Background

Leakage of cerebrospinal fluid (CSF) though the ear structures is a rare but potentially lifethreatening situation that requires rapid intervention. The presence of an abnormal
communication of the sterile subarachnoid space with the flora of the sinonasal tract places
the patient at great risk for meningitis. Indeed, an episode of meningitis may be the
presenting problem for a person with an otologic CSF leak.
Even though a leakage of CSF is occurring through otologic structures, actual leakage of
the fluid from the ear is not always present. CSF otorrhea occurs only if a perforation in the
eardrum or a defect in the external ear canal is present. This is often the case when the
leakage results from trauma or previous ear surgery. However, in the absence of such a
defect, the fluid flows down the eustachian tube and manifests as a clear rhinorrhea. In this
situation, it must be differentiated from a spinal fluid leak from other areas of the skull base,
such as the sinuses. The reverse is also important to remember; just because the leak is
through the nose does not mean that an otologic source is not a possibility.
The subject of CSF otorrhea covers a broad range of potential pathologies. Most of such
leaks are postoperative and are a recognized complication of acoustic neuroma surgery.
Some occur as a result of middle ear surgery or erosive disease, such as cholesteatoma,
sometimes many years later. Some may be the result of congenital abnormalities in the
formation of the inner ear and the mastoid. In this article, the authors attempt to discuss all
these related pathologies.
See the image below.

Coronal CT image of the temporal bone demonstrates a bone defect (small

arrows) in the tegmen tympani with a protruding soft-tissue meningoencephalocele (large arrows). This
patient had cerebrospinal fluid otorrhea after mastoidectomy.

Epidemiology
Frequency
Because this problem is so rare, the exact frequency with which it occurs is unknown. Leaks
as a postoperative complication of skull base surgery represent the bulk of cases. This
complication has been reported to occur in 6-12% of such cases. Leakage as a result of
temporal bone fracture is the second most common etiology and occurs in 21% of patients
with temporal bone fractures. Spontaneous leakage is rare, with less than 500 cases having
been reported in the literature worldwide. Such leakage is more common in children (72%)

than adults. Although rare, spontaneous leakage represents the greatest challenge to the
clinician because the source of the leakage is not readily obvious. [1]
Meningitis is often the presenting symptom. It is found in 93% of children and 36% of adults
with spontaneous CSF leakage. Sensorineural hearing loss is another significant morbidity
associated with spontaneous leaks in children and is present 82% of the time. Seizures are
also a potential morbidity.[2, 3]

Etiology
The underlying etiology of a spinal fluid leak through the temporal bone is a violation of the
bony and meningeal barriers that separate the subarachnoid space from the middle ear and
mastoid. This means that a defect must exist not only in the bone, but also in the dura
mater. Such problems may be postsurgical, posttraumatic, congenital, or related to otologic
disease.

Pathophysiology
CSF leakage can result from a number of underlying pathologies. Such leaks can broadly
be categorized into acquired and congenital.

Congenital cerebrospinal fluid leak

Congenital causes can be due to defects in the otic capsule itself, abnormal patency of
pathways associated with the otic capsule, and defects distant from the otic capsule.
Although congenital sources are more common in children, they can occur in people of any
age and can even be observed in the geriatric population. [4]

Acquired cerebrospinal fluid leak

Acquired leaks result from temporal bone trauma, surgery, or infectious or neoplastic
causes. Acquired spinal fluid leakage is far more common than congenital leakage.
Postoperative leakage following surgery is probably the most common cause of acquired
CSF leaks. It is a recognized complication of acoustic neuroma removal and other skull
base surgery. These leaks are usually evident in the early postoperative period. They rarely
occur more than 2 months postoperatively.
Mastoid surgery for chronic ear disease is also a potential cause of an acquired CSF leak.
When the dura is violated intraoperatively, the defect should be repaired immediately if
possible. Frequently, however, the dura is not injured, but a defect is left in the bony plate of
the tegmen. Over the years, the continuous pulsations of the CSF cause the dura to thin,
allowing the arachnoid or brain to prolapse through this defect. This dura may become thin
and spontaneously rupture, resulting in a leak of CSF many years after the initial surgery. In
addition to dura, portions of brain tissue may also prolapse through the defect, resulting in
an encephalocele.
Even in the absence of surgical intervention, similar problems may result from middle ear
disease, most notably cholesteatoma. Cholesteatoma may erode the tegmen plate and
allow herniation of dura or brain to occur over time. [5]

Spontaneous cerebrospinal fluid leak

Spontaneous leakage is leakage that occurs without an obvious antecedent pathology.

Such leakage is usually due to some congenital defect in the temporal bone.
Understandably, such defects are usually evident in childhood.
Spontaneous CSF leakage may result from a number of congenital deformities of the ear.
Mondini deformity, an abnormal development of the cochlea, is frequently associated with
CSF leakage. Such patients often have a patency at the lateral aspect of their internal
auditory canal, allowing direct movement of CSF into the inner ear. A second defect, usually
of the annular ring of the stapes footplate, then results in drainage of CSF into the middle
ear. This usually causes loss of the remainder of hearing. A similar presentation may be
observed in a patient with a widely patent cochlear or vestibular aqueduct.
Other deformities of the inner ear are also associated with spontaneous CSF leak. CSF can
leak through a patent Hyrtl fissure, a congenital fusion plane found between the otic capsule
and the jugular bulb (also termed the tympanomeningeal fissure). Such leakage may not be
associated with any inner ear abnormalities, and the patient may have no evidence of a
sensorineural hearing loss. Similar presentations may be observed in an abnormal patency
of the petromastoid canal, which normally carries the subarcuate artery. Such patients may
present with recurrent bouts of meningitis associated with what appears to be a unilateral
serous otitis. Frequently, the true nature of the problem is not realized until a myringotomy is
performed, at which time an unceasing flow of clear fluid is returned.
Although spontaneous CSF leakage is more common in children, reports of it occurring in
people of greater age are increasing. In adults, spontaneous CSF otorrhea is often
associated with a defect in the bone separating the middle ear space and the cranial vault.
Such bony dehiscences are most commonly found on the floor of the middle fossa, along
the tegmen plate. Tegmen dehiscences are found in up to 30% of autopsy specimens.
Continuous pressure of the CSF over the years results in formation of a meningocele or
encephalocele. Defects in the middle fossa are associated with 88% of spontaneous leaks
in adults. The remainder of spontaneous leaks are due to posterior fossa defects and
arachnoid granulations, normal structures that absorb CSF. These structures are usually
associated with the venous sinuses of the dura mater, but they have been found within the
temporal bone on some autopsy specimens. Spontaneous leaks have recently been shown
to be often associated with increased intracranial pressure. This is often manifested by a
partially empty sella on the MRI scan.

Presentation
The chief presenting symptom one would expect when a CSF leakage through the ear is
present is a clear watery drainage from the ear. This, however, is not always present and
does not occur unless the eardrum or canal is in some way violated. If this is not the case,
the leakage may be evident as a clear watery nasal discharge. This discharge may be
positional or intermittent in nature and may only become apparent during straining or
leaning forward. Some patients may notice no discharge at all but rather may report a
strange salty taste in the back of the throat.
Often, a patient with an otogenic spinal fluid leakage presents with a unilateral hearing loss.
The nature of the hearing loss is important. A sensorineural hearing loss suggests an
associated abnormality of the inner ear, such as a Mondini deformity. However, the loss

may be conductive, suggesting a leak elsewhere in the temporal bone. Such a presentation
may be very similar to that of a unilateral serous otitis media and may be mistaken for such.
The realization of the true identity of the fluid may only be made at the time of myringotomy,
when clear fluid pours unceasingly from the incision. When such an event occurs, the ear
canal is often packed to prevent continuous drainage from the ear and allow the eardrum to
heal. Other authors think that packing may cause stasis and provide a nidus for infection,
hastening meningitis, and they recommend only a loosely placed, frequently changed sterile
cotton ball placed in the conchal bowl. A full workup should be immediately undertaken.
A common presentation for a spontaneous spinal fluid leakage is an unexplained episode of
meningitis. In such situations, the source of the leakage may not be readily apparent. A
history of unilateral hearing loss or chronic ear disease may suggest which side is involved.
Absence of otologic symptoms and a history of sinonasal disease may suggest an anterior
source of leak. In situations of leakage following surgery and trauma, the source of the leak
usually is readily apparent.
Patients suspected of having a spinal fluid leakage should undergo complete otologic,
neurologic, and head and neck examination. Use binocular microscopy to examine the ears.
Pneumatic otoscopy may be helpful in demonstrating fluid within the middle ear space,
especially when that fluid is very clear. Tuning fork evaluations can be used to demonstrate
a conductive hearing loss. If possible, perform complete audiometric evaluation.
An important part of the physical examination is an attempt to demonstrate the leak.
Usually, the patient is asked to perform for the examiner the maneuver with which they
notice the leak, such as straining or leaning forward. With the Dandy maneuver, the patient
leans forward with the head pointed down while performing a Valsalva. This maneuver often
results in the appearance of clear fluid from the nostril of a person with a CSF leak. The
side of the nostril from which the leakage occurs usually agrees with the side of the otologic
source.

Indications
Leakage of CSF though the ear structures places the patient at great risk of meningitis.
Surgery is therefore indicated to repair this problem in most situations. Leaks occurring
following trauma or recent skull base surgery can often be treated conservatively using a
pressure dressing and a lumbar drain. Surgery is recommended for patients in whom this
conservative therapy is unsuccessful.

Relevant Anatomy
See Etiology and Pathophysiology.

Contraindications
In patients with postsurgical and traumatic leaks, surgery may not be indicated because it is
reserved for those in whom conservative therapy is unsuccessful.

Laboratory Studies

Unless the source is obvious, such as in a case of recent surgery or trauma, attempt to
document that the draining fluid is in fact CSF.

Testing the fluid for glucose level helps distinguish spinal fluid from nasal
secretions, which are low in glucose. Contamination of the specimen with blood, serum, tears,
or saliva may lead to a false-positive result.
Testing for beta2 transferrin, a substance found only in CSF, may identify the true
nature of the substance with a greater degree of certainty. Beta2 transferrin is also found to a
lesser degree in perilymph, but perilymph would not be expected to cause a large volume of
leakage. However, the test for beta2 transferrin may not be readily available and the result
may not be returned for days to weeks.

Imaging Studies

Localization of an otogenic spinal fluid leakage is usually accomplished using imaging

studies.
Obtain a high-resolution CT scan with axial and coronal sections. Unless an otologic
source is certain, the scan should cover all 3 cranial fossae.
Check the otic capsule for abnormal morphology, such as a Mondini deformity.
Note the sizes of the vestibular and cochlear aqueducts. Check the tegmen plates of the
posterior and middle fossae for defects.
Localization of leakage sites with CT scanning may be enhanced with the use of
intrathecal contrast, such as iopamidol or iohexol.
The presence and location of pneumocephalus on CT scanning may help to
identify and localize a CSF leak.
MRI may be helpful in pinpointing the site of a leak.[6]
Spinal fluid, bright on T2 sequences, may be observed entering the middle ear.
In cases where a tegmen defect is observed on CT scanning, MRI may
demonstrate whether or not brain tissue is prolapsed into the middle ear. Because this is
important information for surgical planning, an MRI is a critical adjunct when a defect is found
in the bony plate of the tegmen or the posterior fossa.
A partially empty sella has recently been recognized as a possible sign of
increased intracranial pressure. The increased CSF pressure causes infiltration of the sella
with CSF and displacement of the pituitary tissue. This finding, previously thought to be
unrelated and incidental, has been shown to occur in 71% of patients with spontaneous CSF
leaks.

Medical Therapy

Although the presence of a CSF leak places the patient at risk for meningitis, the use of
prophylactic antibiotics is controversial. Many believe the use of antibiotics in the
absence of infection has the effect of selecting out resistant organisms among the
normal flora, complicating the treatment of meningitis when it does arise. They believe
antibiotics should be withheld unless signs and symptoms of meningitis occur and
diagnosis is confirmed by spinal tap. Then, broad-spectrum antibiotics are instituted until
cultures and sensitivities are returned.
Others believe that the routine use of prophylactic broad-spectrum antibiotics is advised
in CSF otorrhea. The initial signs of meningitis may be subtle, and irreparable harm may
occur to the CNS by the time obvious meningitic signs are present. A number of
published studies have shown that the risk of meningitis is significantly reduced when
prophylactic antibiotics are used in posttraumatic CSF leakage, and their use in this
situation is generally recommended. However, the results of these studies can probably
not be extrapolated to postoperative and spontaneous leaks.
Prophylactic antibiotics should always be used in patients who are immunosuppressed
and when soilage of the central nervous system is obvious. In these situations,

overwhelming infection may be present before signs of meningitis are noted. Antibiotics
are also routinely used when an indwelling device, such as a lumbar or ventricular drain,
is used.
Often, medication can be used to decrease production of spinal fluid. Such medications
include diuretics (eg, furosemide, hydrochlorothiazide), carbonic anhydrase inhibitors
(eg, acetazolamide), and steroids. These medications are not used as a primary therapy
for a CSF leak, but they are valuable adjuncts to treatment. During the diagnosis and
evaluation phase, they may hamper the ability to locate the leak if the output is low.
Otogenic CSF leak secondary to recent surgery or trauma can often be treated
conservatively with a compressive dressing and bed rest with head elevation. Spinal
fluid leaks following acoustic neuroma surgery respond to this treatment 80% of the time.
This is also true of leaks associated with temporal bone fractures, which almost always
seal in 3-4 weeks with this conservative therapy.[7]
Postoperative leakage though an incision line can often be stopped with a single wellplaced mattress stitch.
Continuous lumbar spinal fluid drainage may serve as a useful adjunct to the
conservative treatment of a spinal fluid leak related to surgery or trauma. Continuous
drainage of spinal fluid serves to decrease the pressure head against the leak to allow
natural healing to occur. Do not use spinal drains when the site of leakage is not known
because they may hamper localization and allow air to flow into the cranial vault,
resulting in pneumocephalus. In cases of spontaneous leakage, the lumbar drain may
help facilitate healing after surgical repair.

Surgical Therapy

Primary treatment of a spontaneous otogenic spinal fluid leak is surgical repair. In

patients with postsurgical and traumatic leaks, surgery is reserved for those in whom
conservative therapy is unsuccessful. Surgical approach is dictated by the nature and
location of the defect.
Spontaneous leaks in children with otic capsule defects, such as Mondini deformity, can
often be repaired by a transcanal approach. Because rarely any hearing is present, a
stapedectomy is usually performed and the oval window obliterated with soft tissue. A
transcanal approach can also be used in some cases of CSF leakage due to a patent
Hyrtl fissure.
However, in most cases of spontaneous leakage, a transmastoid approach is preferred.
[8]
This is especially true if the exact site of the leakage is not known or suggested on
imaging studies. The exposure of a mastoidectomy usually allows excellent visualization
of the leakage site. The site can often be repaired with a small amount of fascia
supported by Gelfoam. If the leak is related to a small ( < 1 cm) defect in the bone of the
tegmen, the fascial repair can be supported with a tragal cartilage graft placed between
the intact bony edges and the dura.
Occasionally, a fat or muscle graft may be needed. In rare cases, the exact site of
leakage is not found, and diffuse leak is observed from multiple mastoid air-cell tracts. In
this situation, the mastoid may need to be obliterated with fat. Obliteration of the middle
ear and eustachian tube may also be required, especially if the leakage is not limited to
the mastoid.
A study by Kim et al supported the efficacy of transmastoid procedures for patients with
spontaneous temporal bone CSF leaks. In the study, 15 patients (16 ears) with temporal
bone related chronic otorrhea and conductive/mixed hearing loss underwent
transmastoid surgery. The procedures were multilayered, with autologous mastoid bone,
temporalis fascia, and tissue sealant typically employed. The operations were successful

in all but one ear, which was subsequently treated with a middle fossa craniotomy. None
of the patients suffered serious complications, and the air-bone gap, which had a mean
preoperative value of 19 dB, was 12 dB or less following surgery in the 14 patients for
whom postoperative audiograms were available.[9]
Leaks may occur in ears that have previously undergone canal wall down
mastoidectomy. Removal of the canal wall may also be dictated by the extent of disease
when the leak is associated with an active cholesteatoma. In these situations, the
external ear and mastoid epithelium must be completely removed and the ear canal
sewn over. Abdominal fat is then used for obliteration.
Leaks occurring from defects of the posterior cranial fossa anterior to the sigmoid sinus
present a special problem. This is the area of the basal cistern, where no arachnoid
mesh is present. Leakage from this area is explosive and profuse and is not well
controlled with fascia alone. A large fat graft obliterating the mastoid is usually required.
If a leak is due to a large (>1 cm) defect in the floor of the middle fossa, the problem is
best addressed with a combined middle fossa/transmastoid approach. A mastoidectomy
is performed first to identify the site of leakage. Do not attempt to reduce herniated brain
tissue. Such encephaloceles do not contain functioning brain tissue and should be
excised using bipolar cautery. Once the defect is identified, use the middle fossa
approach for repair. The middle fossa affords excellent visualization of the defect and an
opportunity to use the intact bony edges of the defect to hold any repair material in
place.
Recommended repair of such large defects is with a 3-layer technique. A layer of
calvarial bone is sandwiched between 2 layers of fascia. Use of a bone graft helps to
resist the pulsation of the CSF and prevent formation of a new meningocele. The
calvarial bone can be harvested by splitting the bone flap taken for the craniotomy.
[10]
Fibrin glue is a very useful adjunct to such a repair and helps seal the components in
place.
Fascia for grafting is usually harvested from the temporalis muscle. This muscle can also
serve as the source of a muscle graft. If a vascularized pedicled flap is desired, this can
be harvested from the superficial temporal fascia.
If adequate grafting material is not readily available in the operative wound, it may need
to be harvested from elsewhere in the body. One such site is the fascia lata found on the
lateral thigh. This site is capable of providing large amounts of fascia for dural repair.
Several local vascularized flaps are available to be used for reconstruction in the repair
of a CSF leak. These flaps include temporalis muscle, galeal flaps, and superficial
temporoparietal fascia. These flaps are especially useful following skull base surgery,
where large dural defects are often created. In rare cases of very large defects, a
vascularized free flap may be required.
Several forms of commercially available materials are now available to assist in the
repair of a CSF leak. One such material is made from bovine pericardium. This material
is very tough, but it is pliable and easy to work with. Hydroxyapatite cement may also be
used to aid in reconstruction of bone defects. Fibrin glue alone cannot be used as a
repair material, but it can be used to secure the repair components. Each of these
substances is biocompatible and becomes integrated with host tissue. Other synthetic
materials, such as silicone sheeting, Marlex mesh, titanium plates, and
methylmethacrylate, do not become integrated with host tissue and have a high rate of
infection or extrusion. The author prefers that even biocompatible materials not be used
if the supply of the patient's own tissue is adequate.
Continuous lumbar spinal fluid drainage is an important adjunct to surgical repair of
otogenic CSF leakage. The drain is usually placed at the beginning of treatment, but it

should be left clamped until the leakage site is identified. After the surgery, remove a
small amount of spinal fluid on an hourly basis to decrease the pressure head against
the repair. Care must be taken not to remove too much spinal fluid because this may
draw air into the cranium through the repair, resulting in pneumocephalus. The patient
must remain on bed rest as long as the drain is in place.

Postoperative Details

In most cases, the postoperative patient should be observed in an ICU setting. The
intensive care setting ensures that neurologic changes heralding an arising complication
will be rapidly noticed and addressed. Perform hourly neurologic checks for the first 24
hours after surgery and then every 2 hours until the patient is well enough to leave the
unit.
An important aspect of ICU care is monitoring of the lumbar drain. Drainage should be
accomplished by draining a specific amount every hour, usually about 10 mL.
Occasionally, this causes severe headache, in which case a smaller amount can be
removed or the fluid can be removed less frequently (eg, 5 mL every half hour). Some
have advocated leaving the drain open at a level just below the level of the leak, allowing
the spinal fluid to drain at its own pace while maintaining a low pressure against the
repair. This technique has led to some reports of pneumocephalus and is generally not
recommended. Others have suggested attaching the drain to an IV pump so that a
steady even flow can be maintained. This, too, may be risky because it may cause too
much drainage and result in pneumocephalus.
While the drain is in place, patients should remain on bed rest. Use position changes
and compression boots to prevent bedsores and deep venous thrombosis.
Check spinal fluid drainage every 2 days with a Gram stain and a culture.
The lumbar drain is usually left in place for 2-3 days postoperatively. If no sign of
leakage is present, it is clamped and the patient is observed for an additional 24 hours. If
no further leakage is observed, the drain is then removed.
In many cases of surgical repair of a CSF leak, ICU monitoring may not even be
necessary. These include cases treated by a transcanal approach that do not require
lumbar drainage.

Complications

The risks faced by patients undergoing surgery to repair a CSF leak are far less than
those they face if the leak remains unaddressed. Although some leaks may continue in
some patients for prolonged periods, any leak is likely to eventually result in meningitis if
monitored for long enough. The surgical risks associated with the surgery are no greater
than those for any other similar neurologic surgery.
Neurologic surgery carries risks for intracranial bleeding, cerebral edema,
hydrocephalus, stroke, and of course, meningitis. Any change in mental status in the
postoperative period must be rapidly evaluated and addressed. An immediate CT scan
of the brain performed without contrast usually demonstrates any bleeding, edema,
stroke, or hydrocephalus. If the findings of this study are normal, it should be followed by
a lumbar puncture. Lumbar puncture should not be performed before these entities are
ruled out because removal of spinal fluid may result in brain herniation. However, in most
situations, a lumbar drain is already in place. When mental status changes occur, the
drain should be clamped until the CT scan findings are shown to be normal. A sample of
the fluid in the reservoir can be sent off at any time.