Diagnosis, Treatment and Prevention of Perilymph Fistula

Creator: Dr. Smitha S. Dutt
Keywords: diagnosis of perilymph fistula, treatment of perilymph fistula, prevention of perilymph fistula, balance test, fistula test, hearting test, diuretics, blood patch, surgery, weights, strenuous activity, elevated sleeping position, electronystagmography, electrocochleography, tympanogram, audiogram, magnetic resonance imaging (MRI), computed tomography (CT), surgical exploration, cochlin-tomoprotein

Author - Dr. Smitha S. Dutt, PhD

Medically Reviewed by Dr. Sunil Shroff, MBBS, MS, FRCS (UK), D. Urol (Lond) on Apr 30, 2019

How do you Diagnose Perilymph Fistula?

Perilymph fistula is a challenging condition to be diagnosed since there is no clear defining protein that has been detected. You are first asked about your medical history and if you experienced any trauma in the recent past. A physical examination of the ear, neck, nose, and throat followed by an examination of balance and the cranial nerve are the initial stages in diagnosis. There are diagnostic tests that can verify the presence of perilymph fistula. They are:

Balance test or Electronystagmography (ENG) - Although it is utilized in those with perilymph fistula, it is not accurate and is normally used to test the function of the vestibule. The Fukuda/Unterberger test is a clinically used test to test for balance.
Fistula testing - The fistula between the middle and inner ear is tested with a range of pressures that span positive to negative values. Your balance and the level of dizziness experienced in connection with these changes in pressure are recorded. A microscope is used to detect the leaking of the perilymph, though it is difficult to detect the leak since the fluid leaks out slowly as beads. If there is a continuous discharge, then it is the cerebrospinal fluid is leaking and can cause the excess volume of fluid.
Hearing tests or tympanogram, audiogram, or electrocochleography - These audiometric tests test for the level of hearing loss.
Magnetic resonance imaging (MRI) - An increase in the MRI signal is an indication of the presence of a perilymphatic fistula. This test is also used to detect Meniere`s disease.
Computed tomography- This imaging technique is useful in the initial acute stage when you begin to notice serious symptoms. However, following the acute phase, CT is unable to accurately diagnose the perilymph fistula except for the presence of abnormalities that may cause the fistula.
Surgical exploration of the round or oval windows - This is a reliable technique to confirm the diagnosis of perilymph fistula. This is performed only when the hearing loss is moderate. If the hearing loss is serious, surgical exploration is not recommended.

Recent research indicates the potential of a perilymph-specific protein called Cochlin-tomoprotein. This protein helps to specifically diagnose perilymph fistula since it is not detected in the cerebrospinal fluid, serum, or saliva.

How do you Treat Perilymph Fistula?

Perilymph fistula, if detected early, can be treated with limited damage to hearing. If many weeks pass before treatment is initiated, then this affects hearing.

Treatment includes medical treatment, blood patch, or surgery.

1. Medical treatment - Diuretics are prescribed for the treatment of perilymph fistula.

2. Blood patch - This is an outpatient procedure and can be used as the first line of treatment, and you can go home the same day. In certain cases, the patch has to be repeated for effective treatment. In this procedure, your own blood will be injected into to help seal the inner ear window with the natural fibrin glue in the blood.

The clear advantages of the procedure are low cost, a short procedure and minimum psychological stress to the patients.

3. Surgery - If medicines are not tolerated or the blood patch is not effective, surgery is recommended. A speculum is used to provide a magnified view of the ear. An incision is made to lift the flap to enter the middle ear. The pressure is applied through certain techniques to view the perilymph leak to better ascertain how to patch the leak with a graft.

How to Prevent Perilymph Fistula?

After treatment, the following recommendations should be followed to avoid the condition from occurring again:

Avoid lifting weights greater than 20 pounds
Sleep by keeping your head at a slightly elevated position
Avoid activity that causes a lot of strain

References:

Hornibrook J. A balance test for chronic perilymph fistula. Int J Otolaryngol. 2012;Article ID 163691, 8 pages.
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Hornibrook J. The postural and cognitive disabilities of chronic perilymph fistula (PLF) after mild head trauma. Ann Clin Case Rep.2018;3:1514.
UCI health. Perilymphatic fistula - (http://www.ucihealth.org/medical-services/ear-nose-throat-ent/hearing-ear-disorders/perilymphatic-fistula)
Bhatia N, Lehrer JF. Perilymphatic fistula: an approach to diagnosis and management that provides surer diagnosis and provides medical and surgical management options: report of six illustrative recent cases. Int Tinnitus J. 2012;17(1):61-66.
Al Felasi M et al. Perilymphatic fistula of the round window. Eur Ann Otolaryngol, Head and Neck Dis. 2011;128(3):139-141.
Ikezono T et al. The diagnostic performance of a novel ELISA for human CTP (Cochlin-tomoprotein) to detect perilymph leakage. PLOS One. 2018; 13(1):e0191498 - (https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0191498&type=printable)
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