inner ear malformations: a practical diagnostic approach...resumen la hipoacusia neurosensorial...

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.

Document downloaded from http://www.elsevier.es, day 09/03/2018. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.Document downloaded from http://www.elsevier.es, day 09/03/2018. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

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]

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


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.



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



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).



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



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).



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.

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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

inner ear malformations: a practical diagnostic approach...resumen la hipoacusia neurosensorial...

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