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Perilymph Fistula

Nancy E Conroy, MD, Clinical Assistant Instructor, Staff Physician, Department of Emergency Medicine, SUNY Downstate Medical Center/Kings County Hospital Center, Brooklyn
Mert Erogul, MD, Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center

Updated: Jun 30, 2009

Introduction

Background

A perilymph fistula (perilymphatic fistula [PLF], labyrinthine fistula) is a pathologic communication between the fluid-filled space of the inner ear and the air-filled space of the middle ear, most commonly occurring at either the round or oval window. The primary manifestations of perilymph fistulization are sudden or progressive fluctuating sensorineural hearing loss and vertigo. Other symptoms that may be present include tinnitus, disequilibrium, and aural fullness.

This clinical entity was proposed more than a century ago, yet it remains a topic of controversy, especially regarding the occurrence of spontaneous perilymph fistula (PLF). Trauma and poststapedectomy are etiologies proven to cause PLFs and must be considered in the appropriate settings. A PLF should be considered in pediatric patients with recurrent meningitis, and the option of middle ear exploration should be pursued. It is likely that most pediatric patients with PLFs will have an anatomic abnormality of their middle ear or labyrinth. It is also known that although several potential pathways exist between the perilymphatic space and the middle ear, actual leaking of fluid can be difficult or impossible to prove.

There is no agreed upon diagnostic test with enough sensitivity and specificity to identify the presence or absence of perilymph fistula. This has made it difficult to establish criteria to determine when surgical exploration might be indicated. Additionally, there are no universally accepted criteria to confirm diagnosis at surgery; the determination of the presence or absence of PLF is the subjective decision of the surgeon. Additionally, the presence of clear fluid in the middle ear at the time of surgery may represent perilymph or may be CSF, serum, or local anesthetic. No reliable and accurate test is currently available to reliably distinguish these fluids from one another. No studies have been reported to account for the substantial placebo effect of a surgical procedure for vertiginous symptoms in patients with suspected PLF, and no appropriate long-term follow up of patients after surgery has been reported.

The presenting complaint and symptoms of perilymph fistula are often consistent with Meniere syndrome. Therefore, in the absence of prior surgery or definite traumatic event, distinguishing a perilymph fistula from Meniere syndrome may be difficult.

Pathophysiology

Most commonly, a tear in the round or oval window leads to loss of perilymph into the middle ear. This condition may be the result of stapes prosthesis surgery, trauma, barotrauma, or bony erosion due to infection or neoplasm, or it may be idiopathic. In children, perilymph fistulas are associated with congenital anomalies of the middle or inner ear.

Mortality/Morbidity

In children, the condition may be associated with recurrent meningitis. Acute or chronic perilymph fistulas may significantly affect quality of life.

Sex

Prevalence of perilymph fistula is higher in females than in males.

Age

Perilymph fistulas occur in young children with congenital abnormalities; otherwise, the condition is not known to be age specific.

Clinical

History

History for perilymph fistula may include the following:

Fluctuating sensorineural hearing loss that may be sudden or progressive
Vestibular symptoms
- Vertigo, with or without head position changes
- Dysequilibrium
- Motion intolerance
- Nausea and vomiting
- Disorganization of memory and concentration
- Perceptual disorganization in complex surroundings such as crowds or traffic
Tinnitus - May be roaring in nature
Aural fullness

Physical

Positive test results for vestibular dysfunction or hearing loss, while helpful, are not pathognomonic.
Romberg (feet together) or tandem Romberg (heel-to-toe) test findings may be positive.
Positional nystagmus and benign paroxysmal positional vertigo often are associated with traumatic perilymph fistula.
Sensorineural hearing loss may be confirmed by audiograms.

Causes

Prior stapes surgery
Head trauma, including whiplash injuries
Barotrauma
Acoustic trauma
Idiopathic or spontaneous, possibly related to episodes of valsalvae, nose blowing, or physical exertion (The existence of spontaneous perilymph fistula is still an area of controversy among otologists.)

Differential Diagnoses

Stroke, Hemorrhagic

Workup

Imaging Studies

No diagnostic test can be considered a criterion standard for perilymph fistula, and none are immediately useful in the emergency department.
Clinical history and symptoms are more sensitive than radiographic studies for the assessment of perilymph fistula.
A CT scan or MRI may be indicated with a history of head trauma if temporal bone fracture is considered, but their use as diagnostic studies for nontraumatic perilymph fistulas is questionable.
- High-resolution CT scans are inadequate for evaluation of the round and oval window regions.
- MRI with intrathecal gadolinium enhancement can detect inner and middle ear communication, but a more cost-effective modality is preferred.

Other Tests

Otologists may use the following tests to diagnose perilymph fistulas:
- Audiograms to detect hearing loss
- Electrocochleography
- Fistula tests (Hennebert sign): The subjective fistula test is performed by applying positive and negative pressure to the intact eardrum using a pneumatic otoscope. Positive results include the elicitation of nystagmus or onset of dysequilibrium with the sensation of motion or nausea. Some otologists administer the test with electronystagmography or by using platform posturography.
Perilymph labeling methods
- Initial studies used intrathecal or intravenous fluorescein, but these have been abandoned for perilymph-specific protein testing, such as beta-2 transferrin, apo D, and apo J.
- Beta-2 transferrin, a protein found in perilymph and CSF but not in serum, has been the most actively studied. Initial enthusiasm for a Western blot assay for this protein has wavered due to low sensitivity. The presence of beta-2 transferrin in perilymph has been unreliable, possibly due to current collection methods or small sample volumes, and false-positive results may be secondary to CSF contamination during collection.
- Focus has recently switched to the efficacy of apolipoprotein D (apo D) as a marker for perilymph with encouraging results.
- Results of a study by Ikezono et al strongly suggest that cochlin-tomoprotein may be a specific marker of perilymph leakage. Additionally, cochlin-tomoprotein could be a potential marker, allowing definitive diagnosis of perilymph fistula related hearing loss and vestibular disorders.¹

Procedures

The current criterion standard for confirmation of perilymph fistula is direct visualization either by operative middle ear exploration or by in-office endoscopy.
The subjectivity of this diagnostic maneuver has been questioned because no universally accepted criteria exist for diagnosis during surgery. More objective tests are being developed, particularly those that identify compounds specific to perilymph upon the examination of the fluid contents.

Treatment

Emergency Department Care

The primary purpose of emergency evaluation is to seek other, more emergent causes of symptoms. However, consider the diagnosis with hearing loss and vertigo following head trauma. Vertigo suppressants, such as diazepam and meclizine, may be used, although their benefit remains unproven in this setting. Medical therapy is rarely reported. Some reports exist of spontaneous healing with bedrest, head elevation to 30°, and avoidance of lifting or middle-ear pressure–increasing activities.

Consultations

Consult with an otolaryngologist.

Follow-up

Further Inpatient Care

Bedrest with the head of the bed elevated to 30°
Avoidance of lifting or pressure-increasing activities (ie, Valsalva maneuver)
Surgical intervention is the principle approach to treatment when perilymph fistula is diagnosed. The site of perilymph leak is covered with some form of autologous tissue.

Further Outpatient Care

Strict bedrest for 5-7 days or until reevaluation
Elevation of the patient's head above the heart level
Avoidance of straining
Avoidance of nose blowing or Valsalva maneuver
No lifting (>10 lb)
Stool softeners

Complications

Both the patient and the physician should be prepared for no response to treatment, especially with regard to hearing improvement. Recurrence of postoperative signs and symptoms may occur in 21-47% of patients.
Perilymph fistula may be associated with recurrent meningitis in children.

Prognosis

Because of the difficulty in making the diagnosis of perilymph fistula, the natural history of this disorder is unknown.
Significant improvement in symptoms that are presumed to be from perilymph fistula may occur with conservative treatment.
Surgery is more successful in relieving vertiginous symptoms than in improving hearing.

Miscellaneous

Special Concerns

The condition may be a cause of recurrent meningitis in children.
In head trauma cases, hearing loss with vertigo suggests a perilymph fistula syndrome until proven otherwise.

References

Ikezono T, Shindo S, Sekiguchi S, Hanprasertpong C, Li L, Pawankar R, et al. Cochlin-Tomoprotein: A Novel Perilymph-Specific Protein and a Potential Marker for the Diagnosis of Perilymphatic Fistula. Audiol Neurootol. Apr 15 2009;14(5):338-344. [Medline].
Bhansali SA. Perilymph fistula. Ear Nose Throat J. Jan 1989;68(1):11, 14-6, 21-8. [Medline].
Bruzzone MG, Grisoli M, De Simone T, Regna-Gladin C. Neuroradiological features of vertigo. Neurol Sci. Mar 2004;25 Suppl 1:S20-3. [Medline].
deJong AL. Congenital perilymphatic fistula. Arch Otolaryngol Head Neck Surg. Nov 1998;124(11):1279-81. [Medline].
Fitzgerald DC. Head trauma: hearing loss and dizziness. J Trauma. Mar 1996;40(3):488-96. [Medline].
Fitzgerald DC, Getson P, Brasseux CO. Perilymphatic fistula: a Washington, DC, experience. Ann Otol Rhinol Laryngol. Oct 1997;106(10 Pt 1):830-7. [Medline].
Friedland DR, Wackym PA. A critical appraisal of spontaneous perilymphatic fistulas of the inner ear. Am J Otol. Mar 1999;20(2):261-76; discussion 276-9. [Medline].
Grimm RJ, Hemenway WG, Lebray PR, Black FO. The perilymph fistula syndrome defined in mild head trauma. Acta Otolaryngol Suppl. 1989;464:1-40. [Medline].
Jones R. Current status of perilymphatic fistula. Arch Otolaryngol Head Neck Surg. Nov 1998;124(11):1281-2. [Medline].
Meyerhoff WL, Marple BF. Perilymphatic fistula. Otolaryngol Clin North Am. Apr 1994;27(2):411-26. [Medline].
Minor LB. Labyrinthine fistulae: pathobiology and management. Curr Opin Otolaryngol Head Neck Surg. Oct 2003;11(5):340-6. [Medline].
Wall C, Rauch SD. Perilymph fistula pathophysiology. Otolaryngol Head Neck Surg. Jan 1995;112(1):145-53. [Medline].

Keywords

perilymph fistula, PLF, perilymph leak, inner ear, perilymph fistula treatment, perilymph fistula causes, perilymph fistula symptoms, perilymphatic fistula, labyrinthine fistula, acute perilymph fistula syndrome, chronic perilymph fistula syndrome, hearing loss, sensorineural hearing loss, vertigo, tinnitus, disequilibrium, aural fullness, benign positional vertigo

Contributor Information and Disclosures

Author

Nancy E Conroy, MD, Clinical Assistant Instructor, Staff Physician, Department of Emergency Medicine, SUNY Downstate Medical Center/Kings County Hospital Center, Brooklyn
Nancy E Conroy, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, Medical Society of the State of New York, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Mert Erogul, MD, Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center
Mert Erogul, MD is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Medical Editor

Jerry Balentine, DO, Professor of Emergency Medicine, New York College of Osteopathic Medicine; Executive Vice President, Chief Medical Officer, Attending Physician in Department of Emergency Medicine, St. Barnabas Hospital
Jerry Balentine, DO is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American College of Physician Executives, American Osteopathic Association, and New York Academy of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Mark W Fourre, MD, Program Director, Department of Emergency Medicine, Maine Medical Center; Associate Clinical Professor, Department of Surgery, University of Vermont School of Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD, Medical Director, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: WebMD Salary Employment

Further Reading