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Otolaryngologic Manifestations of Arnold-Chiari Malformation. Syboney Zapata, M.D., F.A.A.P. Pediatric Otolaryngology Austin Ear, Nose, & Throat Clinic. Objectives. Recognize symptoms of ACM pertinent to ENT Discuss differential diagnosis Review diagnostic studies.

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Otolaryngologic Manifestations of Arnold-Chiari Malformation

Syboney Zapata, M.D., F.A.A.P.

Pediatric Otolaryngology

Austin Ear, Nose, & Throat Clinic

objectives
Objectives
  • Recognize symptoms of ACM pertinent to ENT
  • Discuss differential diagnosis
  • Review diagnostic studies
arnold chiari malformation
Arnold-Chiari Malformation
  • Herniation of cerebellar tonsils through foramen magnum
  • Neuronal impairment of brainstem, upper spinal cord, & cranial nerves
symptoms of acm pertinent to the otolaryngologist
Symptoms of ACM Pertinent to the Otolaryngologist

Auditory/vestibular dysfunction

Vocal cord paralysis

Sleep apnea

vestibular symptoms in cerebellar disorders
Vestibular Symptoms in Cerebellar Disorders
  • Cerebellum largely involved in COORDINATION
  • Cerebellar dysfunction presents as unsteadiness, clumsiness, and difficulty with stabilizing eye movements
mechanism of auditory vestibular symptoms
Mechanism of Auditory/Vestibular Symptoms
  • Direct pressure on vestibular areas
  • Increased fluid pressure throughout the brain
  • Increased pressure of inner ear via cochlear aqueduct
mechanism of auditory vestibular symptoms1
Mechanism of Auditory/Vestibular Symptoms
  • Further stretching of elongated nerves
  • Bending of 8th nerve over bony edge of porus acousticus
  • Compression of brainstem nuclei
auditory vestibular symptoms in acm
Auditory/Vestibular Symptoms in ACM
  • Sensorineural hearing loss
  • Vertigo & Dizziness
  • Nystagmus (Downbeating) and Poor Pursuits
diagnostic evaluation of auditory vestibular symptoms in acm
Diagnostic Evaluation of Auditory/Vestibular Symptoms in ACM
  • Electronystagmography (ENG)
  • Rotational chair testing
  • Vestibular Evoked Myogenic Potential Testing (VEMP)
  • Electrocochleography testing (ECOG)
  • Fistula testing
  • Moving platform posturography
diagnostic evaluation of auditory vestibular symptoms in acm1
Diagnostic Evaluation of Auditory/Vestibular Symptoms in ACM
  • Electronystagmography (ENG)
  • Rotational chair testing
  • Vestibular Evoked Myogenic Potential Testing (VEMP)
  • Electrocochleography testing (ECOG)
  • Fistula testing
  • Moving platform posturography
electronystagmography1
Electronystagmography
  • Determines whether or not dizziness is due to inner ear disease
  • Excellent for diagnosis of unilateral ear disorders
  • 4 main parts:

- Calibration test

- Tracking test

- Positional test

- Caloric test

Intended to diagnose CNS disorders

rotational chair testing1
Rotational Chair Testing
  • Determines whether or not dizziness is due to inner ear or brain disorder
  • Obtained in addition to ENG
  • 3 main parts:

- Chair test

- Optokinetic test

- Fixation test

vocal cord paralysis
Vocal Cord Paralysis

Flexible laryngoscopy demonstrates bilateral vocal cords in the paramedian position and no mobility with vocalization

vocal cord paralysis1
Vocal Cord Paralysis
  • Congenital TVC paralysis
  • ACM accounts for most central neurologic causes of bilateral TVC paralysis
  • Tracheostomy often necessary
sleep apnea
Sleep Apnea
  • Central Apnea: Cessation of both airflow and respiratory effort
  • Obstructive Apnea: Cessation of airflow in the presence of continued respiratory effort
  • Mixed Apnea
when to order a sleep study in the pediatric population
When to Order a Sleep Studyin the Pediatric Population
  • Symptoms in morbidly obese patient
  • Apnea in absence of snoring
  • Apnea without adenotonsillar hypertrophy
  • Underlying neurologic disorders
  • Craniofacial abnormalities
central sleep apnea1
Central Sleep Apnea
  • Dysfunction of medullary centers of respiratory control
  • Cranial nerve dysfunction involving laryngeal nerves or bulbar muscles, which mediate airway patency
central sleep apnea4
Central Sleep Apnea
  • Anticipate immediate improvement after decompression surgery
  • Reports of recovery taking up to 6-12 months
conclusions
Conclusions
  • Physical exam in combination with diagnostic studies may indicate central cause of imbalance and hearing loss
  • ACM should be considered in bilateral TVC paralysis
  • High index of suspicion of ACM in central/mixed sleep apnea
slide27
Ada M, et al. Congenital vocal cord paralysis. J Craniofac Surg. 2010 Jan;21(1):273-4
  • Dauvilliers Y, et al. Chiari malformation and sleep related breathing disorders. J Neurol Neurosurg Psychiatry 2007;78:1344-1348
  • Hain TC. www.dizziness-and-balance.com
  • Hershberger ML and Chidekel A. Arnold-Chiari Malformation Type I and Sleep-Disordered Breathing: Review of Literature. J Pediatr Health Care. 2003;17(4)
  • Kuma A, et al. The Chiari I malformation and the neurologist. Otol Neurotol. 2002 Sep;23(5):727-35
  • Murray C, et al. Arnold Chiari type I malformation presenting with sleep disordered breathing in well children. Arch Dis Child. 2006 April;91(4):342-343
  • Rowley JA. Obstructive Sleep Apnea-Hypopnea Syndrome: Multimedia. www.emedicine.com. Updated May 14, 2010