eMedicine Specialties > Emergency Medicine > Ear, Nose, & Throat

Perilymph Fistula: Differential Diagnoses & Workup

Author: Nancy E Conroy, MD, Clinical Assistant Instructor, Staff Physician, Department of Emergency Medicine, SUNY Downstate Medical Center/Kings County Hospital Center, Brooklyn
Coauthor(s): Mert Erogul, MD, Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center
Contributor Information and Disclosures

Updated: Jun 30, 2009

Differential Diagnoses

Stroke, Hemorrhagic

Other Problems to Be Considered

Vertebrobasilar dissection
Cerebellar stroke
Autoimmune etiology
Acoustic neuroma
Hereditary hearing loss
Vascular disease

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.

More on Perilymph Fistula

Overview: Perilymph Fistula
Differential Diagnoses & Workup: Perilymph Fistula
Treatment & Medication: Perilymph Fistula
Follow-up: Perilymph Fistula
References
[ CLOSE WINDOW ]

References

  1. 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].

  2. Bhansali SA. Perilymph fistula. Ear Nose Throat J. Jan 1989;68(1):11, 14-6, 21-8. [Medline].

  3. Bruzzone MG, Grisoli M, De Simone T, Regna-Gladin C. Neuroradiological features of vertigo. Neurol Sci. Mar 2004;25 Suppl 1:S20-3. [Medline].

  4. deJong AL. Congenital perilymphatic fistula. Arch Otolaryngol Head Neck Surg. Nov 1998;124(11):1279-81. [Medline].

  5. Fitzgerald DC. Head trauma: hearing loss and dizziness. J Trauma. Mar 1996;40(3):488-96. [Medline].

  6. 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].

  7. 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].

  8. 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].

  9. Jones R. Current status of perilymphatic fistula. Arch Otolaryngol Head Neck Surg. Nov 1998;124(11):1281-2. [Medline].

  10. Meyerhoff WL, Marple BF. Perilymphatic fistula. Otolaryngol Clin North Am. Apr 1994;27(2):411-26. [Medline].

  11. Minor LB. Labyrinthine fistulae: pathobiology and management. Curr Opin Otolaryngol Head Neck Surg. Oct 2003;11(5):340-6. [Medline].

  12. Wall C, Rauch SD. Perilymph fistula pathophysiology. Otolaryngol Head Neck Surg. Jan 1995;112(1):145-53. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

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

[ CLOSE WINDOW ]

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

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.