Vertigo in Clinical Practice: Evidence-Based Diagnosis and Treatment

Etiology

Vertigo is most commonly caused by a dysfunction in the vestibular system, whether a peripheral or central lesion.[1] Peripheral etiologies encompass common causes of vertigo, including benign paroxysmal positional vertigo (BPPV) and Ménière's disease.[3] BPPV arises from displaced calcium carbonate crystals, or otoconia, predominantly within the posterior semicircular canal. This condition manifests as transient, paroxysmal, and recurrent episodes of vertigo, typically lasting only a few minutes or less. These episodes are frequently accompanied by nausea and other associated symptoms, such as vomiting.[1] In contrast to BPPV, patients diagnosed with Ménière disease often present with tinnitus, hearing impairment, and aural fullness alongside vertigo. Endolymphatic hydrops constitutes a characteristic pathological feature of Ménière disease.[4] [5] The symptoms of Ménière disease are caused by an increased volume of endolymph within the semicircular canals. If left untreated, this condition can lead to a progressive loss of hearing.

Two additional distinct causes of peripheral vertigo include acute labyrinthitis and vestibular neuritis. Both originate from inflammation, frequently induced by a preceding or concurrent viral infection.[1] Another viral-induced cause of vertigo is Herpes zoster oticus, also known as Ramsay Hunt syndrome.[6] In Ramsay Hunt syndrome, vertigo results from the reactivation of latent Varicella-zoster virus in the geniculate ganglion, which can cause inflammation of the facial and vestibulocochlear nerves; this often leads to rashes, facial paralysis, tinnitus, and hearing loss.[1] Uncommon peripheral causes include cholesteatoma, otosclerosis, and a perilymphatic fistula. Cholesteatomas are cyst-like lesions filled with keratin debris that often involve the middle ear and mastoid air cells.[7] Otosclerosis is characterized by abnormal bone growth in the middle ear, resulting in conductive hearing loss and potentially affecting the cochlea, which can lead to tinnitus and vertigo.[8] A perilymphatic fistula is another uncommon cause of peripheral vertigo and often results from trauma.[1]

Central etiologies of vertigo should always be considered in the differential diagnosis, especially in older adults. Ischemic or hemorrhagic strokes, particularly involving the cerebellum or brainstem, are life-threatening and must be ruled out through history, physical examination, and other diagnostic tests if warranted.[1][9] Other significant central causes include tumors, especially those originating from the cerebellopontine angle. (see Image. Cerebellopontine Angle [CPA] Tumor, Magnetic Resonance Image [MRI]).[10] 

Examples of such tumors include a brainstem glioma, medulloblastoma, and vestibular schwannoma, which can lead to sensorineural hearing loss as well as vertiginous symptoms.[1] Vestibular migraines are a common central cause of vertigo, characterized by unilateral headaches associated with nausea, vomiting, photophobia, and phonophobia. Additionally, multiple sclerosis (MS) has been linked to both central and peripheral causes of vertigo; MS can cause vertigo by developing demyelinating plaques in the vestibular system pathways.[11] BPPV is a common peripheral cause of vertigo in patients with MS.[1] Among the different diseases previously discussed, other causes can lead to vertigo, such as medication-induced vertigo and psychological disorders, including mood, anxiety, and somatization. Medications associated with vertigo include anticonvulsants (eg, phenytoin) and salicylates.[1]

Epidemiology

Vertigo affects both men and women, but it is about 2 to 3 times more common in women than in men.[1] This condition has been associated with various comorbid conditions, including depression and cardiovascular disease. Prevalence increases with age and varies depending on the underlying diagnosis. Based on a general population survey, the 1-year prevalence of vertigo is approximately 5%, and the annual incidence is 1.4%. Dizziness, including vertigo, affects approximately 15% to over 20% of adults yearly.[12] Almost half of patients with migraine complain of vertigo, motion sickness, or mild hearing loss. [13] 

For benign paroxysmal positional vertigo, the 1-year prevalence is about 1.6%, and is less than 1% for vestibular migraine. The impact of vertigo should not be underestimated, as nearly 80% of survey respondents reported an interruption in their daily activities, including employment, and required additional medical attention. The prevalence of Menière disease has recently been reported to be 0.51%, which is significantly higher than previous estimates.[12][14]

Pathophysiology

Most cases of vertigo are attributed to peripheral causes that typically present acutely. In contrast, central vertigo is, by definition, caused by pathology originating within the central nervous system. Nevertheless, the pathophysiology of the balance system demonstrates significant overlap. The peripheral labyrinths, brainstem, and cerebellum share the same blood supply, namely the vertebrobasilar arterial system.[15][16] Furthermore, disturbances may occur in the neuronal input processed by the central nervous system from cranial nerve VIII and in the integration of motor and sensory inputs related to vision and proprioception.[17][18]

Abnormalities in the peripheral vestibular system can cause symptoms of vertigo. These abnormalities may result from damage or dysfunction in parts like the vestibular labyrinth or the vestibular nerve, or occur alongside disturbances in the central vestibular system, specifically in the brainstem and cerebellum.[1] Although a lasting peripheral structural vestibular disturbance may persist, the symptom of vertigo is not permanent, as the central nervous system adapts over days to weeks due to neuroplasticity.[19][20][21] 

Infections can cause vertigo through involvement of the peripheral or central vestibular system, with viral labyrinthitis being the most common example. Otomastoiditis is an infection of the tympanic and mastoid cavities, usually caused by bacterial agents, especially Streptococcus pneumoniae and Haemophilus influenzae. Acute cerebellitis is a form of encephalitis limited to the cerebellum. This condition mainly affects children, with the Varicella-zoster virus identified as the primary causative agent. Finally, cholesteatoma can be acquired or congenital, occurring in the pars flaccida or pars tensa, and results from abnormal proliferation of keratinized stratified squamous epithelium.[22]

Central vertigo may arise from a variety of etiologies, including positional vertigo, cranial nerve impairments, transient ischemic attacks, acute cerebellar pathology, caudal cerebellar infarction, cerebrovascular disorders, cardiovascular risk factors such as atrial fibrillation, and neurological conditions. Tumors can induce vertigo by compressing structures within the central vestibular system. Schwannoma is the most common type of lesion in the cerebellopontine angle.[23] Meningioma is an extra-axial tumor in adults that can induce vertigo, ranking as the second most prevalent lesion in the cerebellopontine angle. Other neoplasms associated with vertigo include Glomus jugulare and glomus jugulotympanicum. These represent the primary tumors of the chemoreceptor system situated at the jugular foramen. Additionally, metastases should be considered in patients presenting with vertigo, including those with known primary neoplasms or multiple brain lesions. 

History and Physical

The initial diagnosis aims to confirm whether the patient is experiencing vertigo since most patients report dizziness as their primary complaint. Vertigo is an abnormal sensation of movement or rotation experienced by the patient or their environment. To elicit actual vertigo symptoms, a clinician may ask, "Does it feel like the room is spinning around you?" or "Do you feel like you are the one spinning?"[1] Once vertigo has been identified, a comprehensive medical history helps the clinician distinguish between central and peripheral etiologies of vertigo. Eliciting a detailed timeline of symptom progression is among the most effective methodologies for determining the underlying cause. A clinician may classify symptoms as acute or chronic, recurrent or persistent, and progressive or nonprogressive. For example, chronic and recurrent vertigo lasting a few minutes or less is frequently associated with benign paroxysmal positional vertigo. A solitary episode lasting from minutes to hours may be attributable to a vestibular migraine or potentially a more serious underlying condition, such as a transient ischemic attack. Conversely, episodes of longer duration, extending over days, can occur in both peripheral and central causes, such as vestibular neuritis or lesions at the cerebellopontine angle tumors.[1]

Once a timeline has been established, it is essential to assess for associated symptoms, as this can further assist in differentiating between central and peripheral etiologies. Nausea and vomiting are common during acute episodes of vertigo and are not indicative of any specific cause. Given the importance of ruling out central causes that may be progressive or life-threatening, such as vertebrobasilar stroke or multiple sclerosis, clinicians should inquire about any focal neurological symptoms, including diplopia, dysarthria, dysphagia, and numbness or weakness.[24] Additionally, other signs that clinicians should investigate include depressed consciousness, nystagmus, internuclear ophthalmoplegia, cranial nerve deficits, muscle weakness, hyperesthesia, ataxia, and cardiac signs such as murmurs, irregular heartbeats, tachycardia, or bradycardia.

An absence of focal neurologic symptoms does not entirely exclude the possibility of a serious central process; however, their presence serves as a red flag that necessitates further investigation. When considering the differential diagnosis of central causes of vertigo and related symptoms, clinicians should inquire about symptoms such as headache, photophobia, and visual auras, as these frequently accompany vestibular migraines. Additional symptoms associated with vertigo may originate from a peripheral lesion. For example, patients might experience deafness and tinnitus attributable to Ménière disease.[25] They may report a recent viral infection that can cause acute labyrinthitis and vestibular neuritis. Finally, it is essential to review a patient's medication list and social history for any substance or alcohol use. Medications that can impact vestibular function include anticonvulsants, salicylates, and antibiotics.[26]

When combined with a comprehensive history, a focused physical examination can help further differentiate between a peripheral and a central cause of vertigo. Assessing for nystagmus is a key portion of the physical examination when a patient presents with vertiginous symptoms.[1] A functional vestibular system allows one to maintain gaze during rotation through vestibulo-ocular reflexes. With unilateral dysfunction in the vestibular system, the eyes drift slowly away from a target and then correct rapidly in the opposite direction, resulting in a “beat” in the direction of the fast phase. In a peripheral vestibular lesion, the rapid phase is away from the affected side, and the frequency and amplitude of nystagmus increase with a gaze toward the side of the fast phase. For instance, a rightward gaze increases right-beating nystagmus. In peripheral lesions, the predominant direction of nystagmus remains the same regardless of the direction of gaze, while central lesions may present with nystagmus that reverses direction.[27]

Central lesions can present with nystagmus in any direction, while peripheral lesions often present with horizontal nystagmus with a torsional component. Noting that nystagmus resulting from a peripheral lesion is never purely torsional or vertical is essential. The head impulse or thrust technique is a physical examination technique to further determine the etiology. In this exam, patients are asked to fix their gaze on a distant target, using prescription eyeglasses if necessary. The head is turned quickly to the right or left by about 15 degrees. A typical response occurs when the eyes remain on the target. An abnormal response is when the eyes are dragged off the target in the direction of head turning, followed by a corrective saccade back to the target. This response implies a peripheral lesion resulting in a deficient vestibulo-ocular reflex on the side of the head turn.

Finally, a clinician may test for skew, which involves the examiner covering one eye and observing for a vertical or horizontal shift in the uncovered eye. Central lesions sometimes produce a slight skew deviation. When the head impulse test is combined with an examination of nystagmus and a test for skew, this is referred to as the Head Impulse-Nystagmus-Test for Skew (HINTS) test.[28] A standard head impulse test on both sides with direction-changing nystagmus and/or skew deviation is concerning for a central lesion. An abnormal head impulse test with unidirectional nystagmus and absent skew deviation strongly suggests a peripheral lesion. The HINTS test may be more sensitive for diagnosing acute stroke than magnetic resonance imaging (MRI) within the first 48 hours following symptom onset.[29]

Other physical examination techniques may be used to diagnose and treat vertigo, including the Dix-Hallpike maneuver.[30] This is the diagnostic test of choice when BPPV involving the posterior semicircular canals is suspected. Dix-Hallpike consists of 2 maneuvers. A patient sits on an exam table facing forward with eyes open, and the clinician turns the patient's head 45 degrees to the right. The clinician supports the patient's head while the patient quickly lies back to a supine position, with the head hanging about 20 degrees off the end of the table. The patient remains in this position for 30 seconds before returning to the upright position, where they are observed for another 30 seconds. This maneuver is repeated with the head turned to the left. The test is positive if, at any point, the maneuvers produce vertigo with or without nystagmus.[1] 

Another helpful diagnostic maneuver for peripheral vertigo is the Unterberger test, which consists of 2 maneuvers. A patient stands in an upright position with their eyes closed. The clinician then instructs the patient to walk in place for 60 seconds. The test is positive if the body is rotated to one side while walking in place.[31] Furthermore, gait and balance testing can also help localize the issue. Patients with unilateral peripheral disorders often lean or fall toward the side of the lesion. In contrast, patients with cerebellar lesions frequently cannot walk without assistance, and the direction of falling during Romberg testing is variable. Many primary care and specialist clinicians overlook the fundamentals of history and physical examination, resulting in unnecessary imaging and medication.[32]

A thorough head, neck, and neurologic examination is essential. The otoscopic exam should rule out obvious infections, such as acute otitis media, and bedside hearing tests can help distinguish other causes of vertigo. Weber and Rinne tests are often done at the bedside to screen for conductive and sensorineural hearing loss.[33] However, audiometry and tympanometry are more sensitive than bedside testing in detecting hearing loss and middle ear fluid.

A unilateral hearing loss points strongly to a peripheral etiology, but further diagnostic imaging with MRI, usually with gadolinium, is warranted if a cause can not be identified. Another practical test that can be performed is the auditory brain response, also known as brainstem auditory evoked potentials, which are objective measurements of the auditory pathway function from the auditory nerve to the mesencephalon. There is insufficient high-quality evidence to support the diagnostic value of the absence of hearing loss, as assessed by pure tone audiometry, in predicting BPPV in patients with vertigo.[34]

Evaluation

The acronym STANDING describes a 4-step algorithm based on nystagmus observation and well-known diagnostic maneuvers; it includes the discrimination between SponTAneous and positional or gaze-evoked nystagmus, evaluation of the Nystagmus Direction (whether it is unidirectional or direction-changing), the head Impulse test, and the assessment of equilibrium (staNdinG).[35] Laboratory testing often fails to identify the cause of vertigo. Diagnostic brain imaging is recommended if a central lesion is suspected. Clinicians may find it difficult to distinguish between a central lesion, such as an infarction, and a peripheral lesion, like vestibular neuritis, where vertigo symptoms can last for days.

In such cases, neuroimaging is advised for patients with stroke risk factors, focal neurologic deficits, a new headache, or when the physical exam does not fully match a peripheral lesion. The preferred methods are brain MRI and magnetic resonance (MR) angiography, as computed tomography (CT) scans are less sensitive than MRI for detecting and evaluating central lesions due to their limited resolution of posterior fossa structures. However, if a brain MRI is unavailable or contraindicated, a CT scan with thin cuts may be used, primarily through the brainstem and cerebellum.[22]

Treatment / Management

Managing vertigo depends on its etiology, and addressing the underlying cause often alleviates the symptoms. Pharmacological interventions may help suppress vestibular symptoms during acute episodes, which can persist for several hours to days. The most frequently employed for symptomatic relief include antihistamines, calcium channel blockers, benzodiazepines, and antiemetics. Among antihistamines, meclizine and betahistine are used.[36] Calcium channel blockers, such as cinnarizine, are included in this category. Due to their sedative effects, caution is recommended when prescribing antihistamines, benzodiazepines, and antiemetics to older adults. Steroids can be beneficial in some instances. Healthcare professionals should be aware that vestibular suppressants should be used for a limited duration, as excessive use may unnecessarily hinder the brain and central nervous system's natural compensatory mechanisms for peripheral vertigo. (A1)

Additional nonpharmacologic treatments for patients with permanent unilateral or bilateral vestibular dysfunction include physical therapy with vestibular rehabilitation.[37] Vestibular rehabilitation exercises train the brain to maintain balance using alternative visual and proprioceptive cues. Several randomized controlled trials have demonstrated the benefits of vestibular rehabilitation, including a reduction in vertiginous symptoms, a decrease in position- and movement-provoked dizziness, and an improvement in activities of daily living.[38][39][40] In some patients, particularly those diagnosed with vestibular neuritis, a combination of pharmacologic and nonpharmacologic therapy is recommended.(A1)

Corticosteroids are recommended in the acute setting, in addition to vestibular rehabilitation, for vestibular neuritis. In patients with Ménière disease, lifestyle adjustments, medication, and vestibular rehabilitation may be effective.[41]  Patients with Ménière disease may be sensitive to a high-salt diet, caffeine, and alcohol. Avoiding known triggers can help alleviate symptoms. Diuretics may also be prescribed when diet modification alone is not sufficient in controlling symptoms. Acute episodes can be symptomatically treated with vestibular suppressants like meclizine.

Patients with benign paroxysmal positional vertigo benefit from nonpharmacologic agents. The primary treatment for BPPV focuses on head rotation maneuvers that dislodge calcium deposits back to the vestibule through canalith repositioning, also known as the Epley maneuver.[42] The benefit of the Epley maneuver is that it can be performed at home by the patient. To perform a modified Epley maneuver (as shown in the image), instruct the patient to position themselves upright on a bed with their head turned 45 degrees to the left and a pillow behind them. The pillow should be placed directly under the shoulders when lying on one's back. Once the patient is in position, they should quickly lie back onto the pillow so that their head is reclined on the bed. They should hold this position for 30 seconds. Without raising their head, they should turn their head 90 degrees to the opposite side (right) and hold this position for another 30 seconds.(A1)

After 30 seconds, they should turn their body and head another 90 degrees to the right and wait for 30 seconds. Finally, they should sit up on the right side of the bed. This maneuver can be repeated, starting on the opposite side, and should be performed at least 3 times a day until the patient has no further episodes of positional vertigo for 24 hours. The Epley maneuver is effective in 50% to 90% of patients.[43] Unfortunately, BPPV is intractable in a select number of patients, and surgical treatment can be an option, particularly if symptoms are disabling. Surgical options include occlusion of the posterior canal with bony plugs or transection of the posterior ampullary nerve—either surgical procedure risks hearing loss (see Image. Epley Maneuver).

Management of central vertigo should focus on identifying and addressing the underlying cause. Migraine and related vertigo are not entirely understood, but they should be considered. [44] Transient ischemic attacks can cause vertigo and focal neurological symptoms. Stroke and stroke prevention should be considered in the differential diagnosis, along with heart disease. Some patients will present with orthostatic hypotension, and it is essential to determine the etiology instead of using vestibular suppressants.[45] Additionally, neurological causes, especially multiple sclerosis, tumors, and malformations of the posterior fossa, need to be evaluated.[46](B2)

Differential Diagnosis

The differential diagnosis of vertigo is broad because it can result from a central or peripheral lesion in the vestibular system. Therefore, it is important to distinguish vertigo from symptoms of disequilibrium, presyncope, and lightheadedness (see Table. Disease Category and Disease Entities in Vertigo). A lengthy list of vascular, infectious, traumatic, inflammatory, demyelinating, metabolic, iatrogenic, and neoplastic causes can lead to these symptoms.[47]

Table. Disease Category and Disease Entities in Vertigo

Prognosis

The prognosis is contingent upon the etiology of vertigo; typically, peripheral causes are associated with a more favorable outlook, whereas central causes tend to have a less favorable prognosis. The recurrence rate of benign paroxysmal positional vertigo is approximately 50% over 5 years. Furthermore, dizziness attributable to anxiety persists in nearly one-third of patients one year following vestibular treatment for neuritis.[14] According to Perrez-Garrigues et al, the number of episodes of vertigo is higher in the first years of the disease and decreases in later years, regardless of whether patients receive treatment; most patients reach a "steady-state phase free of vertigo."[48] 

As with vertigo, hearing loss is most pronounced in the early years of the disease and tends to stabilize in later years. Usually, there is little to no recovery from hearing loss.[49] The acute vertigo associated with labyrinthitis typically resolves within a few days; milder symptoms may persist for several weeks. The prognosis is usually good if the patient has no serious neurological sequelae. However, patients with neurological complications from central causes may require further interventions.[50]

Complications

The key to arriving at a diagnosis is to differentiate vertigo from other causes of dizziness or imbalance and distinguish between central and peripheral causes of vertigo. An accurate diagnosis is essential and critical in cases of life-threatening conditions. Peripheral causes of vertigo are usually associated with little to no complications. Complications arising from the central causes of vertigo are variable, depending on whether the etiology is accurately identified and properly diagnosed and managed.[51]