July 2014 

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Spontaneous CSF Otorrhea: A Neurotological Manifestation of Morbid Obesity?
About Dr. Meyer

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Summer greetings from Charleston! In this issue, Ted A. Meyer, M.D., PhD discusses spontaneous CSF Otorrhea and its possible connection to obesity. He explains why this awareness is important for otolaryngologists, and the steps for diagnosis and treatment.   

More information on Dr. Meyer follows the article below and is on our website. Your feedback or questions about the E-Udate articles, your patients, or any other ENT issue are always welcome. Write to us at [email protected] and please forward this E-Update to your colleagues who may be interested.

You may also find information in our SCOPE Spring 2014 newsletter helpful in your practice.

Many thanks for your continued interest and support. 
 

 

Paul R. Lambert, MD

Professor and Chair

Ted A. Meyer, M.D., PHD - Associate Professor and
Director, Cochlear Implant Center, MUSC
Spontaneous CSF Otorrhea:
A Neurotological Manifestation of Morbid Obesity?

Obesity has risen dramatically in the United States over the past generation. The percentage of adults considered obese is over 30% in many states. On the extreme of the obesity scale is morbid obesity, which can be defined as patients who are 100 pounds overweight, or as patients with a BMI greater than either 35 or 40. This group of patients has increased at an alarming rate which is reflected in my practice and likely in yours. I cannot recall the last clinic where I did not see at least one patient who is considered morbidly obese.

 

As health care providers, we should be well aware of the manifestations and common pathologies associated with obesity including hypertension, obstructive sleep apnea, diabetes, joint disorders, and a host of other medical problems. As a neurotologist, I see what I view as a specialty-specific manifestation of morbid obesity, a significant number of patients with skullbase defects and spontaneous CSF leaks into the temporal bone.

 

Patients in our practice commonly present to a primary care provider complaining of fullness and hearing loss in one ear. Fluid is noted behind the eardrum. The patient is often placed on oral antibiotics and/or a nasal steroid with the assumption that this is either an ear infection or Eustachian tube disease, and when this does not resolve, the patient is referred to a general otolaryngologist. A thorough history and physical examination by the otolaryngologist usually confirms a lack of significant Eustachian tube disease, a clear nasopharynx, and a unilateral serous otitis media. Often after further treatment, such as more nasal steroids, without improvement, a myringotomy with or without PE tube placement is performed. The patient drains clear fluid either intermittently or continuously through the tube/myringotomy, drops are often started, but the drainage continues. Often, after a substantial period of time, the fluid is collected, and sent for B-2 transferrin analysis. Results are dependent on the volume and composition of the otorrhea. If the test results are positive, a diagnosis of CSF otorrhea is confirmed. If the test results are negative or non-diagnostic, the diagnosis of CSF otorrhea should continue to remain high on the differential.

 

A CT scan is often obtained demonstrating a significantly attenuated skullbase. Many patients have a clear defect, and it is common to see an encephalocele herniating into the temporal bone. The defect can lie above the mastoid, above the ossicles, or even further anterior to the heads of the ossicles. Multiple defects are also common. Even when the defect is small, intracranial contents can seep through the defect and a substantial amount of dura or neural tissue from the temporal lobe can migrate into the temporal bone. Anecdotally, at surgery, a substantial inflammatory response is often noted around the encephalocele, presumably a response by the body to repair the skullbase defect and to protect the brain.

Axial CT scan demonstrating opacification of the left mastoid and middle ear. 
Coronal CT scan demonstrating a large skullbase defect on the left above the heads of the ossicles. Note the thinning in the same region on the right side.
Coronal CT scan demonstrating an opacified left mastoid with an area of thinning over the mastoid. Note the thinning in the same region on the right side.
Coronal CT scan demonstrating a large skullbase defect on the right above the heads of the ossicles. Note the solid tegmen in the scan on the left for comparison.
Coronal CT scan demonstrating a moth-eaten appearance of the tegmen mastoidium on the right with a solid tegmen for comparison on the left.
Coronal MRI scan demonstrating encephalocele herniating into the right temporal bone (thin arrow) with CSF in the air cells (thick arrow).

Audiological testing should demonstrate a conductive hearing loss in the affected ear and a flat tympanogram with low volume. If the patient has a PE tube without a significant amount of drainage, the volume could be large.

 

Treatment

Spontaneous CSF leaks can be intermittent. In my experience, however, I have not seen one stop permanently without intervention. Acetazolamide is commonly used for patients with benign intracranial hypertension (BIH) for its ability to decrease production of CSF, but I do not have firsthand knowledge, nor have I seen literature to support its use as a single treatment of CSF otorrhea. Untreated CSF leaks are associated with a relatively high rate of meningitis so surgical repair is recommended. In the past year, I have seen two patients develop meningitis from spontaneous CSF leaks.

 

The surgical approach is dependent upon the location of the leak, the patient's anatomy, the familiarity of the surgeon with the different options, and a host of other factors. In general, the repair is either through a mastoid, a middle fossa, or a combined approach. In most cases, I approach the skullbase defect from the mastoid initially, unless there is good reason not to. If needed, a small middle fossa craniotomy can be added if exposure is poor, if the anatomy is difficult, or if something out of the ordinary arises during the surgical procedure.

 

The surgeon has many different choices of materials available for repairing the defect. A combination of cartilage with or without perichondrium, free fascia grafts, fat grafts, and pedicled muscular or pericranial flaps have worked well in many surgeons hands over the years. I find these materials useful in the majority of skullbase repairs. However, in patients with multiple defects or with the entire skullbase demonstrating a moth-eaten appearance, a simple repair with natural material might not be appropriate, considering the potential for further problems during the remainder of the patient's life. Collagen matrices are readily available, bioglues can be helpful, titanium plates, and calcium-based products are also quite helpful in cases with large defects that might not be able to be covered well with the patient's own tissues.

Surgical view of encephalocele and inflammatory response in the left mastoid.
Surgical view of encephalocele in the middle ear.
Surgical view of tegmen defect from the middle fossa.

Complications from surgery, including the need for revision surgery, are rare. At MUSC, we admit patients after surgery at least overnight. If the patient has a PE tube, I normally leave it in place and use it to monitor for persistent CSF leak, and thus use the tube as a measure of the success of the procedure. Once the ear is determined to be dry, and if the PE tube remains in place and if the Eustachian tube is working relatively well, the PE tube can be removed in the clinic. Other surgeons remove the tube and repair the perforation at the time of the skullbase repair. Rarely, CSF diversion, with a VP or other shunt is required.

 

Summary

Spontaneous CSF Otorrhea is relatively common and appears to be related to obesity, and perhaps more so to morbid obesity.   Practicing otolaryngologists should be acutely aware of this pathology in patients with unilateral otitis media or unilateral rhinorrhea. In a morbidly obese patient with a persistent unilateral effusion, after a thorough history and physical examination, including evaluation of the nasopharynx, the next test in my evaluation is a high-resolution CT scan of the temporal bone. In a patient with a history of a myringotomy and/or PE tube with continual otorrhea, I also obtain a CT scan and search carefully for skullbase defects.

 

Ted A. Meyer, M.D., PHD

Medical University of South Carolina

- REFERENCES -

  • Goddard JC, Meyer T, Nguyen S, Lambert PR. New considerations in the cause of spontaneous cerebrospinal fluid otorrhea. Otol Neurotol. 2010 Aug;31(6):940-5.
  • Gubbels SP, Selden NR, Delashaw JB, Jr.et al. Spontaneous middle fossa encephalocele and cerebrospinal fluid leakage: diagnosis and management. Otol Neurotol. 2007;28:1131-9.
  • Kutz, J. W., Husain, I. A., Isaacson, B. and Roland, P. S. (2008) Management of Spontaneous Cerebrospinal Fluid Otorrhea. The Laryngoscope, 118: 2195-2199.
  • LeVay, A., Kveton, J. Relationship between obesity, obstructive sleep apnea, and spontaneous cerebrospinal fluid otorrhea. The Laryngoscope, 2008 Feb; 118:275-278.
  • Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol. 2006 Jul;115(7):495-500.
  • Scurry, W. C., Ort, S. A., Peterson, W. M., Sheehan, J.M., Isaacson, Jon, Hershey, PA. Idiopathic temporal bone encephaloceles in the obese patient. Otolaryngology-Head and Neck Surgery, 2007; 136: 961-965.
  • Stucken EZ, Selesnick SH, Brown KD. The role of obesity in spontaneous temporal bone encephaloceles and CSF leak. Otol Neurotol. 2012;33:1412-7.
  • Wang E. W., Vandergrift W., Schlosser RJ. Spontaneous CSF leaks. Otolaryngolgic Clinics of North America. 2011 Aug; 44(4). 
  • Woodworth, BA, Palmer, JN. Spontaneous cerebrospinal fluid leaks. Current Opinion in Otolaryngology & Head and Neck Surgery. 2009 Feb; 17(1):59-65.
About Dr. Meyer... 
  Ted A. Meyer

Ted A. Meyer , M.D., PHD

 Associate Professor
Director, Cochlear Implant Center
Advanced M.D. & PHD:
University of Illinois
Residency: Indiana University
Fellowship: University of Iowa

Special interest: Adult and pediatric otology, hearing loss, chronic ear diseases, Cholesteatoma, Cochlear implantation.

 

Medical University of South Carolina Department of Otolaryngology - Head & Neck Surgery

135 Rutledge Avenue, MSC 550, Charleston, SC 29425|Phone: 843.792.8299|Website: ENT.musc.edu|� 2014