Ménière disease
Ménière disease (or idiopathic endolymphatic hydrops) is an inner ear disorder and as such can affect balance and hearing.
Clinical presentation
One or both ears can be affected. The chief symptoms are:
- vertigo (often attacks which can be incapacitating)
- hearing loss
- tinnitus
- a sensation of fullness in the ears
- Tullio phenomenon occasionally experienced
The American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium set criteria for diagnosing Ménière disease, most recently revised in 1995 :
- certain: a definite disease with histopathological confirmation
- definite: requires two or more definitive episodes of vertigo with a hearing loss plus tinnitus and/or aural fullness
- probable: only one definitive episode of vertigo and the other symptoms and signs
- possible: definitive vertigo with no associated hearing loss
Pathology
Although considered to be idiopathic, there is an association with inner ear effusions and endolymphatic hydrops.
Radiographic features
The role of imaging is controversial , but improved spatial resolution in CT and MRI is likely to show where the endolymphatic flow is impaired.
CT
Reduced visualization of the saccular duct and endolymphatic sinus are featured . At high-resolution temporal bone CT, the vestibular aqueduct is either small or not visible at all, in contrast to the congenital large vestibular aqueduct syndrome.
MRI
Historically, MRI has been used to exclude alternative diagnoses such as vestibular schwannoma or other cerebellopontine angle lesions, and superior semicircular canal dehiscence.
During the past decade, the morphologic substrate of Ménière disease, i.e. endolymphatic hydrops, has become visible using non-contrast in addition to contrast-enhanced endolymphatic hydrops MRI techniques .
Non-contrast MRI technique uses a heavily T2-weighted sequence.
Contrast-enhanced techniques typically use a 3D fluid-attenuated inversion recovery (FLAIR) sequence or a 3D inversion recovery (IR) sequence either after intratympanic gadolinium administration either 4 hours after intravenous gadolinium administration . The latter endolymphatic hydrops MRI technique is the most frequently used. It has been shown to detect definite Menière’s disease with high sensitivity and specificity .
History and etymology
It is named after the French physician Prosper Ménière (1799-1862) who first recognized vertigo as an inner ear disorder.
Siehe auch:
