What Is A Perilymph Fistula?

Overview

A perilymph fistula (PLF) is when there is a disruption to the thin membrane that connects the inner ear and outer ear. This results in perilymph fluid, which is found in the fluid-filled inner ear, leaking into the air-filled middle ear and causing pressure changes. Typically, fistulas occur at the round or oval window in the inner ear and the structures around it. This may also be referred to as a labyrinthine fistula.1 This can cause issues with hearing and/or the vestibular system. Furthermore, perilymph fistula may cause symptoms such as hearing loss, vertigo, imbalance, dizziness, and tinnitus. 

Causes of perilymph fistula

The cause of perilymph fistula can be divided into two classifications: symptomatic or idiopathic. This means those that have an identifiable cause and those that do not.  Some identifiable causes include surgical complications, head trauma, middle and inner ear diseases, and barotrauma. PLF may also be triggered by internal events such as sneezing or coughing or external events such as flying or heavy lifting.  

Research also indicates that a potential cause of idiopathic PLF could be micro-fissures. Micro-fissures are small cracks in bones, and in this case, these occur in the temporal bone, more specifically near the oval window, round window, and ear canal.2 If you believe that this may have occurred, you should contact a healthcare professional.

After contacting a healthcare provider, they will arrange a variety of diagnostic tests to confirm a diagnosis and develop a treatment plan accordingly.1 Usually, this involves more conservative methods like bed rest. However, depending on the severity of the PLF, further treatment may be necessary.3

Signs and symptoms of perilymph fistula

Most symptoms that occur due to perilymph fistula are easily identifiable and are often more noticeable during certain acts that affect the pressure within your ears. Some of the most common symptoms to look out for include:3

  • Hearing loss
  • Dizziness
  • Vertigo
  • Nausea
  • Balance impairments
  • Headaches
  • Tinnitus

These symptoms are often only present in one ear at a time but may occur in both ears occasionally. 

Management and treatment for perilymph fistula

Like many other conditions, the most suitable treatment for a perilymph fistula depends on the severity of the impairment. The first step after suspecting a PLF is to contact a professional to confirm your diagnosis.

Milder cases may resolve by themselves alongside some bed rest. Reducing your activity can allow enough time for the rupture to heal and prevent any further damage occurring from activity-related triggers. Typically, one to two weeks of bed rest is recommended.3

 However, for more severe cases, further treatment options may be suggested. A new treatment called blood patch injections has been introduced as a next-line treatment and, so far, has had promising results. This procedure involves an injection of your blood into the middle ear to help repair the damaged membrane.3

If these procedures are suitable, the next recommended treatment is surgery. The surgery involves gently placing tissue graft over the damaged membranes between the inner and outer ear to repair the tissue damage that is causing the adverse effects.  Typically, people feel better after only a couple of days, but it may take up to a few weeks for the full healing process. In the meantime, you should be sure to follow all post-op guidelines your doctor provides you to ensure the best and quickest recovery. Some of these measures include sleeping with your head elevated and avoiding trigger activities.3

Diagnosis of perilymph fistula

Although it was previously difficult to diagnose PFL because the symptoms are similar to other diseases, persistent research has led to the development of diagnostic criteria.2

The criteria indicate that if a patient is experiencing 1-3 of the symptoms listed above preceding a barotrauma or direct trauma, they should be considered for further testing. 

The two types of further testing are lab testing using a perilymph biomarker and an observation of the perilymph leakage. After treatment of the leak, the symptoms should resolve, and there should be no observable leakage.2

To diagnose PFL, a combination of CT and MRI scans is used instead of using exploratory procedures. So far, a combination of these techniques has had a high success rate due to their high sensitivity for PFLs. MRIs are used mostly to identify congenital abnormalities, whereas CTs are used to eliminate any other possible causes.2

Other tests that may be used to confirm a diagnosis range from hearing and balance tests to tests that measure inner ear pressure to a PFL test, which is when pressure is increased in your ears, and a professional will analyse the effect it has on your eye movements.3

FAQs

Can perilymph fistula be prevented?

Yes, you can take steps to minimise your risk of developing PLF. These include avoiding loud noises or wearing ear plugs, preventing ear infections, avoiding straining, and activities that increase intracranial pressure, such as heavy lifting.3

How common is perilymph fistula?

PLF is a rare condition and only occurs in 1.5/100,000 adults.3 If you are experiencing any of the above-mentioned symptoms, it is likely that you may be experiencing a similar condition that is more common. For example, Meniere syndrome, secondary endolymphatic hydrops, or acoustic neuromas.4 However, it is important to contact a professional to confirm any diagnosis. 

Who is at risk of perilymph fistula?

Anyone can develop perilymph fistulas at any age, even infants. However, it is more common in people who are prone to recurrent ear infections.3

When should I see a doctor?

As soon as you start experiencing any changes to your hearing or balance or any other named symptoms, you should contact a healthcare professional. It is also good to consider reaching out if you have experienced physical trauma to the head or ears or taken part in any activities that put you at risk of developing a perilymph fistula.3

Summary

Perilymph fistula is a disruption to the membrane that separates the fluid-filled inner ear and air-filled middle ear. This causes pressure changes, which result in hearing and balance impairments. PLF is mostly caused by some form of trauma to the head region but can also be triggered by internal and external events that affect intracranial pressure. If you suspect you have developed PFL, you are advised to seek medical attention, and a healthcare professional will be able to perform the necessary diagnostic tests. Following a PLF diagnosis, the most common treatment is bed rest, ranging from a few days to a few weeks. If this is not enough, further treatments such as blood patch injections or surgery may be suggested. Although that may sound intimidating, a professional will explain the procedure in more depth and provide all the risks and limitations of all the available options.

References

  1. Furhad S, Bokhari AA. Perilymphatic fistula. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 Nov 1]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK563221/
  2. Sarna B, Abouzari M, Merna C, Jamshidi S, Saber T, Djalilian HR. Perilymphatic fistula: a review of classification, aetiology, diagnosis, and treatment. Front Neurol [Internet]. 2020 Sep 15 [cited 2023 Nov 1];11:1046. Available from: https://www.frontiersin.org/article/10.3389/fneur.2020.01046/full 
  3. Althaus SR. Perilymph fistulas: The Laryngoscope [Internet]. 1981 Apr [cited 2023 Nov 1];91(4):538–62. Available from: http://doi.wiley.com/10.1288/00005537-198104000-00007
  4. Battisti AS, Haftel A, Murphy-Lavoie HM. Barotrauma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 Nov 1]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK482348/
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Ellie Kerrod

BSc Neuroscience - The University of Manchester, England

I’m a Neuroscience BSc student studying at The University of Manchester, UK and have experience in medical writing. I am passionate about ensuing that everyone can assess accurate medical information and I am committed to bridging the gap between complex medical concepts and the public.

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