Meniere disease (endolymphatic hydrops) is a chronic vestibulocochlear disorder characterized by abnormal expansion of the endolymphatic space in the inner ear. Endolymph (inner ear fluid) volume increases and endolymphatic membranes (Reissner membranes) are distended — this expansion manifests as hearing loss in the cochlea, vertigo in the vestibulum, and tinnitus in both structures. Classic clinical tetrad: episodic vertigo (minutes to hours), fluctuating sensorineural hearing loss (initially low frequencies), tinnitus, and aural fullness. Diagnosis is based on clinical criteria (Barany Society 2015). Imaging with MR hydrops protocol directly demonstrates endolymphatic expansion: after intratympanic gadolinium injection, 24 hours wait → on 3D-FLAIR sequence the perilymphatic space takes up gadolinium contrast (bright) while endolymphatic space does not (dark) → enlarged endolymphatic space filling >33-50% of the vestibule (Nakashima Grade 1-2) is diagnostic. CT is usually normal and mainly used to exclude structural inner ear anomalies such as large vestibular aqueduct syndrome.
Age Range
20-70
Peak Age
45
Gender
Equal
Prevalence
Uncommon
The pathophysiology of Meniere disease is built upon endolymphatic hydrops — volume increase of the endolymphatic space. Endolymph is a potassium-rich, sodium-poor fluid found within the membranous labyrinth of the inner ear, present in the cochlea (scala media), vestibule (utricle, saccule), and semicircular canals. In normal homeostasis, endolymph production (stria vascularis) and absorption (endolymphatic sac) are balanced. In Meniere disease, this balance is disrupted — endolymphatic sac absorption capacity decreases or production increases → endolymph volume progressively increases → Reissner membrane (thin membrane separating endolymphatic and perilymphatic spaces) balloons toward the endolymphatic space. In the cochlea: scala media expansion → disturbance of basilar membrane mechanics → low-frequency hearing loss (initially low frequencies are affected as basilar membrane is more elastic at cochlear apex where hydrops starts). In the vestibule: saccular and utricular expansion → mechanical deformation of otolith organs. Acute vertigo attacks are explained by membrane rupture theory: micro-ruptures form in Reissner membrane → potassium-rich endolymph leaks into perilymphatic space → potassium toxicity at vestibulocochlear nerve endings → acute vestibular crisis (vertigo). After rupture heals, symptoms resolve. In MR hydrops protocol, intratympanic gadolinium distributes into perilymphatic space (gadolinium remains in perilymph, does not cross to endolymphatic space — blood-labyrinth barrier opens only to perilymphatic side) → on 3D-FLAIR, perilymphatic space appears bright, endolymphatic space dark → enlarged endolymphatic space identified as dark filling defect.
Endolymphatic space expansion on MR hydrops protocol (delayed 3D-FLAIR after intratympanic or intravenous gadolinium) — according to Nakashima classification: Grade 0 = normal (endolymphatic space <33% of vestibule), Grade 1 = mild hydrops (33-50%), Grade 2 = significant hydrops (>50%). Grade 2 strongly correlates with clinical Meniere diagnosis. Vestibular and cochlear hydrops are graded separately. This finding is the pathognomonic imaging sign confirming Meniere disease and supports definite diagnosis together with the clinical tetrad.
Expansion of vestibular endolymphatic space on 24-hour delayed 3D-FLAIR images after intratympanic gadolinium — perilymphatic space appears bright with gadolinium while endolymphatic space remains dark and occupies a large portion of the vestibule. Nakashima classification: Grade 0 = normal (endolymphatic space <33% of vestibule), Grade 1 = mild hydrops (33-50%), Grade 2 = significant hydrops (>50%). Grade 2 correlates with definite Meniere diagnosis. Bilateral involvement seen in 30-40% of cases.
Report Sentence
Vestibular endolymphatic space expansion on 3D-FLAIR after intratympanic gadolinium on __ side, consistent with endolymphatic hydrops Nakashima Grade __.
Scala media (endolymphatic space) expansion in the cochlea — in normal cochlea, scala media is a narrow band; in hydrops, scala media expands and balloons toward scala vestibuli. This finding is more difficult to evaluate than vestibular hydrops because cochlear structures are very small. Cochlear hydrops is usually accompanied by vestibular hydrops but isolated cochlear hydrops may also be seen (cochlear Meniere).
Report Sentence
Scala media expansion in the cochlea, consistent with cochlear endolymphatic hydrops.
Temporal bone CT is usually completely normal in Meniere disease — bony labyrinth, vestibular aqueduct, cochlea, semicircular canals, and internal acoustic canal are of normal size and morphology. Vestibular aqueduct midpoint width <1 mm (normal) — this finding excludes large vestibular aqueduct syndrome. The role of CT is primarily to exclude structural anomalies (LVAS, cochlear malformation, superior semicircular canal dehiscence).
Report Sentence
Inner ear structures, vestibular aqueduct, and internal acoustic canal are normal on temporal bone CT without structural anomaly; MR hydrops protocol recommended for Meniere disease.
Perilymphatic space (scala vestibuli, scala tympani, vestibular perilymphatic compartment) appears hyperintense with gadolinium on 3D-FLAIR sequence 24 hours after intratympanic gadolinium injection — this contrast enables delineation of the endolymphatic space boundaries (gadolinium-free, remaining dark). Homogeneous gadolinium distribution confirms successful intratympanic injection and adequate diffusion time. Alternatively, intravenous gadolinium (4 hours later) can be used as a less invasive approach but contrast is lower.
Report Sentence
Homogeneous contrast uptake in perilymphatic space after intratympanic gadolinium, enabling assessment of endolymphatic space boundaries.
Detailed anatomic evaluation of inner ear fluid structures (cochlea, vestibule, semicircular canals, internal acoustic canal) on high-resolution 3D CISS (Constructive Interference in Steady State) or 3D FIESTA sequence — assessment of labyrinthine morphology, cochlear turns, semicircular canal integrity, and 7th-8th cranial nerve courses. In Meniere disease, labyrinthine morphology is usually normal; this sequence is primarily used to exclude structural anomalies (IP-II, cochlear aplasia, superior canal dehiscence) and for treatment planning (intratympanic injection, cochlear implant).
Report Sentence
Inner ear structures (cochlea, vestibule, semicircular canals) show normal morphology on 3D CISS/FIESTA sequence without structural anomaly.
Criteria
Vestibular and cochlear symptoms together: episodic vertigo + fluctuating SNHL + tinnitus. Vestibular and cochlear hydrops on MRI.
Distinct Features
Most common type. Unilateral onset 60-70%, bilateral progression 30-40% over years. Hearing loss initially low-frequency predominant.
Criteria
Fluctuating SNHL + tinnitus + aural fullness but NO vertigo. Isolated cochlear hydrops on MRI.
Distinct Features
May progress to classic Meniere over time (addition of vertigo). Treatment similar.
Criteria
Episodic vertigo but NO hearing loss or minimal. Isolated vestibular hydrops on MRI.
Distinct Features
Differential diagnosis is broad (BPPV, vestibular migraine). MR hydrops protocol critical in diagnosis. Cochlear involvement may be added over time.
Distinguishing Feature
In LVAS, CT shows vestibular aqueduct >1.5 mm width (pathognomonic); in Meniere, vestibular aqueduct is normal size (<1 mm). LVAS is congenital, Meniere is acquired. LVAS is pediatric, Meniere usually presents in adulthood.
Distinguishing Feature
Temporal bone fracture appears in acute trauma setting with fracture line and hemotympanum; Meniere has completely normal temporal bone and chronic clinical course.
Distinguishing Feature
Mastoiditis shows mastoid cell opacification and septal erosion; mastoid is completely normal in Meniere. Mastoiditis is acute infectious, Meniere is chronic vestibulocochlear disorder.
Urgency
routineManagement
medicalBiopsy
Not NeededFollow-up
6-monthMeniere disease is a chronic progressive condition significantly affecting quality of life. Treatment follows a stepwise approach: 1st line — lifestyle modifications (salt restriction <1500 mg/day, caffeine/alcohol restriction) + diuretics (hydrochlorothiazide, triamterene); 2nd line — intratympanic steroid injection (dexamethasone, for refractory cases); 3rd line — intratympanic gentamicin (chemical vestibular ablation, hearing loss risk); 4th line — endolymphatic sac decompression (surgical) or vestibular neurectomy (last resort). MR hydrops protocol is used for diagnosis confirmation, bilateral involvement detection, treatment response evaluation, and cochlear implant candidacy. Hearing loss follows progressive course with advanced-stage pancochlear permanent SNHL. Cochlear implantation is an effective rehabilitation option in advanced bilateral Meniere patients.
Meniere disease is a chronic condition significantly affecting quality of life. Treatment follows a stepwise approach: salt restriction + diuretics (1st line), intratympanic steroids (2nd line), intratympanic gentamicin (3rd line — chemical ablation), endolymphatic sac decompression or vestibular neurectomy (4th line). MR hydrops protocol guides diagnosis confirmation and treatment response evaluation.