Spontaneous Intracranial Hypotension Syndrome

Spontaneous Intracranial Hypotension Syndrome Accompanied by Bilateral Hearing Loss and Venous Engorgement in the Internal Acoustic Canal and Positional Change of Audiography
Isildak, Huseyin MD; Albayram, Sait MD; Isildak, Hacer MD

Hearing loss, tinnitus, and vertigo are very common complaints in otolaryngology practice.

Here, we describe spontaneous intracranial hypotension (SIH) as a curable reason of hearing loss, tinnitus, and vertigo.

A 29-year-old woman presented to the emergency room with nausea, dizziness, vertigo, instability, hearing loss, tinnitus, and neck and back pain.

Cranial computed tomography, magnetic resonance imaging (MRI), and lumbar puncture were performed.

The patient stated that the hearing loss and tinnitus became worse after effort or standing for prolonged times.

Therefore, we performed audiogram in sitting and standing positions.

The tinnitus severity index was used to evaluate tinnitus.

Lumbar puncture revealed no cerebrospinal fluid, and cerebrospinal fluid could be obtained by aspiration.

Cranial MRI showed dural thickness and venous engorgement in the internal acoustic canals bilaterally.

Audiography showed worse hearing capacity in standing position than in sitting position and revealed especially low-frequency hearing loss bilaterally.

The patient's tinnitus severity index was 48 of 60.

The patient was diagnosed as having SIH and treated with autologous blood punch.

Cranial MRI and audiogram were normal after the treatment.

The patient had no tinnitus after the treatment.

Spontaneous intracranial hypotension, which may cause Ménière syndrome-like symptoms, is a curable reason of hearing loss, tinnitus, and vertigo.

In addition, the fluctuation of the hearing loss with positional changes supports the use of positional audiometry when evaluating hearing loss-related SIH.

Venous engorgement in the internal acoustic canal may be related to the symptoms.

Fuente: Journal of Craniofacial Surgery:January 2010 - Volume 21 - Issue 1 - pp 165-167,doi: 10.1097/SCS.0b013e3181c50e11,Clinical Studies
foto: img.medscape.com/.../336139-338989-9413.jpg

Spontaneous intracranial hypotension
contributed by Dr Frank Gaillard on May 2, 2008

Spontaneous intracranial hypotension most commonly results from CSF leaks in the cervical and thoracic spine [4], and leads to alterations in the equilibrium between the volumes of intracranial blood, CSF, and brain tissue (Monro–Kellie hypothesis).

A decrease in CSF volume leads to compensatory dilatation of the vascular spaces, mostly venous side due to its higher compliance.

It is identical to CFS leaks that occur following lumbar puncture, surgery or trauma.
Clinical presentation

BIH presents as positional headache, relieved by recumbent position, and is confirmed by assessing opening pressure on LP (less than 7cm CSF) [2].

Not that when this is done fluoroscopically it should be done in lateral position to allow for accurate measurement of pressure.

Occasionally presentation is more sinister with even decreased level of consciousness and coma reported [3].
Radiographic features
CT

* subural collection
* cerebellar tonsils in the foramen magnum (acquired Chiari I malformation)

CT Myelogram

Myelography is useful in identifying the location of CSF leak. Ideally the lumbar puncture is performed on the CT table. 10 mls of myelofraphic contrast is slowly introduced into the thecal space.

Following removal of the needle the patient is asked to roll once and is immediately scanned. It is important to scan early as contrast will track a long way from the leak in a relatively short time.
MRI

* diffuse brain swelling [3]
* sagging brainstem
* droopy penis sign: perhaps only an Australianism to denote the shape of the downward drooping splenium of the corpus callosum
* subdural effusions
* rounding of the cross-section of the dural venous sinuses
* contrast-enhanced MR imaging demonstrates
o venous sinus engorgement
o pachymeningeal enhancement (infra- and supra-tentorial)
o enlargement of the pituitary gland

Treatment

Epidural blood patch ideally at the sight of CSF leak is the treatment of choice.
Fuente: Radiopaedia.org