Value of Endolymphatic Hydrops and Perilymph Signal Intensity in Suspected Ménière Disease

J.M. van Steekelenburg; A. van Weijnen; L.M.H. de Pont; O.D. Vijlbrief; C.C. Bommeljé; J.P. Koopman; B.M. Verbist; H.M. Blom; S. Hammer

doi:10.3174/ajnr.A6410

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Fig 1.
Axial delayed gadolinium-enhanced 3D-FLAIR MR imaging centered at the pars inferior of the vestibule, with graphic correlations. A, Normal labyrinth: saccule (dashed arrow), utricle (dotted arrow), scala media (short arrow), scala vestibuli (small arrowhead), and scala tympani (large arrowhead). B, Mild vestibular EH: the saccule (dashed arrow) is equal in size or larger than the utricle, but not confluent. C, Moderate vestibular EH with confluence of the saccule and utricle that encompasses >50% of the vestibule (dashed arrow). A rim of surrounding perilymph remains visible (long arrow). Moderate cochlear EH with dilation of the scala media (short arrow), resulting in partial obliteration of the scala vestibuli. D, Severe vestibular EH with total effacement of the perilymphatic space in the vestibule (dashed arrow). Severe cochlear EH with complete obliteration of the scala vestibuli (short arrow).
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Fig 2.
Axial 3D-FLAIR image 4 hours after intravenous gadolinium at the midcochlear level in a patient with unilateral left-sided sudden deafness, showing diffuse perilymphatic enhancement in the cochlea and vestibule.
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Fig 3.
Axial 3D-FLAIR image 4 hours after intravenous gadolinium at the level of the basal cochlear turn of a patient with unilateral right-sided definite MD and a visually increased perilymphatic enhancement. The basal cochlear turn (oval) and the left middle cerebellar peduncle (circle) indicate the region of interest to calculate the SIR.

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Table 1:

Clinical diagnosis of ears with other VAIEP

Clinical Diagnosis	Ears (n = 128) (%)
Autoimmune inner ear disease	2 (1.6)
Benign paroxysmal positional vertigo	6 (4.7)
Cochlear migraine	1 (0.8)
Fluctuating low-to-medium frequency sensorineural hearing loss eci	3 (2.3)
Focal monostotic fibrous dysplasia	1 (0.8)
Hyperventilation	14 (10.9)
Labyrinthitis	1 (0.8)
Migraine	3 (2.3)
Presbycusis	10 (7.8)
Schwannoma	1 (0.8)
Sudden deafness	10 (7.8)
Tinnitus eci	1 (0.8)
Vertigo eci	43 (33.6)
Vestibular migraine	24 (18.8)
Vestibular neuritis	8 (6.3)

Note:—eci indicates e causa ignota (Latin for no cause found).

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Table 2:

Interobserver agreement

	κ (95% CI)
Clinicians	0.81 (0.73–0.88)
Neuroradiologists
EH (cochlear and/or vestibular)	0.92 (0.88–0.97)
Cochlear hydrops	0.93 (0.89–0.98)
Vestibular hydrops	0.92 (0.87–0.97)
PE visual	0.90 (0.85–0.96)

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Table 3:

Presence of EH and PE^{^a}

	Definite MD (n = 149) (%)	Probable MD (n = 14) (%)	Asymptomatic (n = 149) (%)	Other VAIEP (n = 128) (%)
EH	137 (91.9)^{^b}	9 (64.3)^{^b}	7 (4.7) P = .45	9 (7.0)
Vestibular EH	133 (89.3)^{^b}	9 (64.3)^{^b}	7 (4.7) NS	8 (6.3)
Cochlear EH	126 (84.6)^{^b}	8 (57.1)^{^b}	3 (2.0) NS	4 (3.1)
Isolated vestibular EH	11 (7.4) NS	1 (7.1) (P = .47)	4 (2.7) NS	5 (3.9)
Isolated cochlear EH	4 (2.7) NS	0 (0.0) NS	0 (0.0) NS	1 (0.8)
PE	123 (82.6)^{^b}	6 (42.9) (P = .003)	5 (3.4) P = .045	12 (9.4)
PE and hydrops	122 (81.9)^{^b}	6 (42.9)^{^b}	2 (1.3) P = .42	4 (3.1)
PE and vestibular EH	119 (79.9)^{^b}	6 (42.9)^{^b}	2 (1.3) P = .42	4 (3.1)
PE and cochlear EH	118 (79.2)^{^b}	6 (42.9)^{^b}	2 (1.3) NS	3 (2.3)

Note:—NS indicates not significant.
↵a PE is scored visually.
↵b P < .001 (Fisher Exact) compared with other VAIEP ears.

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Table 4:
Sensitivity, specificity, PPV, and NPV in definite MD ears
Sensitivity Specificity PPV NPV
EH 0.92 0.93 0.94 0.91
PE 0.83 0.91 0.91 0.82
EH + PE visual 0.82 0.97 0.97 0.82
EH + PE visual or measured 0.86 0.97 0.97 0.86
Note:—PPV indicates positive predictive value; NPV, negative predictive value.
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Table 5:
Generalized estimating equation for the mean SIR of PE with other VAIEP as a reference category
B SE P Value
Intercept 1.094 0.0282 P < .001
Definite MD 0.550 0.0590 P < .001
Probable MD 0.221 0.1603 P = .167
Asymptomatic 0.218 0.0844 P = .010
Note:—SE indicates standard error; B, beta coëfficiënt.