ORIGINAL RESEARCH
Adult spontaneous CSF otorrhea: Correlation
with radiographic empty sella
Christopher N. Prichard, MD, Brandon Isaacson, MD, John S. Oghalai, MD,
Newton J. Coker, MD, and Jeffrey T. Vrabec, MD, Houston, Texas
OBJECTIVE: To determine the prevalence of radiographic empty
sella in patients with spontaneous cerebrospinal fluid (CSF) otorrhea.
STUDY DESIGN AND SETTING: Retrospective case series
of adult patients with CSF otorhinorrhea at an academic tertiary
medical center. Patients with history of skull base surgery, trauma,
tumor, or chronic ear disease were excluded. Available imaging
studies were reviewed with attention to the sella turcica.
RESULTS: Eight patients were diagnosed with spontaneous
CSF otorrhea. Five of seven patients with adequate imaging stud-
ies (71%) had a radiographic empty sella. Seven of eight patients
were clinically obese, with a body mass index BMI 30 kg/m
2
.
CONCLUSIONS: Empty sella is a common radiologic finding
in patients with spontaneous CSF otorrhea. This supports the
theory that increased intracranial pressure contributes to develop-
ment of spontaneous CSF otorrhea.
SIGNIFICANCE: Radiographic empty sella predicts elevated
intracranial pressure, which may require further evaluation and
treatment in patients with spontaneous CSF otorrhea.
EBM rating: C-4
© 2006 American Academy of Otolaryngology–Head and Neck
Surgery Foundation. All rights reserved.
C
erebrospinal fluid (CSF) otorrhea represents a chal-
lenging, though relatively rare, clinical dilemma. Most
frequently CSF otorrhea results from fracture of the tempo-
ral bone.
1-3
It also results from post-operative defects, tumor
and chronic infection. CSF leaks in the absence of any of
these risk factors are deemed “spontaneous CSF otorrhea.”
4
Spontaneous CSF otorrhea presents in 2 distinct forms—
congenital and adult spontaneous CSF otorrhea. Congenital
CSF leaks typically present in children 5 years of age, and
are invariably associated with meningitis, most commonly
from Streptococcus pneumoniae.
2
Inner ear anomalies are
common in these children, creating an abnormal communi-
cation of the subarachnoid space to the perilymph compart-
ment.
5
This occurs via a defect in the cribriform area in the
fundus of the internal auditory canal. The CSF then reaches
the middle ear space via defects in the region of the oval
window. Other rare sites of congenital CSF otorrhea include
an enlarged Hyrtl’s fissure or via the fallopian canal.
Adult spontaneous CSF otorrhea patients typically present
with unilateral persistent serous effusions causing a sensa-
tion of aural fullness or conductive hearing loss.
6
Meningitis
is uncommon in these patients. Diagnosis is often made
when a myringotomy is carried out for a persistent effusion
and unrelenting clear otorrhea develops.
Two major hypotheses have been proposed in the etiol-
ogy of adult spontaneous CSF otorrhea. The “congenital ori-
gin theory” postulates that these adults have tiny persistent
congenital tegmen defects due to abnormal embryologic
development, which have remained quiescent throughout
life. Presumably some stimulus later in life allows connec-
tion between the CSF space and middle ear/mastoid.
5
Al-
ternatively, Gacek et al
7
proposes that the bony floor of the
skull base contacts embryologically abnormal arachnoid
granulations that do not contact a venous termination. Sub-
sequent erosion through anatomically thin portions of the
tegmen ultimately results in CSF leaks.
6
Certain risk factors have been implicated in the devel-
opment of spontaneous CSF otorrhea, including age, low-
grade inflammation, and increased intracranial pressure.
5
Autopsy studies demonstrate a relatively high prevalence of
tegmen defects (15% to 34%).
6,8
Given these high percent-
ages of anatomic defects in contrast to the relative rarity of
adult spontaneous CSF otorrhea, one could conclude that
From the Bobby R. Alford Department of Otolaryngology–Head and
Neck Surgery, Baylor College of Medicine, Houston, Texas.
Reprint requests: Jeffrey T. Vrabec, MD, Baylor College of Medicine,
Department of Otolaryngology–Head and Neck Surgery, 6550 Fannin,
Suite 1727, Houston, TX 77030.
E-mail address: jvrabec@bcm.tmc.edu.
Otolaryngology–Head and Neck Surgery (2006) 134, 767-771
0194-5998/$32.00 © 2006 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved.
doi:10.1016/j.otohns.2006.01.002