Rare Causes of Acute Recurrent Vertigo or Dizziness

Unusual and Rare Causes of Repeated Vertigo Can Be Disabling

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What are some of the rare, uncommon, or unusual causes of vertigo?. BSIP/UIG/Getty Images

What are some of the rare or uncommon reasons for vertigo or dizziness? If you have been coping with these symptoms, but the common sudden causes of vertigo don't fit, what else could it be?

What is Vertigo?

Vertigo is an uncomfortable type of dizziness in which someone suffers from an illusory sense of movement. Almost everyone has experienced this sensation after spinning quickly and repeatedly in a circle: the world seems to continue moving around you although you know that you've stopped moving.

Sudden, repeated attacks of vertigo can disable people by making it impossible to walk without feeling nauseous or even falling over. Fortunately, most cases of vertigo are caused by common problems that are diagnosed with relative ease.

What else could it be if your symptoms aren't consistent with the common causes of vertigo and dizziness? There are actually dozens of explanations which are possible, but let's take a look at some of the "most common" uncommon causes of vertigo.

Rare and Uncommon Causes of Vertigo

Most often, people who are experiencing vertigo or dizziness will have one of the more common causes. These include conditions such as benign positional vertigo, vestibular migraines, Meniere's disease, a brainstem stroke or transient ischemic attack, or vestibular neuritis.

Sometimes, though, the cause is actually something rare. Even though these uncommon causes of vertigo occur less often, it's important to be aware of these other diagnoses so that your symptoms are not mistaken for something more commonplace.

Let's take a look at these unusual causes of vertigo.

Autoimmune Inner Ear Disease

Sometimes, the immune system mistakes part of our own body for an invading infection. When this occurs—when the body attack itself—it is referred to as an autoimmune disorder. If this happens in the inner ear, it can cause progressive hearing loss, as well as vertigo.

About a quarter of such people will have other autoimmune diseases such as systemic lupus erythematosus, polyarteritis nodosa, or Wegener granulomatosis. About half of these people will respond to corticosteroids.

Labyrinthine Concussion

Concussions often cause headaches, nausea, vomiting, and dizziness. A sense of vertigo after a concussion can result from injury to the vestibular organs after the head is hit. Depending on the nature of the injury, there may be blood in the inner ear. While this is usually worst directly after the head injury, the vertigo may come and go for a while afterwards. Occasionally, post-traumatic changes in the pressure gradient between components of the inner ear (endolymphatic hydrops) can develop, leading to Meniere syndrome.

Perilymphatic Fistula

Head injury, heavy lifting, or injury due to changes in pressure (such as with scuba diving) can sometimes lead to an abnormal connection between parts of the ear which not meant to be connected. An abnormal connection between two regions of the body which are not ordinarily connected is called a fistula.

Symptoms of a perilymphatic fistula are often worsened by a change in external or internal pressure, such as sneezing, straining, coughing or loud noises.

Changes in elevation may also exacerbate symptoms, such as flying in a plane or even riding an elevator.

The diagnosis of perilymphatic fistula can be difficult unless an inciting incident is described. The treatment usually involves resting with the head elevated and avoiding all forms of straining. In those who do not improve with such treatment, surgery may be necessary.

Another related phenomenon is dehiscence, in which the connection between two chambers is not complete, but bone may be thinner than it usually would be. An example is dehiscence of the superior canal of the inner ear, which causes unique symptoms like sound induced vertigo.

About half also have autophony, meaning they hear internal sounds like their own voice, heartbeat, or sometimes even their own eye movements at a disturbingly loud volume.


Otosclerosis is a sometimes inherited disorder in which bones are reabsorbed, and new bone formation occurs in the middle and inner ear (a number of genes are associated with familial otosclerosis, so sufferers may or may not have an affected parent). This usually begins between the second and fourth decades of life. The result is bilateral progressive hearing loss. About 20 percent of people with otosclerosis also have vertigo or imbalance, which results from destruction of the inner ear. Others may develop endolymphatic hydrops with Meniere syndrome. An audiogram can be helpful in making the diagnosis.

Epileptic Vertigo

Rarely, spells of vertigo can actually be due to seizures. Many people are familiar with typical tonic clonic seizures (grand mal seizures) but there are actually many different types with very different symptoms. Abnormal electrical activity can occur in parts of the brain that process the vestibular system. An electroencephalogram (EEG) can be used to determine whether spells are epileptic in nature. Some people may have an epileptic aura prior to the vestibular seizures, which can help point to the possible diagnosis.

Chiari Malformation

A Chiari I malformation is a congenital abnormality (something you are born with) in which the bottom of the cerebellum (the part of the brain which controls balance and coordination) extends lower than it normally would. Usually, this doesn't cause any symptoms, but can sometimes lead to headache, gait imbalance, and vertigo. When the vertigo is present, it may be worsened by bending the neck backwards (head motion induced vertigo.)

While nystagmus (uncontrolled eye movements) is often present in any form of vertigo, in Chiari malformations, the nystagmus may beat in the downward direction instead of the side, which is unusual. Surgery may be needed if symptoms are severe with a Chiari malformation. That said, most people with Chiari malformations do not require surgery.


A wide number of medications can cause dizziness, and some cause vertigo specifically. Antibiotics known as aminoglycosides are particularly problematic, and can even lead to permanent damage. Lithium toxicity may also cause vertigo. In addition, vertigo is a possible side effect with medications such as anti-convulsants (anti-seizure drugs), anesthetics, antidepressants, non-steroidal anti-inflammatory agents, diabetes medications, sedatives, tranquilizers, and more.

Episodic Ataxia

Episodic ataxia, and episodic ataxia type 2, in particular, can cause severe episodes of vertigo with nausea and vomiting in childhood or early adult life. Nystagmus may be present both during and between attacks. The attacks often become progressively worse, and symptoms may begin to occur between attacks as well.

Bottom Line on Uncommon Causes of Vertigo

The vast majority of people with periods of sudden vertigo will never have to worry about any of these rare and uncommon causes listed above. Even so, it's best to be aware of other, less common possible explanations for vertigo, so that nothing is missed in the search for relief from dizziness.


Chimirri, S., Aiello, R., Mazzitello, C. et al. Vertigo/Dizziness as a Drugs’ Adverse Reaction. Journal of Pharmacology and Pharmacotherapeutics. 2013. 4(Suppl1):S104-S109.

Fernandez, A., Guerrero, A., Martinez, M. et al. Malformations of the Craniocervical Junction (Chiari Type I and Syringomyelia: Classification, Diagnosis and Treatment). BMC Musculoskeletal Disorders. 2009. 10(Suppl 1): S1.

Meldrum, J., and P. Prinsley. Perilyph Fistula: The Patients’ Experience. Journal of Laryngology and Otology. 2016. 130(6):526-31.

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