Otolaryngologic Manifestations of Arnold-Chiari Malformation
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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

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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


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

Auditory/vestibular dysfunction

Vocal cord paralysis

Sleep apnea


Vestibular Symptoms in Cerebellar Disorders

  • Cerebellum largely involved in COORDINATION

  • Cerebellar dysfunction presents as unsteadiness, clumsiness, and difficulty with stabilizing eye movements


Vestibular Findings in Cerebellar Disorders


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 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

  • Sensorineural hearing loss

  • Vertigo & Dizziness

  • Nystagmus (Downbeating) and Poor Pursuits


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 ACM

  • Electronystagmography (ENG)

  • Rotational chair testing

  • Vestibular Evoked Myogenic Potential Testing (VEMP)

  • Electrocochleography testing (ECOG)

  • Fistula testing

  • Moving platform posturography


Electronystagmography


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 Testing


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

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


Vocal Cord Paralysis

  • Congenital TVC paralysis

  • ACM accounts for most central neurologic causes of bilateral TVC paralysis

  • Tracheostomy often necessary


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 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 Apnea


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 Apnea


Central Sleep Apnea


Central Sleep Apnea

  • Anticipate immediate improvement after decompression surgery

  • Reports of recovery taking up to 6-12 months


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


Thank You


  • 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


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