A. Migraine:
o vertigo, tinnitus, decreased hearing, ataxia, dysarthria, visual symptoms,
diplopia, paresthesias, headaches, decreased level of consciousness
Postural Orthostatic Tachycardia Syndrome (POTS):
• Symptoms: near syncope, lightheadedness, vertigo, TIAs, tachycardia, weakness,
fatigue, exercise intolerance
• symptoms caused by transient alterations in autonomic tone that produce
neurocardiogenic hypotension and bradycardia
• Children and adolescents
Tests: Tilt table testing
Post-traumatic Epilepsy/ Epileptic Vertigo:
• 5-7% of closed heard injuries
• damage to superior temporal lobe, middle temporal gyri and supramarginal and
angular gyri
• vertigo or dizziness
• normal hearing
• central vestibular system: frontal, parietal and temporal lobes; these areas are
multisensory integration units rather than pure sensory cortex
• Epileptic discharges may present as vertigo
• Seizure may stem from pursuit eye movements rather than vestibular dysfunction
Tests:
EEG – abnormal; may need to do in sleep deprived state (document temporo-parietal spikes)
ENG – directional preponderance ± labyrinthine preponderance
Vestibular Neuronitits
See vestibular labyrinthitis in previous section
References:
Versino M, Sances G, Anghileri E et al. Dizziness and migraine: a causal relationship?
Functional Neurology, 2003; 18(2): 97-101.
Al-Twaijri W and Shevell M. Pediatric migraine equivalents: occurrence and clinical
features in practice. Pediatr Neurol, 2002; 26:365-8.
Bachor E, Wright CG, and Karmody CS. The incidence and distribution of copular deposits
in the pediatric vestibular labyrinth. Laryngoscope, 2002; 112: 147-151.
Karas B, Grubb B, Boehm K and Kip K. The postural orthostatic tachycardia syndrome: a
potentially treatable cause of chronic fatigue, exercise intolerance and cognitive impairment
in adolescents. Pace, 2000. 23: 344-351.
Kluge M, Beyenburg S, Fernadez G and Elger C. Epileptic vertigo: Evidence for vestibular
representation in human frontal cortex. Neurology, 2000; 55: 1906-8.
Chronic dizziness with hearing loss
Autoimmune:
See acute dizziness with hearing loss section
Otitis Media (Complications):
See acute dizziness with hearing loss section
Ototoxicity:
See acute dizziness with hearing loss section
Acoustic Neuroma (Retrocochlear Lesions):
• Most common posterior fossa tumor in children
• Associated with NF II -- Diagnostic criteria: (from Miyamoto)
o Bilateral vestibular schwannomas
o Parent, sibling or child w/ NF2 plus
ƒ Unilateral vestibular schwannoma
ƒ Meningioma, glioma, neurofibroma or posterior subcapsular lens
opacity
o Unilateral vestibular schwannoma plus one in second entry above
o Multiple meningiomas plus unilateral vestibular schwannoma, glioma,
neurofibroma, schwannoma, posterior subcapsular lens opacity, cerebral
calcifications
• Asymmetrical hearing loss – high-freq SNHL
• Speech Discrim disproportionate to PTA
• ABR: abnormal waveform or wave V latency ≥ 0.2 msec
• MRI w/ Gad – detection of tumors as small as 2 mm
References:
Miyamoto RC and Miyamoto RT. Pediatric Neurotology. Seminars in Ped Neurol, 2003;
10: 298-303.
Chronic dizziness without hearing loss
Chiari I Malformation:
• Common clinical manifestations: torticollis, opisthotonus (titanic spasm of back
muscles – trunk is arched forward, head and lower limbs backward), headaches, vocal
cord paralysis, apnea, swallowing difficulties, ataxia,
• Variable vestibular symptoms and signs: oscillopsia, diplopia, blurred vision and
vertigo
• Horizontal or downbeat (most common) nystagmus, jerky pursuit, Abducens palsy,
increased blind spot; other types of nystagmus may also be seen
• The result of oculomotor dysfunction and central vestibular dysfunction (including
vestibulocerebellum)
• Diagnosis: MRI – herniation of cerebellar tonsils
• Significant improvement and/or resolution of symptoms w/ posterior fossa
decompression
Multiple Sclerosis:
• Progressive Demyelinating disease characterized by recurrent episodes of
demyelination separated by time and space
• Course and severity – highly variable
• Mean age of onset - 28 yrs
• Women > Men
• Through course of disease can affect vestibulo-spinal tract
• Disequilibrium, balance dysfunction, dizziness, ataxia, nausea/vomiting, double
vision, loss of vision, Intranuclear ophthalmoplegia, nystagmus
• Variable other neurologic symptoms
• MRI – white matter changes
Tests: VEMP – prolonged p13 and N23 latencies; SVV – many will have large deviations
even w/o vestibular symptoms;
Ocular Abnormality:
• In one study, 5% of 523 children referred for vestibular testing had only an ocular
disorder
• Sx usually present in children > 6yrs (activities involving prolonged attention)
• Sx at end of day or w/ fatigue, w/ prolonged use of computers/televisions, sometimes
related to walking or rapid head movements
• Sx: sensation of rotation (vertigo), displacement of the environment, rolling,
headaches, nausea/vomiting,
• Diagnoses: Refractive errors (myopia, hyperopia or astigmatism), amblyopia (
unilateral loss of vision without an organic lesion), latent strabismus, convergence
insufficiency
Tests: vestibular testing – normal in 2/3. Subtle VOR anomalies – 1/3 borderline normal
Post-concussive:
See labyrinthine concussion in acute dizziness with hearing loss section
Familial Periodic Cerebellar Ataxia/Vertigo, Episodic Ataxia Type 2:
• AD – variable clinical phenotype b/w and within families
• Bouts of vertigo, ataxia, dysarthria, nystagmus, nausea, vomiting and fatigue.
• Lasts ½ hour to 2 hrs
• About half of pts have associated migraine headaches
• Some have hemiplegia, epilepsy
• Triggers: emotional stress, exercise; occasionally: heat, coffee, small amts of
alcohol, bright sunshine
• Symptoms relieved by sleep
• Onset before age 20 (mean age 12
• Neuro exam – normal to cerebellar ataxia
• Mutation in CACNA1A gene on chromosome 19p13 (sequence analysis – diagnostic)
• MRI: normal to atrophy of cerebellar vermis
• May be due to defect in cerebella pH homeostasis
Tests: Oculomotor studies: interictal gaze-evoked, rebound and downbeat nystagus;
abnormal saccades, ocular fixation and postural stability; may see square-wave jerks with
lateral gaze, hypometric saccades, and both horizontal and vertical components during
diagonal saccades. Reduced saccadic peak velocity may be earliest manifestation.
Vertebro-basilar insufficiency:
• Lateral medullary infarction (Wallenberg’s Syndrome)/cerebellar infarction
o Occlusion of vertebral a or PICA or one of it’s branches
• Can also occur secondary to arterial dissection
• Vertigo, ataxia, dysarthria and headache
• Diverse types of nystagmus: horizontal, horizonto-rotary, torsional, see-saw and
others have been described.
o Wallenberg: Eyes open, horizontal nystagmus beats away from side of lesion
• Klippel-Feil – recurrent vertebrobasilar embolism
Tests: Wallenberg’s Syndrome – one case report of VEMP – normal latencies but
contralateral large amplitude VEMP, Calorics – normal, Oculomotor testing – dysmetria
(saccades – overshoot to side of infarct and undershoot to contralateral side; smooth pursuits
– mildly saccadic in nature), CDP – falls on conditions 5 and 6.
References:
Alpini D, Pugnetti L, Caputo D et al. Vestibular evoked myogenic potentials in multiple
sclerosis: clinical and imaging correlations. Mult Scler, 2004; 10: 316-21.
Anoh-Tanon MJ, Bremond-Gignac D and Wiener-Vacher SR. Vertigo is an underestimated
symptom of ocular disorders: dizzy children do not always need MRI. Ped Neurol, 2000;
23: 49-53.
Crevits L, Venhovens J, Vanoutive J and Debruyne J. False perception of visual verticality
in multiple sclerosis. Eur J Neuro, 2007; 14: 228-232.
Harno H. Migraine and periodic ataxia type 2: A clinical, genetic, neurotologic and
magnetic resonance spectroscopy study. Academic Dissertation – Dept of Neurology,
Helskinki University Central Hospital. University of Helsinki, Finland. 2005.
Korres S, Balatsouras D, Zournas C et al. Periodic alternating nystagmus associated with
Arnold-Chiari malformation. J Laryngol Otol, 2001; 115: 1001-4.
Liebenberg WA, Georges H, Demetriades AK and Hardwidge C. Does posterior fossa
decompression improve oculomotor and vestibulo-ocular manifestations in Chiari 1
malformation? Acta Neurochir (Wien), 2005; 147: 1239-40.
Lundy L, Zapala D and Olsholt K. Dorsolateral medullary infarction: a neurogenic cause of
a contralateral, large-amplitude vestibular evoked myogenic potential. J Am Acad Audiol,
2008; 19: 246-256.
Silverboard G and Tart R. Cerebrovascular arterial dissection in children and young adults.
Sem Ped Neurol, 2000; 7: 289-300.
Tusa R, Saada A and Niparko J. Dizziness in childhood. J Child Neurol, 1994; 9: 261-74.
Williams B, Skinner H, and Maria B. Increased intracranial pressure in a case of pediatric
multiple sclerosis. J Child Neurol, 2008; 23: 699-702.
Bedside Exam
• Basic head and neck exam
• Cranial nerves
• Ears
o Fistula test (symptoms and nystagmus with pneumatic otoscopy)
o Tuning forks
• Eyes
o EOM (30° R and L)
o Spontaneous nystagmus – w/ and w/o vision
ƒ Pt fixates on target about 4 ft away – look for nystagmus
ƒ W/ VNG googles –monitor for nystagmus
• Head Impulse Test (Halmagyi head thrust)
o Gently grasp pts head on both sides and tilt forward 30° (LSCC coplanar w/
ground)
o Ask pt to focus on your nose as examiner turns head 15-20 degrees to one
side then other rapidly and abruptly (>2000 deg/sec) – observe eyes
ƒ Normal – eyes move almost exactly 180° out of phase w/ head
ƒ Peripheral vestibular d/o on left side – head turned to left, eyes do a
catch up saccade to the right
• Head Shake
o Pt wears VNG or Frenzels (eliminate visual fixation)
o Pt shakes his/her head back and forth for 10-30 cycles – look for post-head
shake nystagmus (transient nystagmus)
ƒ Peripheral lesions – fast phase of nystagmus toward contralesional ear
ƒ Central lesions – prolonged, vertical or disconjugate nystagmus
• Valsalva-induced nystagmus
o VNG or Frenzels
o Valsalva 1: increase air pressure in sinuses and ME: pt takes a deep breath,
pinches nose and closes mouth tightly – then blows as if equalizing pressure
in the ears when descending in an airplane
o Valsalva 2: increase venous pressure in cranium: strain against a closed
glottis and lips as if pressuring lungs to stabilize trunk while lifting a hvy
weight
o Nystagmus seen w/ Arnold-Chiari malformation, PLF and SCCD
ƒ Fast phase towards site of lesion
• Cerebellar Signs
• Dix-Hallpike
• Gait
• Tandem gait
• Romberg
• Fukuda Stepping Test
o Pts feet should be shoulder width apart, eyes closed and arms outstretched.
o Have pt march in place – bringing knees as high as poss
o Peripheral vestibular d/o: Turn 45° toward site of lesion
Jacobson, G and Shepard N. Balance function assessment and management. San Diego:
Plural Publishing 2008.