Acoustic Neuroma
Basics
Basics

Basics
Description
Description
Description
Acoustic neuromas (vestibular schwannomas) are slow-growing, benign, intracranial, extra-axial tumors originating from the vestibulocochlear nerve.
- Originated from Schwann cells of the nerve sheath
- Usually arise in the internal auditory canal near the cerebellopontine angle
- Most are unilateral; bilaterally seen in neurofibromatosis type 2 (NF2)
Epidemiology
Epidemiology
Epidemiology
- 6–10% of all intracranial tumors
- 80–90% of cerebellopontine angle tumors
- 95% of cases are unilateral.
- Present most commonly in the 5th to 6th decades, although signs and symptoms can occur earlier
- Female predominance
- 5% of acoustic neuromas are a result of NF2.
- Bilateral acoustic neuroma occurring in NF2 present before age 30 years
Incidence
- 1/100,000 per year
- Asymptomatic lesions may be more common.
Prevalence
3,000 diagnosed annually in the United States
Etiology and Pathophysiology
Etiology and Pathophysiology
Etiology and Pathophysiology
- Compression of acoustic and facial nerve when located within internal acoustic canal
- Compression of brainstem, 4th ventricle, and trigeminal nerve when tumor is at the cerebellopontine angle
- Vestibular schwannomas are related to the NF2 gene and its product merlin. Merlin acts as a tumor suppressor and as a mediator of contact inhibition. Thus, deficiencies in both NF2 genes lead to vestibular schwannoma development.
Genetics
- Unknown for unilateral acoustic neuroma
- NF2: bilateral acoustic neuromas
- Autosomal dominant
- Gene located on chromosome 22q11
Risk Factors
Risk Factors
Risk Factors
- High-dose ionizing radiation
- Pregnancy and epilepsy may increase risk.
- There is some literature suggesting that smoking cigarettes may be protective. However, further research must be conducted to support these claims.
- No proven increase in risk of acoustic neuroma with regular use of a mobile phone
Commonly Associated Conditions
Commonly Associated Conditions
Commonly Associated Conditions
- NF2
- Pregnancy may accelerate the growth of the tumor.
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