Otology & Neurotology

26:547 Ó 2005, Otology & Neurotology, Inc.

Imaging Case of the Month

Michel’s Aplasia

Menachem Gross, Ron Eliashar, and Josef EIidan

Department of Otolaryngology—Head and Neck Surgery, Hadassah University Hospital, Jerusalem, Israel.

High-resolution computed tomography (CT) is the

imaging method of choice for evaluation of the temporal
bone in congenital deafness (1). The density contrast
between the otic capsule and the surrounding air cells
provides optimal computed tomographic characteristics
for diagnosis. However, in certain congenital malfor-
mations (such as inner ear or cochleovestibular nerve
aplasia) magnetic resonance imaging (MRI) provides
important additional information (1).
In the present case, axial CT scan of the temporal
bones revealed total absence of inner ear structures
(Fig. 1). The internal auditory canal, the bony separation
between the internal carotid artery and the jugular vein,
could not been identified. MRI examination confirmed
the diagnosis of MichelÕs aplasia.
MichelÕs (labyrinthine) aplasia constitutes only 1%
of cochlear bony malformations (2). It is defined by an
absence of differentiated inner ear structures on both
sides. Initially described in 1863, this rare congenital
malformation of both osseous and membranous laby-
rinth occurs as a result of an arrest of otic capsule
development in the fetus before the third week of
gestation (1).
The incidence of complete labyrinthine aplasia is FIG. 1. (A) High-resolution axial CT scan of the temporal bone in
the plane of the ‘‘otic capsule,’’ demonstrating total absence of the
overestimated in the radiological literature because it inner ear structures with labyrinthine ossification (arrow). (B)
is often confused with labyrinthine ossification, which is Coronal reconstruction of the axial CT scan of the temporal bone,
an acquired condition with a sizable, dense otic capsule. demonstrating a normal middle ear. The medial wall of the middle
High-resolution MRI examination can demonstrate the ear is in close contact with the infratentorial nervous structures.
presence of a membranous labyrinth, thus distinguishing
between the two conditions. In this regard, routine T1-
and T2-weighted spin-echo images may not be sufficient
for the diagnosis of inner ear congenital malformation. confirmed by sagittal or reconstruction images. High-
Only thin, gradient-echo MRI scans allow detailed study spatial-resolution MRI examination is also the method
of the membranous labyrinth with visualization of the of choice to demonstrate fibrous obliteration of the scala
intralabyrinthine fluid spaces (1). Another advantage of tympani and scala vestibuli in a patient with congenital
gradient-echo MRI scans is the ability to demonstrate deafness requiring cochlear implant.
the facial nerve and the vestibulocochlear nerve in the
internal auditory canal. Absence of a nerve can be
REFERENCES
1. Hasso AN, Casselman JW, Broadwell RA. Temporal bone
congenital anomalies. In: Som PM, Curtin HD, eds. Head Neck
Address correspondence and reprint requests to Menachem Gross, Imaging, 3rd ed. St. Louis: Mosby, 1996.
M.D., Department of Otolaryngology—Head and Neck Surgery, 2. Jackler RK, Luxford WM, House WF. Congenital malformations of
Hadassah University Hospital, Jerusalem 91120, Israel; Email: ron@ the inner ear: a classification based on embryogenesis. Laryngo-
eliashar.com scope 1987;97:2–14.

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