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7/23/2013 Mark Howell, MD, FAC S Ear,Nose andThroat Associates Johnson City ,Tn. Michelle Tanner, MS4 Quillen College of Medicine Disclosures  I declare that I do not have any relevant financial interest or other relationship with a commercial entity regarding the topic of vertigo in children  I do not have any financial arrangements or affiliations with a commercial entity 1 7/23/2013 Learning Objectives  Basic understanding of vestibular system  Review of pertinent anatomy and function of the vestibular system  Pediatric approach to diagnosis of vertigo  Understand various pediatric etiologies and presentations of vertigo  Be aware of physical exam maneuvers used  Overview of more formal vertigo testing Clinical Scenario  HPI: 8 yo F with 7 days of vertigo  Described as “room spinning around her” or “riding a roller coaster”  Lasted 30 minutes, during which she laid down  Occurred three times over last week  Sudden onset with nausea and balance difficulty  Teacher reported “her eyes moved funny”  No auras, tinnitis, hearing loss, visual field changes, diplopia or loss of consciousness http://www.pediatriceducation.org/ Clinical Scenario  PMHx: healthy, immunizations UTD  FHx: +migraines and vertigo (mother)  No history of hearing loss, deafness or neurological disease  SHx: Doing well in school  ROS: negative except for URI 3 weeks ago  No fevers, chills, or cold sores http://www.pediatriceducation.org/ 2 7/23/2013 Clinical Scenario  Pertinent PE:  General: well appearing, growth parameters 25‐50%  HEENT: left ear +serous fluid, PERRLA  Neuro: EOMs intact, DTRs 2+/2+, negative Babinski, normal rapid alternating movements, finger to nose testing, tandem gait and Romberg testing; no nystagmus could be elicited  Diagnosis??? http://www.pediatriceducation.org/ Definitions  Dizziness: altered perception of position in the environment; can represent vertigo, presyncope or dysequilibrium  Vertigo  illusion of movement, most often sensation of rotation; less frequently, linear displacement or tilt  key symptom of vestibular pathology  often accompanied by nausea, vomiting, pallor and perspiration  Vertigo differs in children:  Often ignored  Communication difficulties  Compared to adult disease processes, frequency differs  Prevalence:  Estimated 15% of school age children with at least one attack of vertigo over the last 12 months 3 7/23/2013 Relative Frequencies of Vertigo Syndromes in Children and Adults Jahn K. Vertigo and balance in children – diagnostic approach and insights from imaging. Eur J Paediatr Neurol. 2011 Jul;15(4):289‐294 Anatomy and Function 4 7/23/2013 Vestibular System  Provides orientation of the body with respect to gravity  Enables balanced movement  Ensures gaze stabilization  Alters homeostatic milieu after body reorientation Vestibular System  Structurely fully developed sensory systems at birth  Balance function matures with sequential acquisitions of motor milestones in infancy(head control;sitting;standing;walking)  Experiential learning and adaptation into adolescence  Reflexes:Moro;Tonic neck;head righting;parachute;Doll’s eyes  Most mature 5‐6 months Vestibular System  Responsible for sensing and controling motion  3 semicircular canals  2 otolith organs(utricle and saccule) 5 7/23/2013 Semicircular Canals  3 matched canals  90 degree angle relationship to each canal  Superior,Posterior,Lateral  Respond to angular acceleration 6 7/23/2013 Semicircular Canals  3 matched canals  90 degree angle relationship to each canal  Superior,Posterior,Lateral  Respond to angular acceleration 7 7/23/2013 Otolith Sensitivity  Saccule  Utricle  Linear acceleration and head tilt to gravity 8 7/23/2013 Balance System  Vestibular  Visual  Sensory/Proprioceptive CNS  Afferents all systems vestibular,visual and sensory/proprioceptive integrate  Output from brainstem control motor responses to maintain balance compensate for head motion and position CNS  Vestibular‐occular control  Vestibulo‐spinal tract 9 7/23/2013 Maintenance of balance Higher centers : * Extra pyramidal system * Cerebellum * Reticular formation ( Effector pathways ) (Sensory systems) Vision Proprioception Brain stem integrating center (Vestibular nuclei) Vestibular labyrinths Perception of orientation (in Vestibular cortex) Oculomotor system (Vestibulo‐ocular reflex) Antigravity muscles controlling posture & gait (Vestibulo spinal reflex) Pathophysiology  Balance requires –  Normal functioning vestibular system  Input from visual system (vestibulo‐ocular)  Input from proprioceptive system (vestibulo‐spinal)  Central causes compromise central circuits that mediate vestibular influences on posture, gaze control, autonomic fx  Disruption of balance between inputs results in vertigo  Goal of treatment: restore balance between different inputs 10 7/23/2013 Pathophysiology  Vestibular system influences autonomic system  Intimate linkage in brainstem pathways between vestibular and visceral inputs  Alteration of vestibular inputs results in:  nausea, vomiting  Pallor  Respiratory/circulatory changes Components of History and Physical Exam History  “playground synonyms”‐true vertigo?  Hearing loss or change (intolerance noise)  Loss of Consciousness, mental status changes,       headache, neurologic (ataxia,cranial nerve changes) Trauma –head or middle ear and neck Infection‐fever, otitis media, viral illness Recurrence vertigo Use of Validated Structured Case Histories Family history migraine Drug ingestion 11 7/23/2013 Ravid and Colleagues. A simplified diagnostic approach to dizziness in children. Pediatr Neurol 2003;29(4):317‐20             Vertigo Acute Paroxysmal Hearing loss Change of symptoms with head position Headache Fever Vomiting Anxiety Depression Change in consciousness Head trauma         Drugs Family medical history Hearing loss Migraine Seizures Dizziness Chronic Continuous  Age (>5, <5)  Neuro exam: nl vs. abnl  PE: nl vs. abnl Ravid and Colleagues. A simplified diagnostic approach to dizziness in children. Pediatr Neurol 2003;29(4):317‐20 Specific Physical Exam Findings  Acute otitis media findings or mastoiditis  TM retraction pocket with middle ear mass(cholesteatoma)  Hemotympanum,otorrhea,perforation(trauma)  Hearing loss  Vertigo or nystagmus from loud noise or with pneumatic otoscopy of intact TM(Tullio phenomenon)  Vesicles on auricle(Ramsey Hunt)  Nystagmus 12 7/23/2013 Physical Examination Informal Formal  Nystagmus  Video‐Oculography  Vestibulo‐Ocular Reflex  Computerized Rotary Chair  Head‐Thrust Testing  Computerized Dynamic Posturography  Dynamic Visual Acuity  Dix‐Hallpike  Vestibular Evoked Myogenic Potentials  Postural Stability Nystagmus  Observed movement of the eye in response to stimulating the labyrinth, retro‐cochlear vestibular or central vestiulo‐ocular pathway.  Classified by:  Spontaneous or gaze‐evoked  Direction (fast component)  Fatigability *Observe for conjugate gaze Search for spontaneous and gaze‐evoked nystagmus. Frenzel glasses (high diopter lenses) prevent fixation and aid in identification of nystagmus. 13 7/23/2013 Nystagmus Peripheral Direction Sometimes reverses Unidirectional, fast direction when patient component toward the normal ear; never reverses looks in the direction of slow component direction Type Horizontal with a torsional component, never purely torsional or vertical Central Can be any direction Effect of visual fixation Suppressed Not suppressed Other neurologic signs Absent Often present Postural instability Unidirectional instability, Severe instability, patient walking preserved often falls when walking Deafness or tinnitus May be present Absent Vestibulo‐Ocular Reflex  Purpose is to stabilize gaze and maintain clear vision when the body or head is in motion  Objects of interest are maintained on the fovea of the retina through inputs from the semicircular canals and otolith organs  “head‐shake nystagmus” 14 7/23/2013 Semicircular Canal → Vestibular Nuclei → Oculomotor Nuclei III and VI → Vestibulo‐Ocular Reflex Dynamic Imbalance: Head‐Thrust Testing 15 7/23/2013 Dynamic Visual Acuity  Modification of the typical Snellen Eye Chart  Reads smallest line possible  Attempts again to read during back and forth rotation of the head  Loss of one line = insignificant  Loss of 3 lines = VOR deficiency Dix‐Hallpike 16 7/23/2013 Screening Postural Stability  Romberg Maneuver  Tandem Romberg  Unterberger‐Fukuda Test http://www.youtube.com/watch?feature=player_detailpage&v=atXCNq_CgHk Video‐Oculography  Infrared cameras embedded  Battery of tests  Central vs. Peripheral determination 17 7/23/2013 Computerized Rotary Chair  Clarification of questionable VOG  Used if caloric irrigations not tolerated  Assessment of the VOR at multiple test frequencies Computerized Dynamic Posturography Dizziness Differential  Life‐threatening conditions      Head trauma 1 Central nervous system infection 2 Intracranial tumor or abscess Stroke Drug overdose and other poisons  Other conditions         Vestibular neuritis Benign paroxysmal positional vertigo Meniere disease Perilymphatic fistula Seizures Ramsay Hunt syndrome Multiple sclerosis Congenital defects  Common conditions  Otitis media 1  Migraine syndromes 2  Benign paroxysmal vertigo of     childhood (BPVC) 3 Adverse effects of medications Motion sickness Paroxysmal torticollis of infancy Pseudovertigo 18 7/23/2013 Cochlear‐Vestibular Integrity  Accurate audiometric testing critical to assess integrity of VIII cranial nerve and membranous labyrinth  Violation of cochlea or vestibular system can be anticipated with auditory dysfunction True vertigo* Pseudovertigo Benign paroxysmal positional vertigo Arrhythmia Benign positional vertigo of childhood Anemia Cholesteatoma CNS infection• Congenital defectsΔ Head trauma Anxiety Depression Heat illness Hyperventilation Labyrinthitis (Vestibular neuritis) Hypoglycemia Mastoiditis Orthostatic hypotension Meniere disease Poisoning or adverse effect of medication Middle ear trauma Migraine Motion sickness Multiple sclerosis Otitis media Perilymph fistula Pregnancy Presyncope Visual disturbances Poisoning or adverse effect of medication Ramsay Hunt syndrome Seizure Stroke Dizziness Differential  Life‐threatening conditions  Head trauma  Central nervous system infection  Intracranial tumor or abscess  Stroke  Drug overdose and other poisons  Common conditions  Otitis media  Migraine syndromes  Other conditions  Vestibular neuritis  Labryinthitis  Benign paroxysmal positional vertigo  Meniere disease  Perilymphatic fistula  Seizures  Ramsay Hunt syndrome  Multiple sclerosis  Congenital defects  Motor/Developmental delay  Benign paroxysmal vertigo of childhood (BPVC) Adverse effects of medications Motion sickness  Paroxysmal torticollis of infancy  Pseudovertigo   19 7/23/2013 True Vertigo Pseudovertigo 20 7/23/2013 Most Common Pediatric Disorders Balatso Bower Choun D'Agos Erbek Ravid Riina et 23 3 uras et & g et tino, 1 et et al, 200 9 16 17 21 al, 200 al, 200 5 al, 200 Cotton, al, 200 997 10 7 1995 3 6 3 Weisle Wienerder & Vacher, 24 8 Fife, 2 2008 001 % 54 34 31 >2000 Migraine n (%) 116 16.89 11 (20.4) 4 (11.8) 17 (30.9) 15 (5.4) 17 (34) 24 (39) 17 (14.3) 11 (35.5) 25% BPVC n (%) 133 19.36 9 (16.7) 5 (14.7) 14 (25.5) 60 (21) 6 (12) 6 (19.4) 20% Otitis media n (%) 22 3.20 5 (9.2) 5 (14.7) x Viral n (%) 98 infection 14.26 15 (27.7) 4 (11.8) 1 (1.8) 53 (18.8) Trauma 14.99 3 (5.5) 3 (8.8) 85 (30.3) Summary of studies Total subjects 687 n (%) 103 55 4 (7.3) 282 50 62 10 (16) 23 (19.3) x 2 (4) 119 12 (10.1) 9 (14) 14 (11.8) 5% 2 (3) 6 (5) 10% McCaslin DL, Jacobson GP, Gruenwald JM. The predominant forms of vertigo in children and their associated findings on balance function testing. Otolaryngol Clin North Am. 2011 Apr;44(2):291‐307 Migraine‐Associated Vertigo  Most common diagnosis in children with vertigo and      dizziness Family History Key 50% of dizzy children also have headache Temporal relationship is variable 20% of children with migraine suffer dizziness Basilar Migraine variant  3‐19% with migraine, usually < 7 years old  Aura: audio vestibular with tinnitus, loss of hearing acute imbalance and vertigo 21 7/23/2013 Migraine‐Associated Vertigo  Pathophysiology: currently unresolved  Theories:  asymmetric activation of brainstem vestibular nuclei  Defective calcium channels shared by brain and the inner ear, with spreading cortical depression Migraine‐Associated Vertigo  Balance Function Findings  Central and Peripheral Impairment  Vestibular manifestations varied and included spontaneous positional nystagmus, post head shaking nystagmus, BPPV, vibration induced nystagmus, absence of vestibular evoke myogenic potentials and unilateral or bilateral caloric reductions Migraine‐Associated Vertigo  Treatment  Vertigo does not respond well to acute therapy with triptans or NSAIDs  Clinical studies in children are sparse  Magnesium‐aspartate  Topiramate 22 7/23/2013 Benign Paroxysmal Vertigo of Childhood  Episodic attacks of vertigo lasting from seconds to minutes with nystagmus  Typically < 4 years old, rarely after age 8  Migraine equivalent as recognized by International Headache society  Sensation of spinning  Without loss of consciousness  Complete recovery  Unable to stand unsupported Benign Paroxysmal Vertigo of Childhood  Criteria for BPVC:  3 transient episodes  Sensation of rotation  Interferes with normal activity  No loss of consciousness  No neurologic auditory abnormality Benign Paroxysmal Vertigo of Childhood  Pathophysiology: unknown but strong supporting evidence for migraine headache variant  Many children develop migraine later in life  Presumed episodic vasospasm  Vascular disturbance of the posterior circulation with effects on the vestibular nuclei 23 7/23/2013 Benign Paroxysmal Vertigo of Childhood  Diagnosis: depends on characteristic history, usually family history of migraine present  Balance Function Findings: great variability  Treatment: resolves with time Trauma  500,000 ED visits per year  Vestibulopathy secondary to traumatic brain injury or labyrinthine concussion  Incidence of dizziness, anywhere from 18‐78% May even follow minor head injury Trauma  Mechanisms:  Direct trauma to eighth nerve complex and root entry zone at brainstem; Labyrinthine concussion  Disruptions of microcirculation of the vestibule with resultant hemorrhage and inflammation  Pressure wave may disrupt membranous labyrinth 24 7/23/2013 Trauma  Direct injury to tympanic membrane and ossicles with disruption integrity of oval window or round window ie:perilymph fistula Trauma  Perilymphatic fistula:  More frequent in children  Caused by minor head trauma  Sudden onset of hearing loss and vertigo  Round or oval window rupture, creating a fistula Trauma  Balance Function Testing: results rarely reported  Sustained head trauma refer for hearing evaluation  Spontaneous and/or positional nystagmus  Children usually recover, may develop BPPV 25 7/23/2013 Vestibular Neuritis  Post‐viral origin  Primarily adolescents  Symptoms: sudden onset vertigo, nausea, vomiting with postural instability  Spontaneous nystagmus  No hearing loss Vestibular Neuritis  Caloric Examination: gold standard  Electronystagmography  Episodes self limiting, with decreasing intensity Labyrinthitis  Preceding infection  Acute, unilateral  Rotary vertigo for days, N/V  Falls to affected side  Hearing loss 26 7/23/2013 Labrynthitis  Findings:  Rotary horizontal nystagmus  Head Impulse Test  Caloric Testing  Treatment  Vestibular suppressants Otitis Media  “unsteadiness” and “clumsy”  Etiology:  Serous labyrinthitis  Middle ear pressure changes  Typical otoscopic appearance  Abnormal tympanograms  Mild to moderate hearing loss Otitis Media  Abnormal electronystagmography  Treatment: antibiotic therapy  Myringotomy with tympanostomy tube if worsening vestibular symtoms ,mastoiditis,or unresponsive to medical therapy 27 7/23/2013 Otitis Media and Retraction Pocket ETD and Retraction Pocket Summary  History may be difficult to obtain ; infrequent complaint Hearing; trauma; age ;fever ;pattern; migaine Hx Exam :nystagmus; ear findings; neuro findings Testing: audiogram; eyes/nystagmus Vestibular testing :difficult ; equipment adapted from adult studies(normative data limited)  Most vestibular illnesses self limited  Must identify and differentiate the more serious causes     28 7/23/2013 Summary  Vestibular system impairment needs to be documented  Appropriate rehabilitative therapy prescribed  Guides management and treatment  Important to recognize vestibular dysfunction in children  May lead to slowed normal locomotor development via motor incoordination and visual disturbances Back to Clinical Scenario  HPI: 8 yo F with 7 days of vertigo  Described as “room spinning around her” or “riding a roller coaster”  Lasted 30 minutes, during which she laid down  Occurred three times over last week  Sudden onset with nausea and balance difficulty  Teacher reported “her eyes moved funny”  No auras, tinnitis, hearing loss, visual field changes, diplopia or loss of consciousness http://www.pediatriceducation.org/ 29 7/23/2013 Clinical Scenario  PMHx: healthy, immunizations UTD  FHx: +migraines and vertigo (mother)  No history of hearing loss, deafness or neurological disease  SHx: Doing well in school  ROS: negative except for URI 3 weeks ago  No fevers, chills, or cold sores http://www.pediatriceducation.org/ Clinical Scenario  Pertinent PE:  General: well appearing, growth parameters 25‐50%  HEENT: left ear +serous fluid, PERRLA  Neuro: EOMs intact, DTRs 2+/2+, negative Babinski, normal rapid alternating movements, finger to nose testing, tandem gait and Romberg testing; no nystagmus could be elicited  Diagnosis???  Serous Otitis Media with probable Benign Paroxysmal Vertigo of childhood http://www.pediatriceducation.org/ References  McCaslin DL, Jacobson GP, Gruenwald JM. The predominant forms of vertigo in children and their associated findings on balance function testing. Otolaryngol Clin North Am. 2011 Apr;44(2):291‐307  Jahn K. Vertigo and balance in children‐‐diagnostic approach and insights from imaging. Eur J Paediatr Neurol. 2011 Jul;15(4):289‐94. doi: 10.1016/j.ejpn.2011.04.010. Epub 2011 May 14. Review.  O'Reilly R, Grindle C, Zwicky EF, Morlet T. Development of the vestibular system and balance function: differential diagnosis in the pediatric population. Otolaryngol Clin North Am. 2011 Apr;44(2):251‐71 30 7/23/2013 References  O'Reilly RC, Greywoode J, Morlet T, Miller F, Henley J, Church C, Campbell J, Beaman J, Cox AM, Zwicky E, Bean C, Falcheck S. Comprehensive vestibular and balance testing in the dizzy pediatric population. Otolaryngol Head Neck Surg. 2011 Feb;144(2):142‐8.  Benun J. Balance and vertigo in children. Pediatr Rev. 2011 Feb;32(2):84‐5. doi: 10.1542/pir.32‐2‐84. Review.  Valente LM. Assessment techniques for vestibular evaluation in pediatric patients. Otolaryngol Clin North Am. 2011 Apr;44(2):273‐90,  MacGregor DL. Vertigo. Pediatr Rev. 2002 Jan;23(1):10‐6 Questions?? 31