What it is
The brain and spinal cord are surrounded by cerebrospinal fluid, contained by a membrane called the dura. A cerebrospinal fluid leak occurs when both the dura and the bone that supports it are breached, allowing fluid to escape into an adjacent air-filled space.
When that breach is in the temporal bone — the bone housing the ear — fluid drains into the middle ear and mastoid. From there it either escapes through a perforated eardrum as clear drainage from the ear, or, with an intact eardrum, passes down the Eustachian tube and presents as clear watery drainage from the nose. This second pattern is a common source of diagnostic confusion, because the fluid appears at the nose while the actual defect is in the ear.
Sometimes brain covering, or brain tissue itself, herniates through the bony defect. This is called an encephalocele, and it is often found alongside the leak.
Causes
Spontaneous leaks occur without any injury or prior operation, and have been increasingly recognized over the past decade or two. They are strongly associated with chronically elevated intracranial pressure, which thins the bone of the skull base over years until a defect develops. Contributing factors include elevated body mass index, obstructive sleep apnea, and radiographic findings such as an empty sella. These leaks are frequently found at thin areas of the tegmen, the bony roof of the middle ear and mastoid.
Acquired leaks follow a specific event:
- Surgery on the ear or lateral skull base
- Head trauma, particularly temporal bone fractures
- Chronic ear disease, especially cholesteatoma that has eroded through the roof of the middle ear
- Tumors that erode the skull base
Symptoms
- Clear, watery drainage from one ear, or from one side of the nose, sometimes worse when bending forward or straining
- Aural fullness or a sensation of fluid in the ear
- Hearing loss, typically conductive, from fluid filling the middle ear
- Headache, which may be positional
- Pulsatile tinnitus, particularly when elevated intracranial pressure is involved
- Meningitis — fever, severe headache, neck stiffness, and light sensitivity — which is sometimes the first presentation and requires immediate care
A patient with unexplained recurrent meningitis should be evaluated for an occult skull base defect.
Diagnosis
Beta-2 transferrin testing on a sample of the fluid is the standard confirmatory test. The protein is essentially unique to cerebrospinal fluid, so a positive result establishes the diagnosis.
High-resolution CT of the temporal bone identifies the bony defect and its precise location, which is essential for surgical planning. MRI demonstrates whether soft tissue — meninges or brain — has herniated through the defect, and distinguishes an encephalocele from simple fluid.
Because spontaneous leaks are associated with raised intracranial pressure, evaluation often extends to that question, and may include assessment for sleep apnea and, in some cases, measurement of the pressure itself.
Treatment
Surgical repair is the definitive treatment for spontaneous leaks and for acquired leaks that do not close with conservative measures. The defect is sealed using a multilayer reconstruction, and any herniated tissue is addressed. The approach depends on the location and size of the defect:
- Transmastoid, working through the mastoid bone behind the ear
- Middle cranial fossa, approaching from above the ear to expose the roof of the temporal bone directly
- A combined approach, for large or multiple defects
Multiple defects are common in spontaneous cases, since the same process that thinned the bone in one place has usually thinned it elsewhere. Surgical planning accounts for this.
Managing the underlying pressure matters for durability. When elevated intracranial pressure is contributing, addressing it — through weight management, treatment of sleep apnea, medication, or in some cases a shunt — reduces the likelihood of a repair failing or a new leak developing at another thin spot.