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Radiología. 2017;59(4):297---305
www.elsevier.es/rx
RADIOLOGY THROUGH IMAGES
Inner ear malformations: A practical diagnostic
approach�
M. Mazóna,∗, E. Pontb, A. Montoya-Filardia, J. Carreres-Poloa,
F. Más-Estellésa
a Área Clínica de Imagen Médica, Hospital Universitario y
Politécnico La Fe, Valencia, Spainb Servicio de
Otorrinolaringología, Hospital General de Onteniente, Valencia,
Spain
Received 20 July 2016; accepted 27 September 2016
KEYWORDSDiagnostic imaging;Ear inner;Abnormalities;Cochlear
displasia;Cochlear aplasia;Incomplete partition
Abstract Pediatric sensorineural hearing loss is a major cause
of disability; although inner ear
malformations account for only 20---40% of all cases,
recognition and characterization will be
vital for the proper management of these patients. In this
article relevant anatomy and devel-
opment of inner ear are surveyed. The role of neuroimaging in
pediatric sensorineural hearing
loss and cochlear preimplantation study are assessed. The need
for a universal system of classi-
fication of inner ear malformations with therapeutic and
prognostic implications is highlighted.
And finally, the radiological findings of each type of
malformation are concisely described and
depicted. Computed tomography and magnetic resonance imaging
play a crucial role in the char-
acterization of inner ear malformations and allow the assessment
of the anatomical structures
that enable the selection of appropriate treatment and surgical
approach.
© 2016 SERAM. Published by Elsevier España, S.L.U. All rights
reserved.
PALABRAS CLAVEImagen diagnóstica;Oído
interno;Anormalidades;Displasia coclear;Aplasia coclear;Partición
incompleta
Malformaciones del oído interno: una aproximación diagnóstica
práctica
Resumen La hipoacusia neurosensorial pediátrica es una causa
mayor de discapacidad. Pese a
que solo en el 20-40% de los casos se identifica una
malformación del oído interno, su detección
es de vital importancia para el tratamiento de estos pacientes.
En este artículo se repasan la
anatomía y la embriogénesis del oído interno. Se valora el papel
de la neuroimagen en la hipoa-
cusia neurosensorial pediátrica y en el estudio preimplante
coclear. Se destaca la necesidad de
la utilización de un sistema universal de clasificación de las
malformaciones del oído interno con
� Please cite this article as: Mazón M, Pont E, Montoya-Filardi
A, Carreres-Polo J, Más-Estellés F. Malformaciones del oído
interno: una
aproximación diagnóstica práctica. Radiología.
2017;59:297---305.∗ Corresponding author.
E-mail address: [email protected] (M. Mazón).
2173-5107/© 2016 SERAM. Published by Elsevier España, S.L.U.
All rights reserved.
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dx.doi.org/10.1016/j.rxeng.2017.02.003http://www.elsevier.es/rxhttp://crossmark.crossref.org/dialog/?doi=10.1016/j.rxeng.2017.02.003&domain=pdfmailto:[email protected]
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298 M. Mazón et al.
implicaciones pronósticas y terapéuticas. Por último, se
describen e ilustran de forma concisa
los hallazgos radiológicos clave de cada tipo de malformación.
La tomografía computarizada y
la resonancia magnética desempeñan un papel crucial en la
caracterización de las malforma-
ciones del oído interno y permiten la valoración de las
estructuras anatómicas que posibilitan
la selección del tratamiento y del abordaje quirúrgico
idóneos.
© 2016 SERAM. Publicado por Elsevier España, S.L.U. Todos los
derechos reservados.
Introduction
Sensorineural hearing loss in children is a major cause
ofdisability. Its early diagnosis is very important since
delayeddiagnoses may affect the development of language,
theacademic skills and social and emotional development. Itcan be
due to congenital or acquired anomalies; at leasthalf of them have
a genetic origin and among the acquiredones the infection due to
cytomegalovirus is the most com-mon cause.1 The prevalence of
detectable cochleovestibularmalformations is around 20---40%.2
The cochlear implant is the standard therapeuticproceeding of
mild to profound sensorineural hearing lossin children.3 Previously
inner ear (IE) malformations wereone counter indication for
cochlear implants but advancesin surgical techniques and cochlear
devices have made itpossible to implant cochleas with
malformations.
Image modalities are crucial when it comes to doingpreoperative
assessments of inner ear malformations sincethey allow us to detect
and evaluate the cochlear nerveand identify any anatomic variants
and all potential surgicalcomplications.1
There are several categorization schemes for IE malfor-mations
and yet despite the fact that no scheme is perfectwe should make
the effort of trying to adopt a universalsystem that would allow us
to share our results with thescientific community.
In this paper we will take a brief look into the anatomyand
embryogenesis of the IE. We will also be evaluating therole of
neuroimages in pediatric sensorineural hearing lossand in the
cochlear preimplant study. We will be paying spe-cial attention to
the use of a somehow universal system tocategorize IE malformations
with therapeutic and prognosticimplications. Finally we will be
describing the most widelyaccepted categorization while
illustrating and detailing ina concise way the key radiologic
findings of each type ofmalformation.
Anatomy and embryology of inner ear
The IE is made up of the membranous labyrinth that inturn
surrounds the osseous labyrinth. The cochlea-organresponsible for
hearing is a conical structure consisting ofa duct that makes
between 2.5 and 2.75 turns around acentral core called modiolus
(Fig. 1). From the modiolusa thinned-osseous spiral layer reaches
out to divide theduct into vestibular duct (upper) and tympanic
duct (lower).
Through the cochlear opening the cochlea meets the fun-dus of
the internal acoustic canal (IAC) the cochlear nervepasses through.
The vestibular system is made up of thevestibule and three
semicircular ducts: superior, lateral, andposterior.
Both the duct and the endolymphatic sac are containedby the
vestibular aqueduct reaching out from the labyrinthtoward the
posterior fossa epidural space.
The IAC runs through the petrous part of the temporalbone and
communicates the cerebellopontine angle cisternwith the labyrinth
through which the cranial nerves VII andVIII pass. In an oblique
sagittal plane perpendicular to theIAC we can see the facial nerve
in the anterior-superiorquadrant, the cochlear nerve in the
anterior-inferior quad-rant and the upper and lower vestibular
nerves in theposterior quadrants.
The IE stems from the otic placode that starts develop-ing
during the 3rd week of pregnancy. The development ofthe cochlea is
completed in the 8th week of pregnancy, thevestibule in the 11th
week and semicircular ducts betweenthe 19th and 22nd weeks of
pregnancy. The first semicircularduct to develop is the superior
one followed by the posteriorone and the last one to develop is the
lateral one.4
The role of neuroimaging in inner earmalformations
Most candidates for a cochlear implant do not show anoma-lies in
their temporal bone that can be identified throughimages,1 but if
they show such anomalies finding them is veryimportant. Both
computed tomography (CT) and magneticresonance imaging (MRI) give
us an excellent representa-tion of IE malformations and they are
used in the systematicpractice of the study of pediatric
sensorineural hearing lossand cochlear pre-implants. Because of
their complementaryrole the use of both image modalities is
recommended sincethe rate of malformation detection increases
dramatically.5
CT allows us to detect bone malformations, also the facialnerve
possible aberrant trajectory (more common in thispopulation6),
vascular structure malformations and assesscoexisting anomalies of
both the inner and middle ears.
When it comes to the MRI it allows us to assess
fluid-filledspaces of the IE and assess the cranial nerve VII and
any otherpossible intracranial anomalies.
Combined they provide the surgeon with the necessarypresurgical
information that will allow him/her to make atherapeutic decision,
give advise for parents and give the
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Inner ear malformations: A practical diagnostic approach 299
Figure 1 Chart showing the EI made up of the cochlea (co), the
vestibule (v) and the semicircular ducts (csc). The modiolus
(m)
is the base of the cochlea at the fundus of the internal
acoustic canal (IAC). The cochlear coils are separated by the
interscalar
septum (ti); the coil is split into vestibular duct (rv) and
tympanic duct (rt) by the spiral lamina (le) that stems from the
modiolus.
The vestibular aqueduct (av) stems from the vestibule toward the
epidural espace of the posterior fossa with a trajectory across
the
petrous part of the temporal bone and perpendicular to the IAC.
(B) Representation of anatomical structures in a transverse
image
using computed tomography in bone window. (C) Representation of
anatomical structures in a transverse image using a
T2-weighted,
echo-gradient, high-resolution, steady-state MRI sequence. (D)
Oblique, sagittal reconstruction perpendicular to the IAC of the
MRI
sequence showing the facial nerve (nf) in the superior-anterior
quadrant, the cochlear nerve (nc) in the inferior-anterior
quadrant,
the superior vestibular nerve (nvs) in the superior-posterior
quadrant, ant the inferior vestibular nerve (nvi) in the
inferior-posterior
quadrant. In this image the anterior part is marked with an A
and the posterior one with a P.
most likely prognosis for the cochlear implant, choose
thesurgical approach and the appropriate implants and warnabout
anatomic variants and any other possible
surgicalcomplications.1
Classification
There are many classifications when it comes to IE
malforma-tions and it is precisely the absence of a common
languagethat makes understanding hard for the scientific
commu-nity. We need a universal classification system that allowsus
to determine the link among the different types of mal-formations
and clinical prognosis. The most widely acceptedcategorization is
Sennaroglu classification7---the classifica-tion this paper is
based on. It is based on embryogenesis.Every malformation is the
consequence of an interruption indevelopment at one time or
another. Generally speaking itis useful and the more serious the
malformation, the more
surgical complications we will find and worse the outcomeof
cochlear implant will be.2,3,5,6
In our radiologic report on top of classifying mal-formations we
also give detailed information on threeanatomic structures involved
in the cochlear implant:cochlear lumen---where the electroded will
be placed: themodiolus---target of electrostimulation; and the
cochlearnerve---the route through which the stimulus travels.2
Now let us take a look at the different types of malfor-mations
we can see (table available online).
Complete labyrinthine aplasia
Also known as Michel aplasia it is the result of the
interrup-tion of the otic placode development before the 3rd weekof
pregnancy. It amounts to 1% of IE malformations only.8 Itcounter
indicates cochlear implantation and the best ther-apeutic option is
the brainstem implant.8
Figure 2 Complete labyrinthine aplasia. (A) Transverse image
using computed tomography in bone window with identifiable
total
absence of inner ear elements (asterisk). We can see hypoplasia
in the petrous part of the temporal bone (white arrow), the
atresic
internal acoustic canal (IAC) with a reduced caliber (black
arrow), and the flattening of the inner ear medial side
(arrowhead). (B)
Transverse image using T2-weighted, echo-gradient, steady-state
MRI sequence where the aforementioned findings can be seen as
well as the cranial pairs; in this case there is an absolute
deficit of the vestibulocochlear nerve and only the facial nerve
(arrowhead)
can be seen in the cerebellopontine angle cistern and the
IAC.
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300 M. Mazón et al.
Figure 3 Cochlear aplasia. (A) The chart shows the total absence
of the cochlea (asterisk) and the normalcy of the inner ear
remaining structures. (B and C) Transverse image using computed
tomography sequence in bone window---superior and inferior,
respectively, where we can easily identify the absence of the
cochlea (black asterisk). The vestibule is dysplasic (black arrow)
and
the facial nerve shows an aberrant itinerary with a widening of
its labyrinthic part (white arrow). In C we can see the
flattening
of the inner ear medial side (arrowhead)---with this finding we
can distinguish this malformation from labyrinthitis ossificans.
This
patient also showed chronic otitis media and inflammatory
erosion of the ossicular chain; the occupation of the middle ear
and
mastoid air cells by a soft dentistry tissue/material and almost
total erosion of the ossicular chain (white asterisk).
It is characterized by a total absence of IE struc-tures (Fig.
2). The IAC is atresic, there is aplasia of thecochleovestibular
nerve and the facial nerve trajectory isaberrant. It is associated
with multiple anomalies of thetemporal lobe among which the
following ones are the mostcommon of all: petrous apex hypoplasia,
absence of roundand oval windows and flattening of the middle ear
medialwall due to absence of promotorium.4,9
Cochlear aplasia
It is due to developmental interruption at the end ofthe 3rd
week of pregnancy10 and it amounts to 3% of allIE malformations.1
It is a counterindication for cochlearimplantation.
The cochlea is absent and the vestibular system can benormal,
dysplasic, distinguishable from the cochlea due toits posterior
location with respect to the IAC (Fig. 3). Thelabyrinthic segment
of the facial nerve has an aberrant tra-jectory and the inner ear
medial wall is flattened.
We should remember that: in labyrinthine apla-sia and cochlear
aplasia there is flattening of theinner ear medial wall---useful
finding to distinguish themfrom labyrinthitis ossificans (Fig. 4
available online).This differentiation is clinically relevant since
in thelabyrinthitis ossificans the cochear implant is possiblehard
to accomplish.
Common cavity
It is the result of developmental interruption duringthe 4th
week of pregnancy and amounts to 25% of allmalformations.4,10
Cochlear implant is possible though hardto accomplish and the rate
of complications is higher.11
It is characterized to be the confluence of the cochleaand the
vestibule in one only cystic cavity without internal
Figure 4 Online Labyrinthitis ossificans Transverse image
using computed tomography sequence showing an almost com-
plete cochlear ossification (black arrow). The inner ear
medial
side is not flattened and the promontorium can be easily
iden-
tified (white arrow) --- findings that will allow us to
distinguish
it from labyrinthine and cochlear aplasias.
architecture (Fig. 5). There is a total absence of modio-lus and
a large communication between the cavity and theIAC.12 Semicircular
ducts are usually dysplasic though thereare times that they may
look normal.4
Incomplete partition type I
Also known as cystic cochleovestibular malformation it isdue to
developmental interruption during the 5th week ofpregnancy.10
The cochlea shows a total absence of the modiolusand cystic
appearance without internal architecture; thevestibule is dilated.
The labyrinth as a whole has the appear-ance of the figure eight
(Fig. 6). In this malformation wecan see the cochlea and the
vestibule which in turn allows
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Inner ear malformations: A practical diagnostic approach 301
Figure 5 Common cavity. (A) The chart shows common cystic cavity
without internal architecture made up by rudimentary
cochlea and vestibule. (B and C) Transverse image and coronal
multiplanar reconstruction using computed tomography in window
bone, respectively showing the common cavity (white arrows) and
slightly dysplasic, widened and short semicircular ducts (black
arrows). The fundus of the IAC showing cribiform plate deficits
(arrowhead) enters the center of the common cavity; finding
that
allows us to distinguish this malformation from cochlear aplasia
with dysplasic dilated vestibule in which the fundus of the IAC
location is anterior to the cavity that is more posterior in the
normal situation of the vestibule.
us to distinguish it from the common cavity. The cribiformregion
located between the cochlea and the IAC is
usuallydefective.11,13
Incomplete partition type II
It is due to developmental interruption during the 7th weekof
pregnancy and it is the most common dysplasia of all(present in 50%
of all malformations).4
It shows fusion of the middle and apical turns and a cys-tic
appearance due to a defect in the apical segment of themodiolus,
the interscalar septum and the osseus spiral lam-ina (Fig. 7). The
modiolus basal coil and the basal segmentlook normal.
It is usually associated with an enlarged vestibular aque-duct
(due to duct and endolymphatic sac dilation)14 and witha minimal
vestibular dilation which makes up the Mondinideformity triad.7
We should remember that: it is not right to defineincomplete
partition as a cochlea consisting of 1.5 turnssince that is
reserved for cochlear hypoplasia.
Incomplete partition type III
It is a recessive genetically inherited disease linked
tochromosome X and it is not due to the developmentalinterruption
of the otic placode. It usually affects males andmixed hearing loss
of rapid progression.15
There is a total absence of the modiolus though the inter-scalar
septum is present (Fig. 8). The cochlea is locatedlaterally to the
IAC that is in turn widened and both widelycommunicated due to a
complete deficit of the criboselayer.15,16
We should remember that: malformations with crib-iform plate
deficits and a wide communication betweenthe IAC and the cochlea
(common cavity, incom-plete partitions types I and III) predispose
to a higherrisk of recurrent meningitis, perilymphatic fistula
andcomplications during surgery.
Figure 6 Incomplete partition type I. (A) The chart shows the
cochlea with a total absence of modiolus and interscalar septum
without internal architecture. The vestibule is dysplasic,
slightly dilated and the labyrinth as a whole shows the shape of
figure eight.
(B) Transverses image using computed tomography in bone window.
(C) Maximum intensity projection reconstruction of transverse
image using T2-weighted, steady-state, echo gradient MRI
sequence. In B and C we can see the following findings: cochlea
without
internal architecture (white arrow) and slightly dilated
vestibule (black arrow) making up an eight shaped-labyrinth.
Cribiform
plate deficit (arrowhead) with wide communication between the
IAC and the cochlea can be identified here. In C the cochlear
nerve
deficit can be seen (black asterisk in the theoretical location
of the nerve). The patient shows chronic otitis media; in B we can
see
the occupation of the middle ear (white asterisk).
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302 M. Mazón et al.
Figure 7 Incomplete partition type II. (A) The chart shows the
confluence of the middle and apical cochlear coil due to the
absence
of the apical part of the modiolus and interscalar septum that
gives the apex a cystic appearance (arrows). The vestibular
aqueduct
is usually widened due to duct and endolymphatic sac dilation
(arrowhead). (B) Transverse image using computed tomography
(CT)
in bone window. (C) Transverse image using T2-weighted,
steady-state, echo-gradient MRI sequence. In B and C we can
identify the
following findings: cystic apex due to fusion of middle and
apical coils (white arrows), and dilated vestibular aqueduct in the
CT,
and duct and endolymphatic sac dilation in the MRI (arrowhead).
(D) Oblique sagittal reconstruction perpendicular to the IAC of
the T2-weighted, steady-state, echo-gradient sequence where a
normal size cochlear nerve (arrow) can be seen. The upper part
is
marked with an A and the posterior one with a P. The patient
showed chronic otitis media; in B and C we can see the occupation
of
the middle ear (asterisk).
Figure 8 Incomplete partition type III. (A) The chart shows
rotated cochlea located directly in the lateral edge of the IAC
with a
total absence of the modiolus but with the interscalar septum.
There is cribiform plate deficit with wide communication between
the
IAC and the cochlea. (B) Transverse image using computed
tomography in bone window. (C) Transverse image using
T2-weighted,
steady-state, echo-gradient MRI sequence. In B and C we can see
rotation of the cochlea (the curved arrow shows the rotation
direction) directly lateral to the IAC showing a total absence
of the modiolus (asterisk) but the interscalar septum (arrowhead).
In
C we can see a cochlear nerve of normal thickness (arrow).
Cochlear hypoplasia
It is due to developmental interruption during the 6th weekof
pregnancy.4 The dimensions of the cochlea are smallerbut there is a
well-established differentiation betweenthe cochlea and the
vestibule. When in lack of expe-rience measurements should be taken
to facilitate itsdetection.17
It is a generic term and three subtypes should be takeninto
consideration here (Fig. 9):
Type I, ‘‘yolk sac’’ of the cochlea: the cochlea is a
smallexcrescence stemming from the vestibule with no
internalarchitecture and a total absence of modiolus and
inter-scalar septum.Type II, cystic hypoplasic cochlea: the cochlea
showsreduced dimensions, no mediolus or interscalar sep-tum but its
external structure is normal. It shows wide
communication with the IAC that predisposes to a higherrisk of
complications during surgery. The vestibular aque-duct is usually
widened and the vestibule is slightlydilated.7
Type III, cochlea with less than two turns: the cochleashows
less than two turns, the modiolus is smaller andthe interscalar
septum is shorter but both its internal andinternal architectures
are normal. The vestibule and thesemicircular ducts are usually
hypoplasic.7
Widened vestibular aqueduct syndrome
Its etiology is controversial; it could be due to postna-tal
developmental disorders.18 It consists of an isolatedvestibular
aqueduct widening due to the duct and endolym-phatic sac abnormal
developments being the remainder ofthe labyrinth normal (Fig. 10).
The aqueduct is said to be
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Inner ear malformations: A practical diagnostic approach 303
Figure 9 Cochlear hypoplasia. (A) The chart shows the existing
three types of cochlear hypoplasia: type I ‘‘yolk sac’’, like a
small
excrescence stemming from the vestibule; type II, cystic
hypoplasic of normal external architecture but small dimensions and
no
internal architecture due to the absence of modiolus and
interscalar septum; type III, less turns with normal external and
internal
architecture but shorter modiolus and interscalar septum which
conditions the cochlea with less than two turns. (B) Transverse
image using computed tomography (CT) in bone window showing
hypoplasic ‘‘yolk sac’’-like cochlea stemming from the
vestibule
like a small excrescence (type I). (C) Transverse image using CT
in bone window showing one hypoplasic cystic cochlea of normal
external architecture but reduced dimensions and no internal
architecture (type II). (D) Transverse image using CT in window
bone
showing one hypoplasic cochlea with less than two turns (type
III).
Figure 10 Dilated vestibular aqueduct syndrome. (A) Transverse
image using computed tomography in bone window showing
vestibular aqueduct widening (white arrow); it is useful to use
the caliber of the superior semicircular duct as a reference of
the upper threshold of normalcy (arrowhead). (B) Transverse
image using T2-weighted, steady-state, echo-gradient MRI
sequence
showing duct and endolymphatic dilation (white arrow) and the
posterior semicircular duct as a reference (arrowhead). Both
the
cochlea and the rest of the inner ear are normal (black arrow in
A and B).
widened when its caliber is larger than 0.8 mm in its
centralpoint in an oblique plane at 45◦ (plane of Pöschl).19
Vestibular system malformations
It is due to developmental disorders between the 6th and22nd
weeks of pregnancy. In isolation it is not relevant forthe cochlear
pre-implant study; its importance has to do
with a strong association with other IE malformations andcertain
syndromes. The vestibule and the lateral semicircu-lar duct
dysplasia is one of the strongest ones and consistsof a short and
wide lateral semicircular duct fused or sepa-rated through a small
bony islet to the vestibule of globularappearance (Fig. 11). There
is a strong correlation betweenthe aplasia of semicircular ducts
and CHARGE syndrome20
(Fig. 12).
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304 M. Mazón et al.
Figure 11 Vestibular malformation. Transverse image using
MRI in bone window showing the vestibule of globular appear-
ance fused into the lateral semicircular duct (arrow).
Figure 12 Vestibular malformation. Transverse image using
MRI in bone window in patient with CHARGE syndrome showing
vestibular hypoplasia (black arrow) and semicircular ducts
apla-
sia (asterisk). It shows cochlear hypoplasia type II
(arrowhead)
and fused ossicular chain dysplasia (white arrow). The
CHARGE
syndrome is a genetic poliformative disease whose abbrevia-
tion stands for Coloboma of the eye, heart defects, atresia
of
the choanae, retardation of growth and development, and ear
abnormalities.
Conclusions
The CT and the MRI play a crucial role both in the
char-acterization of IE malformations and in the assessment ofthe
anatomical structures that will allow us to choose theappropriate
therapy, management and approach. The useof a universal system of
classification all clinicians agree onis necessary in order to
determine the correlation betweenthe different types of
malformation and clinical prognosis. Inthis paper we tried to
describe each and every single type of
malformation and illustrate and give precise details on
keyradiological findings.
Ethical responsibilities
Protection of people and animals. The authors declare thatthe
proceedings followed fully abide by the ethical rules
andregulations from the human experimentation committee andare in
full compliance with the World Health Organization(WHO) and the
Declaration of Helsinki.
Confidentiality of data. The authors confirm that they
havefollowed their centers protocols on the publication and
dis-closure of data from patients.
Right to privacy and informed consent. The authors con-firm that
they have received written informed consent fromthe patients and/or
individuals referred to in this paper. Thismanuscript belongs to
the corresponding author.
Authors
1. Manager of the integrity of the study: MM and EP.2. Study
Idea: MM, EP, AMF, JCP and FME.3. Study Design: MM and EP.4. Data
Mining: MM, EP, AMF, JCP and FME.5. Data Analysis and
Interpretation: N/A.6. Statistical Analysis: N/A.7. Reference: MM,
EP, AMF, JCP and FME.8. Writing: MM and EP.9. Critical review of
the manuscript with intellectually rel-
evant remarks: MM, EP, AMF, JCP and FME.10. Approval of final
version: MM, EP, AMF, JCP and FME.
Conflicts of interests
The authors declare no conflict of interests associated withthis
article whatsoever.
Appendix A. Supplementary data
Supplementary material related to this article can be found,in
the online version, at
http://dx.doi.org/10.1016/j.rx.2016.09.009.
References
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Inner ear malformations: A practical diagnostic
approachIntroductionAnatomy and embryology of inner earThe role of
neuroimaging in inner ear malformationsClassificationComplete
labyrinthine aplasiaCochlear aplasiaCommon cavityIncomplete
partition type IIncomplete partition type IIIncomplete partition
type IIICochlear hypoplasiaWidened vestibular aqueduct
syndromeVestibular system malformations
ConclusionsEthical responsibilitiesProtection of people and
animalsConfidentiality of dataRight to privacy and informed
consent
AuthorsConflicts of interestsAppendix A Supplementary
dataReferences