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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: • • • • 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% Facial N Paralysis 80% of CN abnormalities; can be bilateral CN VIII: Vertigo, Unexplained SNHL, Tinnitus, aural fullness 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 • • • • • • • 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 Unilateral vestibular schwannoma 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 Pt fixates on target about 4 ft away – look for nystagmus 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 Normal – eyes move almost exactly 180° out of phase w/ head 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) Peripheral lesions – fast phase of nystagmus toward contralesional ear 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 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.