History and exam
Key diagnostic factors
common
asymmetric hearing loss
Over 90% of patients exhibit unilateral or asymmetric sensorineural hearing loss. Such findings indicate need for an audiology workup.[2][38][45] Though definitions vary, asymmetric hearing loss is commonly defined by 2 or more contiguous frequencies with >10 dB asymmetry, or ≥15 dB at 1 frequency, compared with the contralateral ear on behavioral audiometry.[46] Some have reported that asymmetry at 3000 Hz is most sensitive.[47] Notably, many people with sudden sensorineural hearing loss will experience improvement, either spontaneously or following corticosteroid therapy. Improvement in hearing does not exclude the diagnosis of a vestibular schwannoma, and MRI should also be obtained in these cases.[42] Though variable, differences of >20% in word recognition should also prompt consideration for MRI screening. Approximately 3% of people with asymmetric hearing loss will have a vestibular schwannoma, or other retrocochlear lesions, identified on imaging, emphasizing that in most cases, asymmetric hearing loss is actually not due to a vestibular schwannoma.
progressive episodes of dizziness
Intermittent or brief imbalance, particularly with quick head turning, is common and results from unilateral vestibular hypofunction. Large tumors may result in dysmetria and ataxia related to cerebellar compression or hydrocephalus.
tinnitus
Tinnitus represents a phantom percept that results from cochlear deafferentation. Ipsilateral or bilateral tinnitus is a common symptom among patients with vestibular schwannomas and often coincides with hearing loss. Notably, tinnitus is highly prevalent in the general adult population and most people with tinnitus do not have a vestibular schwannoma.
Other diagnostic factors
common
difficulty localizing sounds
Significant ipsilateral hearing loss (typically >30 dB compared to the other ear) makes localizing sound and distinguishing voices in crowds difficult.
uncommon
headache
May occur with associated earache or pressure sensation. As headache is a ubiquitous symptom in the general population, separating out headache that is attributable to the tumor from other causes is often challenging. It is believed that most small and medium-sized tumors do not result in headache. Therefore, in most cases, headaches are diffuse and not localizing to the side of the head with the tumor.
facial numbness
Ipsilateral facial numbness often begins in a perioral distribution, although presentation is variable. Diminished or absent sensation, reduced blink reflex, or ipsilateral neuralgiform face pain may occur with tumors that are larger than approximately 2-3 cm in cerebellopontine angle dimension.
facial weakness
diplopia on lateral gaze
Late finding, usually associated with nystagmus.
Rarely related to abducens weakness.
nystagmus
On lateral gaze, often associated with dizziness and/or diplopia.
Usually implies a larger tumor.
loss of balance and coordination difficulties
A late finding indicating a larger tumor.
slower blink
A late finding indicating a larger tumor.
swallowing difficulties
Ipsilateral cranial nerves other than the facial and vestibulocochlear nerves may be affected, such as the lower cranial nerves, which affects swallowing.
gait disturbances
A late finding indicating a larger tumor.
hydrocephalus
A late finding indicating a larger tumor.
papilledema
A late finding indicating a larger tumor.
increased intracranial pressure
A late finding indicating a larger tumor.
Risk factors
strong
neurofibromatosis-related schwannomatosis
This rare autosomal dominant disorder causes benign tumors on the vestibulocochlear nerves and other locations.
A coexisting diagnosis of neurofibromatosis-related schwannomatosis increases the prevalence of vestibular schwannoma.[12]
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