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CONDITION/DISORDER SYNONYMS

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  • Vestibular schwannoma

  • Acoustic schwannoma

  • Cerebellopontine angle tumor

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ICD-9-CM CODE1

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  • 225.1 Benign neoplasm of cranial nerves

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ICD-10-CM CODE2

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  • D33.3 Benign neoplasm of cranial nerves

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PREFERRED PRACTICE PATTERNS3

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  • 5A: Primary Prevention/Risk Reduction for Loss of Balance and Falling

  • 5F: Impaired Peripheral Nerve Integrity and Muscle Performance Associated with Peripheral Nerve Injury

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PATIENT PRESENTATION

A 21-year-old college student noticed a ringing in her left ear the day after a big sorority party. She initially thought it was an effect of the loud music at the party, but the ringing persisted and got louder. A couple of weeks later she developed a headache that was reduced but not relieved by acetaminophen. She struggled with studying her textbooks and computerized notes, and began to get dizzy whenever she reached down to the floor or quickly turned her head. She had no limitations in cervical range of motion, strength, or posture. MRI revealed a tumor on the vestibulocochlear nerve.

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KEY FEATURES
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Description
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  • Disorder of the vestibulocochlear nerve (eighth cranial nerve) that carries vestibular and auditory input from the inner ear to the central nervous system

  • Slow-growing (usually 1–2 mm/yr), benign tumor of the myelin-forming Schwann cells that surround the vestibular portion of the eighth cranial nerve

  • Tumor most commonly originates from the inferior vestibular branch

  • Tumor forms in the internal auditory canal and can extend into the cerebellopontine angle

  • Typically results in a gradual onset of high-frequency sensorineural hearing loss, tinnitus, sense of ear fullness, unsteadiness, dizziness, vertigo

  • As tumor grows, there may be facial nerve involvement (resulting in facial numbness or weakness), headache, hydrocephalus, incoordination, diplopia, hoarseness, difficulty swallowing, brainstem compression, death

  • Tumors classified as sporadic, cystic, or neurofibromatosis type II

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FIGURE 258-1

Magnetic resonance image of a horizontal section through the head at the level of the lower pons and internal auditory meatus. A left acoustic nerve schwannoma with its high intensity is shown in the left cerebellopontine angle (arrow). (From Waxman SG. Clinical Neuroanatomy. 26th ed. http://www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

Graphic Jump Location
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Essentials of Diagnosis
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  • Physical examination normal, unless the cerebellum or brainstem compromised

  • Audiogram abnormal

  • Imaging studies abnormal

  • Neurotologic examination abnormal

  • Thorough and detailed history essential to distinguish acoustic neuroma from other vestibular disorders and central pathology

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General Considerations
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  • Patients typically present with complaints of progressive hearing loss and loss of balance worsening gradually over several months

  • If tumor growth progresses, may present with other cranial nerve (especially V and VII), brainstem, or cerebellar signs and symptoms

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Demographics
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  • Uncommon in ...

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