PowerPoint Template2010.09.24 Presented by R3
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– Presence of CSF within the confines of the temporal bone
– Defect in the dura abnormal communication between subarachnoid space and air- containing space of temporal bone
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Deficiency in the modiolus
• Transient anatomic landmark in the developing fetal temporal bone
• Communication between round window niche (lateral) and pars nervosa of the jugular fossa( medial)
• Tympanomeningeal fissure or hiatus • Closed by progression of ossification
in the 24th week of gestation
External auditory canal
Introduction
• The causes of CSF otorrhea – Trauma (temporal bone fracture) – Iatrogenic( skull base surgery) – Neoplastic – Infectious – Congenital
• Spontaneous CSF otorrhea – Not related to the above-mentioned
causes
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Congenital causes of spontaneous CSF otorrhea
• Mondini dysplasia • Dehiscent stapes footplate • Defect in the lateral end of the IAC • Enlarged cochlear aqueduct • Defect in Hyrtl’s fissure • Dehiscent fallopian canal or oval
window
Two distinct populations
• Young children: – Meningitis after acute otitis media. – Congenital anomalies of the temporal
bone • Obese middle-aged or elderly women:
– Decreased hearing or aural fullness with middle ear effusions
– Persistent serous or clear discharge after myringotomy
Meningoencephalocele with spontaneous CSF otorrhea
Laboratory tests
–Only in CSF, perilymph and vitreous humor
–Greater sensitivity and specificity than glucose test
Imaging study • HRCT of temporal bone
– Most common and helpful – Determine the location of leaks – CT cisternography (intrathecal
injection) • MRI
– Brain tissues herniation detection – Empty or partially empty sella
Pathophysiology of spontaneous CSF otorrhea
• Congenital defect theory (Rao A et al ,2005) – Defects of the middle fossa tegmen
enlarged( constant CSF pressure) – Dural herniation – Bony and dural thinning
Pathophysiology of spontaneous CSF otorrhea
• Arachnoid granulation theory( Gacek, et al 1999) – Abnormally located arachnoid
granulations – Autopsy: middle fossa tegmen(21%)
Posterior fossa(9%) – Minor CSF reservoirs. Abnormal
locations decreased return to the venous systems
– Thinning and erosion of bone
Arachnoid granulations
Additional factors involved • A majority of patients
– Obese, middle-aged females. ( Pappas et al, 1992& LeVay et al, 2008)
– A variant of idiopathic intracranial hypertension (IIH) ( Schlosser et al,2006)
– Empty or partially empty sella turcica: 63% to 100 % of cases (5 to 6 % in normal population)
( Friedman et al, 2002 & John et al, 2010)
Demographic data
• Mean ages: 60 years • Mean BMI: 36.3 kg/m2 (Average BMI
at US: 28 kg/m2 ) • Female predominance (F:M=2:1) • The same findings noted at CSF
rhinorrhea (Schlosser et al, 2006) • The association with idiopathic
intracranial hypertension (IIH)( Schlosser et al, 2008)
BMI v.s. CSF otorrhea
Pathophysiologic of IIH
• Still unknown and multifactorial in nature( Goddard et al, 2010) – Central obesity – Narrowed cerebral venous sinuses – Hormonal dysfunction: association
with polycystic ovarian disease( Connor et al, 2008)
• In spontaneous CSF otorrhea: – ophthalmologic and neurologic
evaluations
Progression of morbid obesity to encephalocele (Scurry et al, 2007)
Morbid obesity
Enlargement
Encephalocele
Partially or empty sella turcica v.s. obesity (Goddard et al, 2010)
Conservative treatment
• Indicated for traumatic CSF otorrhea/otorhinorrhea fracture lines are small in caliber and tortuous
• Successful rates: 82.8% in traumatic CSF leak (Savva, 2003)
• Measures: – Restricted nose blowing – Avoidance of straining – Bed rest and head elevation of 30 degrees – Use of antiemetics, antitussives and stool
softeners – Diuretics and fluid restriction – Lumbar drain
Surgical intervention
• Various approaches – Transmastoid & translabyrinthine – Middle fossa craniotomy – Mini-craniotomy – Keyhole craniotomy
• Materials used to correct bony defect: – Bone, cartilage, fascia, abdominal fat,
silastic and various combinations of autologous tissues.
Surgical intervention
patients( Rao AK, et al. 2005) – Extracranial visualization of middle
and posterior fossa without damage of intracranial tissues.
• Multilayered Closure: – highest rate of definitive closure – lowest rate of recurrence
Multilayered closure for tegmen foundation
Surgical intervention • Middle fossa craniotomy
– Tegmen defect larger than 2 cm – Multiple defects in the tegmen with
extending toward petrous apex • Translabyrinthine approach
– For patients with no hearing – Remove all middle ear structure – Occult Eustachian tube with bone wax,
muscle and fascia – Obliteration of middle ear with muscle or
fat and close EAC
• The diagnosis of spontaneous CSF otorrhea: – persistent, unilateral serous otitis media
w/ previous normal otologic history. • High-resolution CT
– Confirmation of a tegmen defect in 80% of cases
– Assist in surgical planning • The transmastoid approach effective if
– bony dehiscence and/or meningocele can be widely visualized
Conclusions
• Patients with spontaneous cerebrospinal fluid otorrhea – middle-aged and obese – females being affected nearly twice as often
as males. • Empty or partially empty sella
– 80% of patients with spontaneous cerebrospinal fluid otorrhea on MRI
Conclusions
otorrhea • Neurology and ophthalmology
of idiopathic intracranial hypertension
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