Balkan Med J 2017;34:397-411 Invited Review 397

Classification and Current Management of Inner Ear Malformations

Levent Sennaroğlu, Münir Demir Bajin

Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey

Morphologically congenital sensorineural hearing loss During cochlear implantation, there may be facial nerve
can be investigated under two categories. The majority abnormalities, cerebrospinal fluid leakage, electrode
of congenital hearing loss causes (80%) are membranous misplacement or difficulty in finding the cochlea itself.
malformations. Here, the pathology involves inner ear During surgery for inner ear malformations, the surgeon
hair cells. There is no gross bony abnormality and, must be ready to modify the surgical approach or choose
therefore, in these cases high-resolution computerized special electrodes for surgery. In the present review
tomography and magnetic resonance imaging of the article, inner ear malformations are classified according
temporal bone reveal normal findings. The remaining
to the differences observed in the cochlea. Hearing and
20% have various malformations involving the
language outcomes after various implantation methods
bony labyrinth and, therefore, can be radiologically
are closely related to the status of the cochlear nerve, and
demonstrated by computerized tomography and
magnetic resonance imaging. The latter group involves a practical classification of the cochlear nerve deficiency
surgical challenges as well as problems in decision- is also provided.
making. Some cases may be managed by a hearing aid, Keywords: Inner ear malformations, cochleovestibular
others need cochlear implantation, and some cases are malformations, classification, treatment, incomplete
candidates for an auditory brainstem implantation (ABI). partition, cochlear hypoplasia, radiology, surgery

Inner ear malformations (IEM) represent approximately 20% of If we do not have a common language, it is very difficult to
congenital hearing loss cases based on radiology (1,2). Majority understand the findings of another research group. Universally
of these patients have bilateral severe to profound hearing accepted classification of IEMs is particularly important in
loss and are candidates for cochlear implantation (CI). Those the field of CI. Surgeons, audiologists, speech and language
cases with severe malformations may require different surgical specialists should be familiar with this system; otherwise, it will
approach for implant placement. Decision making between be very difficult to understand and compare the outcome after CI
CI and auditory brainstem implantation (ABI) may also be surgery in this particular patient group.
challenging in some cases of IEMs. There are certain challenges in the management of IEMs:
It is very important to classify IEMs correctly and have 1. Cerebrospinal fluid gusher and risk for meningitis
a universally accepted system. There is a large variety of 2. Facial nerve anomalies
malformations which complicate diagnosis and management. 3. Decision making for the surgical approach and the type of
Proper classification is as important as using a common language. electrode

Address for Correspondence: Dr. Levent Sennaroğlu, Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
e-mail: [email protected]
Received: 15 March 2017 Accepted: 24 August 2017 • DOI: 10.4274/balkanmedj.2017.0367
Available at www.balkanmedicaljournal.org
Cite this article as:
Sennaroğlu L, Bajin MD. Classification and Current Management of Inner Ear Malformations. Balkan Med J 2017;34:397-411
©Copyright 2017 by Trakya University Faculty of Medicine / The Balkan Medical Journal published by Galenos Publishing House.
398 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

4. Choosing the correct implantation method; CI vs ABI turns. The cochlear aperture (CA) (bony canal for cochlear
5. Timing of surgery nerve) is the central bony passage at the base of the modiolus
Classification of IEMs is based on differences in cochlear transmitting the cochlear nerve and blood vessels.
anatomy in various malformations. With this classification, Section inferior to the mid-modiolar view, passes through the
cochlear anomalies with similar appearance are grouped area of the round window niche (Figure 1b). This section shows
together. They demonstrate similar clinical findings, and the basal, middle, and apical cochlear turns. Basal turn is in
treatment options. This may not represent the functional continuity at this section. It is important to see the interscalar
outcome with CI, which is closely related to the situation of the septum between the middle and apical turns. This view is very
cochlear nerve. If there is cochlear nerve deficiency, this will important to differentiate cochlear hypoplasia (CH) type IV, in
have a negative influence on the audiological and speech and addition to incomplete partition type II. A thin-section, heavily
language developmental after implantation. Therefore, during T2 weighted image of the cochlea demonstrates fluid filled
preoperative decision making for choosing the method of spaces of the cochlea (scala tympani and vestibuli), modiolus
implantation, three factors should be considered: classification and cochlear nerve (Figure 1c).
of IEMs, situation of the cochlear nerve and preoperative
audiological findings. Only in this way, a clinician may have
a better estimate of the audiological outcome in a given IEM.
This is very important during preoperative counseling of the
family. In the present paper, classification of cochlear nerve
abnormalities is also provided.
This review article describes the characteristics of anomalies in
eight groups together with treatment options.

Normal Cochlea
FIG. 1a. Mid-modiolar view demonstrating modiolus (M) and cochlear
It is important to become familiar with normal anatomy of the aperture, bony canal for cochlear nerve.
cochlea as seen on high resolution computerized tomography
of the temporal bone. Temporal bone HRCT was performed on
sections of 0.5 mm in thickness. The use of a 1.5- or 3-T MR
imaging system is preferred for inner ear examinations, and
it is strongly suggested to be done under general anesthesia.
A thin-section gradient-echo sequence that is heavily T2
weighted is best suited for evaluation of the fluid-filled spaces
of the membranous labyrinth and the eighth cranial nerve. A
section thickness of as little as 0.4-0.7 mm is preferred for
optimal delineation and to allow the generation of high-quality FIG. 1b. Section passing through round window niche (Figure 1b),
multiplanar reformatted images. Oblique sagittal reformatted showing basal (B), middle (M), and apical (A) cochlear turns.
images are obtained in planes perpendicular to the course of the
seventh and eighth nerves in the internal auditory canal (IAC)
and cerebellopontine angle. Routine axial T2-weighted imaging
of the brain should be performed in all patients to exclude
central nervous system causes of sensorineural hearing loss.
Cochlea has 2½ or 2¾ turns (Figure 1). Mid-modiolar view is
the most important section to evaluate internal architecture of
cochlea and differentiate normal cochlea and incomplete partition
anomalies. Mid-modiolar view (Figure 1a) demonstrates the
modiolus as a quadrangular or pentagonal structure in the center
of the basal turn and between the basal and middle turns of the
cochlea (2). Interscalar septa are thicker partitions between the
FIG. 1c. Axial T2 weighted magnetic resonance image showing cochlear
inner wall of the cochlea and the modiolus, which separate the nerve (CN), scala tympani (ST), scala vestibuli (SV), modiolus (M) and
normal cochlea into 2½ or 2¾ turns; the basal, middle and apical vestibule (V).

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Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 399

I-INNER EAR MALFORMATIONS Audiological Findings

These patients either do not show a response during
According to present literature (3,4), IEMs are classified into
audiological evaluation or they may demonstrate profound
eight distinct groups. Characteristics of each group can be
sensorineural hearing loss on low frequencies which should
found in Table 2.
be accepted as vibrotactile stimulation.
1- Complete Labyrinthine Aplasia (Michel Deformity)
Management
Complete labyrinthine aplasia (CLA) is the absence of the It is not possible to perform CI surgery in these children as
cochlea, vestibule, semicircular canals (SCCs), vestibular there is no inner ear development. In the first Consensus
and cochlear aqueducts (Figure 2). The petrous bone may be Meeting on ABI in Children (5), CLA together with other
hypoplastic whereas the otic capsule may be hypoplastic or severe IEMs are accepted as “Definite Indications for
aplastic (4). In the majority of patients, the IAC consists only ABI” (Table 1). ABI is the only surgical option for hearing
of the facial canal and the labyrinthine, tympanic and mastoid habilitation.
segments of the facial nerve can be identified in the temporal
bone. In some patients, however, it may not be possible to 2- Rudimentary Otocyst
observe the facial canal in the temporal bone in spite of normal A rudimentary otocyst is used to define incomplete milimetric
facial functions. Development of middle ear ossicles are usually representations of the otic capsule (round or ovoid in shape)
normal. without an IAC (Figure 3). Parts of the SCCs may accompany
rudimentary otocyst. This pathology represents an anomaly
between a Michel deformity and common cavity (CC). In
Michel deformity, there is no inner ear development, while
in CC, there is an ovoid or round cystic space instead of a
separate cochlea and vestibule. The CC communicates with
the brainstem via the nerves in the IAC. The rudimentary
otocyst is a few millimeters in size without the formation of
an IAC.

FIG. 2. Complete labyrinthine aplasia; absence of cochlea, vestibule,

semicircular canals, vestibular and cochlear aqueducts. Middle ear
ossicles are usually present (M=malleus).

According to radiological findings (4), three subgroups of CLA

are present:

a- CLA with hypoplastic or aplastic petrous bone

In these cases CLA is accompanied by hypoplasia or aplasia of
FIG. 3. Rudimentary otocyst (RO); incomplete millimetric
the petrous bone. Middle ear may be adjacent to posterior fossa. representations of the otic capsule (round or ovoid in shape) without
an IAC.
b- CLA without otic capsule
In this group of CLA, formation of the petrous bone is normal, Audiological Findings
but the otic capsule is hypoplastic or aplastic. Similar to CLA, either there is no response at all or profound
c- CLA with otic capsule loss on low frequency which is vibrotactile stimulation.

Formation of the petrous bone and the otic capsule is normal. Management
Only in this group of CLA with otic capsule development, The fact that there is no connection between the otocyst and
labyrinthine segment of the facial canal is in its normal location. the brainstem is a contraindication to CI surgery. Rudimentary
This shows that otic capsule formation is essential for the facial otocyst is also a definite indication for ABI (Table 1).
canal to obtain its normal position.

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 TABLE 1. Definite and possible indications for auditory brainstem implantation 400
Indications Definite Possible
Complete labyrinthine aplasia (Michel) Cochlear nerve deficiency
Rudimentary otocyst Incomplete partition I and common cavity where distribution of cochlear nerve is not
predictable
Cochlear aplasia Cochlear hypoplasia with hypoplastic CN
Cochlear aperture aplasia Unbranched cochleovestibular nerve
Cochlear nerve aplasia

Balkan Med J, Vol. 34, No. 5, 2017

Incomplete partition type I, common cavity, cochlear hypoplasia with no cochlear
nerve

TABLE 2. Characteristics of the inner ear malformations

Type of IEM Radiology Audiology Gusher FN anomaly Treatment modality Electrode choice
Complete Absent labyrinth Profound SNHL Yes ABI ABI
labyrinthine aplasia
Incomplete milimetric otic capsule Profound SNHL Yes ABI ABI
Rudimentary otocyst
remnant
Cochlear aplasia Absent cochlea Profound SNHL Yes ABI ABI
Round or ovoid cystic structure for Profound SNHL Rarely Yes CI or ABI Transmastoid labyrinthotomy or double
Common cavity cochlea and vestibule labyrinthotomy, AVOID MODIOLAR
HUGGING ELECTRODE
Cochlear size small, four types+ Conductive, mixed, In CH-II possible Yes HA, Stapedotomy, Thin and short electrode
Cochlear hypoplasia
SNHL CI or ABI
Incomplete Cystic cochlea Profound SNHL 50% of the cases Possible CI or ABI Electrode with stopper
partition-I
Cystic cochlear apex Normal to profound Always pulsation Not expected HA or CI, NO ABI Any electrode but electrode with stopper
Incomplete
mixed or SNHL, but gusher <10% of preferred
partition-II
progressive the cases
Modiolus absent, interscalar septa Mixed or SNHL 100% of cases Yes HA or CI, NO Electrode with stopper.
Incomplete
present Stapedotomy, NO AVOID MODIOLAR HUGGING
partition-III
ABI ELECTRODES
Normal cochlea with enlarged VA Normal to profound Always pulsation Not expected HA or CI, NO ABI Any electrode but electrode with stopper
Enlarged vestibular
mixed or SNHL, preferred
aqueduct
progressive
Narrow or absent cochlear aperture Profound SNHL, OAE none Not expected CI for CN Standard CI or ABI
Cochlear aperture
may be normal, profound hypoplasia, or ABI
abnormalities
SNHL if CN is absent
IEM: inner ear malformatrion; SNHL: sensorineural hearng loss; FN: facial nerve; VA: vestibular aquaduct; CN: cochlear nerve; HA: hearing aid; CI: cochlear implantation; ABI: auditory brainstem implantation; OAE:
otoacoustic emissions
Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations
Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 401

3- Cochlear Aplasia In the latter IAC is normally developed, and dilated vestibule
Cochlear aplasia is the absence of the cochlea. The labyrinthine occupies normal location at the posterolateral part of the fundus.
segment of the facial nerve is anteriorly displaced and occupies CI surgery should not be done in CADV. In CC, however, IAC
the normal location of the cochlea. Vestibule and SCCs are in is usually posteriorly directed and opens into the center of CC. If
their normal anatomic location; at the posterolateral part of IAC. cochleovestibular nerve (CVN) is present, CI can be done in CC.
There are two subgroups according to accompanying vestibular However, in some patients, it may be very difficult to distinguish
system: between these entities. As in every CI candidate, during preoperative
a- Cochlear aplasia with normal labyrinth (Figure 4a): evaluation for implantation, audiological findings should be taken
Vestibule and SCCs are normally developed. into account in choosing the right method of implantation.
Cochlear aplasia with normal labyrinth is usually symmetric.
b- Cochlear aplasia with a dilated vestibule (CADV) (Figure
Similar appearance is present in different individuals,
4b): Vestibule and SCCs show dilatation. It is very important
suggesting a genetic etiology. In CADV, however, asymmetric
to differentiate CADV from a common cavity (CC) deformity.
development may be present; pathology may be due to genetic
or environmental factors. Otic capsule development is always
normal.

Audiological Findings
These patients do not have a hearing level, the only stimulation
can be vibrotactile.

Management
As there is no inner ear development, ABI is the only feasible
surgical option to provide hearing in children with cochlear
aplasia (5).

4- Common Cavity
A CC is defined as a single, ovoid or round chamber, representing
cochlea and vestibule (Figure 5). Theoretically, this structure
has cochlear and vestibular neural structures. There may be
accompanying SCC or their rudimentary parts. The IAC usually
enters the cavity at its center. Cases with vestibular dilatation are
FIG. 4a. Cochlear aplasia with normal labyrinth: vestibule and occasionally termed as “vestibular common cavity”; however,
semicircular canals are normal (location and development), Labyrinthine
segment of the facial nerve (LS) is anteriorly dislocated. this is not a correct term.

FIG. 4b. Cochlear aplasia with a dilated vestibule: vestibule (V) and
semicircular canals are located normally but there is vestibular dilatation.
Labyrinthine segment of the facial nerve (LS) is anteriorly dislocated. FIG. 5. Common cavity (CC); a single, ovoid or round chamber,
IAC: internal auditory canal representing cochlea and vestibule.

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402 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

CC needs to be differentiated from cochlear aplasia with dilated • Beltrame et al. (7) described a special electrode for CC.
vestibule (1). CADV (Figure 4b) has a dilated vestibule and This electrode has a inactive tip which is caught by a hook
SCCs at the posterolateral part of the IAC fundus, which is through another hole. Two labyrinthotomy openings are
their usual location. External outline resembles the normal created in the area of lateral SCC, separated by 3-4 mm.
labyrinth. The enlarged vestibule is at its expected location. The terminal nonactive part of the electrode array ends with
The accompanying SCCs may be enlarged or normal. A CC a small ball, which is needed to hook the electrode array.
(Figure 5), on the other hand, is an ovoid or round structure. This nonactive part of the implant is pushed into the superior
SCC’s or their rudimentary parts may accompany a CC. labyrinthotomy until it is seen and hooked using a 0.5 mm
The IAC usually enters the cavity at its center. The location hook through the inferior labyrinthotomy. Then the two arms
of a CC may be anterior but usually posterior to the normal are advanced together to position the array along the inner
location of the labyrinth. It is very important to differentiate wall of the cavity through the inferior labyrinthotomy.
these malformations from each other, because CI in a CC may • In case of insufficient hearing and language development
result in acoustic stimulation, whereas in CAVD, no functional with CI use, ABI may be indicated on the contralateral side
stimulation will occur with CI. In spite of this, it may sometimes (possible ABI indication).
be difficult to differentiate between the two malformations. • If CVN is absent or no IAC is present, ABI is the only option
Correct terminology for the nerve entering the CC is common in the first place.
CVN. CVN has to be demonstrated by 3 Tesla MRI in candidates
undergoing evaluation for CI candidacy. Theoretically CVN Cochlear Hypoplasia and Incomplete Partitions
contains cochlear and vestibular nerve fibers, however with the In these two groups of malformations, there is a clear
present radiological investigations, it is not possible to determine differentiation into a cochlea and vestibule.
the percentage of cochlear fibers within the CVN. Audiological
evaluation is very important to determine if hearing is present 5- Cochlear Hypoplasia
in CC, which indirectly gives an estimate of the cochlear In this deformity, there is a clear differentiation between cochlea
fibers within the CVN. If a behavioral audiometric response or and vestibule. CH represents a group of cochlear malformations
language development is present with hearing aid use, it can be where external dimensions are less than those of a normal cochlea
assumed that a meaningful population of cochlear fibers exists with various internal architecture deformities. In smaller cochlea,
and the patient may benefit from a CI. If the CVN cannot be it is usually difficult to count the number of turns with CT and/
demonstrated with MRI or there is a narrow or long IAC, where or MRI. But the definition “cochlea with 1.5 turns” should be
the presence of cochlear fibers is questionable, an ABI may be used for hypoplasia (particularly type III), rather than for IP-II
a more appropriate option from the outset. As the postoperative cochlea. Four different types of CH have been defined (4):
hearing cannot be accurately predicted before CI surgery, it is
advisable to counsel the family that contralateral ABI may be Types of Cochlear Hypoplasia
necessary in case of limited language development with CI.
a- CH-I (Bud-like cochlea)
These cases are regarded as “Possible Indications for an ABI”
(Table 1) (5). This decision should be done as early as possible. The cochlea is like a small bud, round or ovoid in shape, arising
from the IAC (Figure 6A). Internal architecture is severely
Audiological Findings deformed; modiolus and interscalar septa cannot be identified.
These patients usually have profound hearing loss.
b- CH-II (Cystic hypoplastic cochlea)
Management The cochlea has smaller dimensions with defective modiolus
• For CI surgery, the surgical approach is via a transmastoid and interscalar septa, but with normal external outline (Figure
labyrinthotomy as described by McElveen et al. (6) with a 6b). There may be complete absence of modiolus creating a wide
straight (non-modiolar hugging) electrode. This will have a connection with the IAC, making gusher and misplacement of
position on the periphery of the CC with better contact with CI electrode into IAC possible. The vestibular aqueduct may
the neural tissue. A pre-curved electrode will have the contacts be enlarged and the vestibule may be dilated. They may have
located medially and may not stimulate the periphery of the CC recurrent meningitis because of defective stapes footplate.
efficiently. There is a variation in the size of CC. Therefore,
correct length of the electrode should be estimated before c- CH-III (Cochlea with less than 2 turns)
surgery. The length of the electrode can be calculated using the The cochlea has fewer turns (less than 2 turns) with a short
formula 2πr, where r is the radius of CC. modiolus. The overall length of the interscalar septa is reduced.

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Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 403

The internal (modiolus, interscalar septa) and external outline d- CH-IV (Cochlea with hypoplastic middle and apical
are similar to that of a normal cochlea, with fewer number of turns)
turns and smaller dimensions (Figure 6c). The vestibule and the The cochlea has a normal basal turn, but middle and apical
SCCs are usually hypoplastic. The CA may be hypoplastic or turns are severely hypoplastic and located anterior and medially
aplastic. rather than in their normal central position (Figure 6d). The
labyrinthine segment of the facial nerve is usually located
anterior to the cochlea rather than in its normal location (8).

FIG. 6a. CH-I (Bud-like cochlea); a small bud, round or ovoid in shape,
FIG. 6d. CH-IV (Cochlea with hypoplastic middle and apical turns)
arising from the IAC.
cochlea has a normal basal turn, but middle and apical turns are
severely hypoplastic.

Audiological Findings
These patients may demonstrate the full spectrum of hearing
loss. They may have normal, mild or moderate hearing loss
which can be rehabilitated with hearing aids only. Also pure
conductive hearing loss is not uncommon which may benefit
from stapes surgery. In the case of mixed hearing loss the patient
benefit from stapes surgery and hearing aids. Profound hearing
loss is also possible and can be rehabilated with CI and in the
FIG. 6b. CH-II (Cystic hypoplastic cochlea) cochlea has smaller
case of CN deficiency; with ABI. In conclusion CH patients
dimensions with defective modiolus and interscalar septa, but with represent the most interesting group of IEMs regarding clinical
normal external outline. presentation and management.

Management
Decision making in patients with CH may be challenging. They
may present with a range of different thresholds on audiometric
testing; mild-profound sensorineural, conductive or mixed
hearing loss. Decision making about the amplification options
may be difficult, particularly in patients with a hypoplastic
cochlear nerve.
• Some cases of CH (particularly CH-III and CH-IV) may have
pure conductive or mixed hearing loss where the conductive
component is due to stapedial fixation. They may benefit from
stapedotomy. This can be done in childhood and can result in
FIG. 6c. CH-III (Cochlea with less than 2 turns) The cochlea has fewer better oral language development with or without hearing aid
turns (less than 2 turns) with a short modiolus. use, depending on the bone conduction levels.

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404 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

• Patients with mild to moderate SNHL can be habilitated with Sennaroglu and Saatci (9). These represent approximately
hearing aids and have near normal language development. 20% of IEMs. In this anomaly, there is a clear differentiation
• Majority of CH patients have severe to profound hearing loss between cochlea and vestibule. Cochlea is located in its usual
where a CI would be a reasonable option, if they have a cochlear location in the anterolateral part of the fundus of the IAC and
nerve. During surgery, facial nerve malposition is to be expected lacks the entire modiolus and interscalar septa (Figure 7),
due to associated semicircular abnormalities (particularly giving the appearance of an empty cystic structure. External
lateral SCC). In hypoplastic cochlea, the promontory may not dimensions (height and length) of an IP-I cochlea are similar
have the usual protuberance and it may be difficult to identify to normal cases (10). Cochlea is accompanied by an enlarged,
promontory and round window through the facial recess. In dilated vestibule (Figure 7). Vestibular aqueduct enlargement
these situations, an additional transcanal approach may be is very rare. There may be a defect between the IAC and the
necessary to expose the hypoplastic cochlea. cochlea due to developmental abnormality of the CA and
• As the number of turns are smaller with narrower scalae, it is absence of the modiolus and CSF may completely fill the
strongly advisable to use thin and shorter electrodes. Thick and long cochlea.
electrodes may not be inserted fully into the cochlea. Particularly Recurrent meningitis can occur in IP-I patients even prior to
important is CH II which has a potential for CSF leakage. FORM their CI surgery or in their non-operated ear. This is due to
19 (Med El) electrode is developed for these cases. It is 19 mm defective stapes footplate and CSF filling the cochlea. There is
in length and makes one full turn around the hypoplastic and a cystic structure in the stapes footplate which is easily infected
narrow basal turn in addition to providing effective sealing of the during an attack of otitis media. This is very characterstic for
cochleostomy. If a longer electrode is chosen, it cannot be inserted IP-I. Spontaneous CSF fistula and recurrent meningitis can be
up to the silicon stopper in case of gusher in CH-II. seen although less frequently in CH type II. This is because
• Some patients with CH have CA aplasia with cochlear nerve both IP-I and CH-II have endosteal developmental anomaly
aplasia and thus, an ABI would be the best hearing habilitative leading to defective footplate development (4). It is interesting
option. to note that IP-III cases always have a high volume CSF gusher
• Cochlear nerve deficiency is frequently observed in patients during CI surgery but meningitis is very rarely reported in these
with CH. The best option in these cases is to perform CI in the patients (1,11). This is most likely due to the fact that the stapes
side with better developed cochlear nerve or better audiological footplate is normally developed, because in IP-III pathology
findings. If there is limited hearing and language development is in the outer two layers of the otic capsule and endosteum
with CI, an ABI should be considered for the contralateral side. is normal. Therefore, a defect in the footplate in IP-III is very
In certain cases, who are between 2-3 years of age, with barely unlikely.
visible cochlear nerves, CI and ABI can be done simultaneously; All patients with IP-I and recurrent meningitis who have
this is to avoid the loss of valuable time until ABI surgery if normal tympanic membranes but fluid filling the middle ear
the chance of benefit from CI surgery is low. Simultaneous CI and mastoid should have an exploration of the middle ear with
and ABI surgery has been performed in three patients in our special attention to the stapes footplate. Endaural incision was
department. used, and after entering the middle ear, usually a large cyst

6- Incomplete Partition of the Cochlea

Incomplete partition anomalies represent a group of cochlear
malformations, where there is a clear differentiation between
cochlea and vestibule, with normal external dimensions and
various internal architecture defects. Incomplete partitions
constitute 41% of IEMs according to the database of Hacettepe
University Department of Otolaryngology. There are three
different types of incomplete partition groups according to the
defect in the modiolus and the interscalar septa.

Types of incomplete partition groups

a- Incomplete partition type I (IP-I)

This type of incomplete partition anomaly was termed FIG. 7a. Incomplete partition-I; Cochlea (C) without modiolus and
as “cystic cochleovestibular malformation” in 2002 by interscalar septa, accompanied by an enlarged, dilated vestibule (V).

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Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 405

coming from a defect at the stapes footplate was discovered and and diameter) are similar to that seen in normal cases (10).
excised. After excising the cyst, a defect at the oval window Therefore, it is not correct to define this anomaly as a cochlea
was observed, and CSF gusher was encountered. If the defect with 1.5 turns (10). The term “cochlea with 1.5 turns” should
is small it sould be enlarged with a 0.6 mm dimond bur for be used only for CH.
easier manipulation during plugging of the defect. It is also very A recent study on histopathology demonstrated that modiolar
important to keep stapes suprastructure and the ossicles intact defects may be due to high CSF pressure transmission into the
in order to stabilize the fascia in place. inner ear as a result of EVA (14). An enlarged endolymphatic
sac and duct appears to be the only genetic abnormality that is
Management
causing the other abnormalities allowing high CSF pressure to
• Majority of IP-I patients have severe to profound SNHL. be transmitted into the inner ear. This results in a mild dilatation
They are almost always candidates for CI. in the walls of the vestibule. However, no hydropic changes were
• Size of the cochlea is normal. Therefore, straight electrode observed in the endolymphatic space. Depending on the severity
about 25 mm is preferred. Modiolar hugging electrode should and timing of the insult, the pathology may stay at this stage
not be used. FORM 24 (Med El) is developed for these cases. and cause EVA only, or with the transmission of CSF pressure
The length is 24 mm which makes a full turn around the basal into the cochlea, it may cause a spectrum of anomalies ranging
turn. The conical stopper is used to stop CSF leakage around from scala vestibuli dilatation, scala communis, superior (cystic
electrode (12). It is passed through a 2x2 mm fascia and they apex), partial, subtotal and in some cases complete modiolar
are inserted together. Silicon stopper pushes and stabilizes the defects. The high pressure in the SV causes bulging of the ISS
fascia into the cochleostomy. It also keeps it in place. Digisonic upwards. This is a constant finding in all cases, showing that
Classic and Digisonic Evo electrodes (Oticon) are also have a cochlear pathology may be the result of high pressure in the SV
silicon stopper which might be useful for controlling gusher. and that it happened during the developmental phase, otherwise
• In case of gusher, it is most important to stop the CSF leakage high pressure would have fractured the osseous spiral lamina. If
from the cochleostomy. The surgeon should not leave operation there is higher pressure, it is natural to expect more destruction
room without controlling the leakage. After controlling the
leakage, subtotal petrosectomy can be done as an additional
measure. The benefit of subtotal petrosectomy in CI is to seal
the middle ear space from the nasal passage. But if the leakage
is not controlled and continues around the electrode, subtotal
petrosectomy should not be performed. Most important issue is
controlling the leakage from the cochleostomy.
• As it is possible to have CN aplasia in IP-I, some patients
may not be a candidate for CI surgery. Therefore, an ABI is
indicated in IP-I patients with aplastic CN. Four patients with
IP-I and an aplastic CN have received ABI in our department.
• As in CC, an ABI may be indicated on the contralateral side
in case of insufficient progress with CI as a possible indication
FIG. 7b. Incomplete partition-II; Cystic apical part (A) of the cochlea (C).
for ABI (5).

b-Incomplete partition type II (IP-II)

In IP-II, the apical part of the modiolus is defective (Figure 7b).
This anomaly was originally described by Carlo Mondini and
together with a minimally dilated vestibule and an enlarged
vestibular aqueduct (EVA) (Figure 7c) constitute the triad
of the Mondini deformity. The term “Mondini” should be
used only if the above mentioned triad of malformations is
present (1,9,11,13). The apical part of the modiolus and the
corresponding interscalar septa are defective, giving the apex of
the cochlea a cystic appearance due to the confluence of middle
and apical turns. The external dimensions of the cochlea (height FIG. 7c. Incomplete partition-II; enlarged vestibular aqueduct (EVA).

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406 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

at the upper, and possibly the lower part of the modiolus. During condition for the first time, and this characteristic deformity was
CI surgery in IP-II, pulsation observed at the round window is included under the category of incomplete partition deformities
due to third window defect of EVA transmitting CSF pressure for the first time by Sennaroglu et al. (17) in 2006.
into the cochlea. CSF oozing and gusher sometimes observed This anomaly is the rarest form of incomplete partition cases.
in CI surgery in IP-II are due to modiolar defects occurring as a IP-III constitutes 2% of the IEMs in the database in Hacettepe
result of high CSF pressure transmission. University Department of Otolaryngology.
In IP-III cochlear otic capsule around the membranous labyrinth
Audiological Findings
is thinner when compared to that in a normal cochlea. HRCT
These patients do not have a characteristic hearing level, as demonstrates that in IP-III, the otic capsule around the cochlea
their audiometric threshold testing varies from normal to is thin and follows the outline of the membranous labyrinth as if
profound. The hearing loss can be symmetric or asymmetric, it is formed by a thick endosteal layer. Instead of the usual three
but it is usually progressive. It is also possible to have sudden
layers, probably the second and third layers are either absent
SNHL. In addition, there is an air bone gap particularly at low
or very thin. Innermost endosteal layer appears to be thickened
frequencies. Tympanometry is normal in the absence of otitis
without enchondral and outer periosteal layers (4).
media and acoustic reflexes are generally present. Air bone gap
Phelps et al. (16) reported that there is a bulbous IAC, incomplete
in these children is likely to be due to a “third window” effect
separation of the coils of the cochlea from the IAC. Talbot and
from the EVA, and may resemble the audiometric findings of
Wilson (18) later added that the modiolus is absent and there
superior canal dehiscence syndrome.
is a more medial origin of the vestibular aqueduct with varying
Management degrees of dilatation. Sennaroglu et al. (19) reported that in this
• At a young age, these patients may have near normal hearing deformity the interscalar septa are present but the modiolus
and may not require amplification initially. With fluctuations is completely absent. This gives the cochlea a characteristic
and progressive hearing loss, they become candidates for appearance. From an earlier study, the external dimensions of
hearing aid. the cochlea (height and diameter) were found to be similar to
• Usually the progression in hearing loss continues, ultimately the normal cochlea (17), therefore, it is appropriate to include
creating a need for CI at some point in the future. In our IP-III under the incomplete partition anomalies. In addition,
department we have also followed IP-II cases with profound labyrinthine segment of the facial nerve is located almost above
SNHL since birth necessitating CI surgery at 1 year of age. High the cochlea (20) instead of making a gentle curve around the basal
pulsating CSF pressure may be responsible for the progression turn on axial sections. The labyrinthine segment of the facial
of hearing loss. A role for head trauma has been suggested, and nerve is the most superior structure in the temporal bone. Thin
these patients are advised to wear helmets when playing sports otic capsule around cochlea and labyrinth, consisting of only a
and avoiding contact sports completely. As basal part of the thick endosteal layer, may be responsible for this. Tympanic and
modiolus is normal all kinds of electrodes can be used during mastoid segments appear to be in their normal position.
surgery. Six of the 77 patients with IP-II had severe gusher
Audiological Findings
during CI surgery. Oozing is also common in these patients.
Therefore, electrode with silicon stopper is advisable in IP- In IP-III there may be mixed type HL or profound SNHL.
II. FORM 24 makes one full turn around the basal turn and Conductive component may be due to thin otic capsule. Stapes
controls CSF leakage around the electrode. Digisonic Classic
and Digisonic Evo electrodes (Oticon) are also have a silicon
stopper which might be useful for controlling gusher.
• Stapedotomy should not be performed in these cases as air-
bone gap is most probably due to third window effect of EVA.
• As all cases of IP-II have cochlear nerve, ABI is not indicated.

c- Incomplete partition type III (IP-III)

Cochlea in IP-III has interscalar septa but the modiolus is
completely absent (Figure 7d). IP-III cochlear malformation is
the type of anomaly present in X-linked deafness, which was
described by Nance et al. (15) for the first time in 1971. Phelps FIG. 7d. Incomplete partition-III. Cochlea (C) has interscalar septa (IS)
et al. (16) described the HRCT findings associated with this but the modiolus is completely absent.

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Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 407

surgery is contraindicated in this group as it may lead to gusher into account as well. More correct definition of EVA may be
and further SNHL. “vertical and axial width larger than 1.5 mm on the midpoint
between labyrinth and operculum” (Figure 8b).
Management
Audiological presentation and management is similar to that of
Mixed hearing loss gives the impression of stapedial fixation. IP-II.
Stapedotomy results in severe gusher and further
SNHL, and thus, should be avoided.
• Patients with moderate to severe mixed or SNHL can be
managed with hearing aids.
• Patients with severe HL are candidates for CI. Because of
the absent modiolous and large defect at the cochlear base, all
patients with IP-III have severe gusher during CI surgery and
there is a very high chance of electrode misplacement into IAC.
The position of the electrode should be checked intraoperatively
in all cases of IP-III. Modiolar hugging electrodes are to be
avoided in IP-III. FORM 24 electrodes make one full turn
around the cochlear base and also control CSF leakage around
the electrode. If the interscalar septa are thick they reduce
intracochlear volume and a long electrode may be misdirected FIG. 8a. Axial section; Enlarged vestibular aqueduct (EVA) with normal
into IAC. In such a case FORM 19 is advisable. Digisonic modiolus (M) and normal vestibule (V).

Classic and Digisonic Evo electrodes (Oticon) are also have a

silicon stopper which might be useful for controlling gusher but
they are longer than FORM electrodes, and they might go into
the IAC. Spontaneous CSF fistula through the stapes footplate
and recurrent meningitis is very rare in IP-III in spite of high
volume CSF leak during CI surgery. This is most probably due
to normal endosteal development (hence a normal footplate) in
IP-III.
• All IP-III cases have excellent cochlear nerves. Therefore,
ABI is not indicated in this group of incomplete partitions.

7- Enlarged Vestibular Aqueduct (EVA)

FIG. 8b. Coronal section; Enlarged vestibular aqueduct (EVA) with
This describes the presence of an enlarged vestibular aqueduct vertical dimensions (V white arrow) and horizontal dimensions (H, black
(i.e. the midpoint between posterior labyrinth and operculum arrow).
is larger than 1.5 mm) in the presence of a normal cochlea,
vestibule and SCCs (Figure 8a). Difference between EVA and 8- Cochlear Aperture Abnormalities
IP-II is that cochlea and vestibule are completely normal on
The CA, cochlear fossette, or bony cochlear nerve canal
HRCT and MRI.
transmits the cochlear nerve from the cochlea to IAC. This
In a previous study EVA is thought to be responsible for
can be visualized in the mid-modiolar view as well as coronal
the transmission of CSF pressure into the inner ear causing
sections on HRCT (Figure 1a).
progressive or sudden SNHL (4). It appears to be due to a
genetic defect. But progressive SNHL is a result of a third The CA is considered hypoplastic (Figure 9a) if the width is less
window phenomenon. than 1.4 mm (20). The CA is considered to be aplastic when the
Classically EVA is described when the midpoint between canal is completely replaced by bone or there is no canal on
posterior labyrinth and operculum is larger than 1.5 mm on mid-modiolar view (Figure 9b).
axial sections. In our department, we have made the observation CA abnormalities may be accompanied by a narrow IAC on
that EVA can be observed in a number of successive axial HRCT (Figure 9c). The IAC is considered narrow if the width
images. It may therefore, not be correct to evaluate EVA only of the midpoint of the IAC is smaller than 2.5 mm. Narrow
on axial sections. We have to take vertical dimension of EVA IAC can accompany other malformations or with a normal

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408 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

cochlea. In cases of narrow IAC, MRI should be obtained to

demonstrate if CN is normal, aplastic or hypoplastic. Axial and
sagittal oblique high T2 weighted images (i.e. CISS, Fiesta etc.)
images are necessary for this purpose. In CN aplasia, no nerve
can be identified in the anterior inferior part of the IAC.
On axial section CN is followed until modiolus (Figure 9d).
On sagittal oblique MR sections, four distinct nerves can be
visualized in the IAC (Figure 9e). CA aplasia is typically
accompanied by cochlear nerve aplasia. CN may be hypoplastic
(Figure 9f, 9g) or aplastic when CA is hypoplastic. CA hypoplasia
FIG. 9d. On axial section cochlear nerve (CN) is followed until modiolus.
and aplasia can also be observed in a normal cochlea.

FIG. 9a. Hypoplastic bony canal for cochlear nerve (BCNC).

FIG. 9e. On sagittal oblique MR sections, four distinct nerves can be

visualized in the IAC.
FN: facial nerve; CN: cochlear nerve; SVN: superior vestibular nerve; IVN: inferior
vestibular nerve

FIG. 9b. Aplastic bony canal for cochlear nerve (BCNC).

FIG. 9f. Hypopastic cochlear nerve (CN), axial view.

FIG. 9c. Bilateral narrow internal auditory canal (IAC) accompanying

bilateral absent cochlear nerve. FIG. 9g. Hypoplastic cochlear nerve (CN), saggital oblique view.

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Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations 409

Audiological Findings CN and superior and inferior vestibular nerves in the IAC. In
Severe to profound SNHL is usually present. As the cochlea cases of CC CVN enters the cavity without separating into
is normal, otoacoustic emissions (OAE) may be present and individual nerves (Figure 9j). With radiological precision at the
the child may pass newborn hearing screening if automated present time, it is impossible to determine the cochlear fiber
ABR is not obtained. Their hearing loss is typically discovered content in the CVN but if the size is 1.5-2 times as much as the
later on in childhood based on the family’s concerns of lack of ipsilateral FN or similar to contralateral normal CVN it can be
sound awareness and language development. If the newborn accepted as normal.
screening protocol involves OAE and automated ABR, this
malformation can be diagnosed during infancy. Diagnostic
audiological evaluation will reveal profound hearing loss.

Management
Hearing aids usually do not provide sufficient amplification
in patients with CA hypoplasia and aplasia. In patients with
bilateral hypoplastic CA with hypoplastic cochlear nerve,
hearing aid trial is necessary. If this does not provide adequate
functional hearing, these patients usually become candidates for
CI. The family should be counseled that if CI does not provide
sufficient hearing in terms of auditory perception, contralateral
ABI may be necessary to achieve improved audiologic and
FIG. 9h. Aplastic cochlear nerve, axial view.
language outcomes.
In CA aplasia, ABI is indicated as a first-line therapy.

II- COCHLEAR NERVE ABNORMALITIES

The classification of CVN is also important in the management
of IEMs.

1- Normal cochlear nerve (CN)

It is important to trace the CN until it enters the cochlea on
lower axial sections passing through the IAC (Figure 9d). On
parasaggital sections, there is a separate CN located in the
anterior inferior part of the IAC, entering the cochlea (Figure 9e).
The size of the cochlear nerve is similar in size when compared FIG. 9ı. Aplastic cochlear nerve (CN), saggital oblique view.
with the CN on the contralateral normal side. According to
Casselman et al. (21) on parasaggital view the size of the CN is
similar or slightly larger than the ipsilateral FN.

2- Hypoplastic CN
There is a separate CN but the size is less than the contralateral
normal CN or ipsilateral normal facial nerve (Figure 9f, 9g).

3- Absent CN
There is no nerve in the anteroinferior part of the IAC (Figure
9h, 9ı). This is definitely present in cochlear aplasia. It can also
be seen in CA hypoplasia and aplasia.

4- Normal CVN
Normally cochlear and vestibular nerves originate at the
brainstem together forming the CVN. CVN then separates into FIG. 9j. Cochleovestibular nerve (CVN) entering common cavity (CC).

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410 Sennaroğlu and Bajin. Classification and Current Management of Inner Ear Malformations

5- Hypoplastic CVN Management

If CVN is smaller than contralateral CVN or ipsilateral FN, it After a hearing aid trial, initial approach is a trial with CI.
can be accepted as hypoplastic (Figure 9k). CVN hypoplasia is If the progress of the patient is insufficient, it is advisable to
particularly important in CC. progress with ABI. This procedure is suitable if the initial CI
surgery was done around the age of 6 months; ABI can then
6- Absent CVN be done between 1-1.5 years old with acceptable language
In case of Michel deformity with absent IAC, CVN is also development. However if the patient comes in around the age
absent (Figure 9l). Only facial nerve can be identified. of 2, the time delay between two surgeries increases hence the
outcome of ABI decreases. Therefore, simultaneous CI and
Audiological Findings
ABI maybe a better option in selected cases. In our department
Severe to profound SNHL is usually present. As the cochlea is simultanous CI and ABI have been performed in three such
normal, OAE may be present and the child may pass newborn cases.
hearing screening if automated ABR is not obtained. The
amount of cochlear nerve fibers determine the hearing level CONCLUSION
and management strategy. However, for the present time there’s
no spesific auditory profile characteristics for different CVN IEMs is a special group of patients. Unfortunately, until
subgroups. It’s advisable to evaluate the candidate with the full recent years the term “Mondini” has been used to describe
audiological test battery. many different anomalies. There are variety of IEMs and
they all present in a different way. As can be seen easily each
group has different characteristics in terms of presentation,
radiology, hearing and surgical findings. Proper classification
is very important in the management of IEMs. If the anomaly
is correctly classified, appropriate treatment can be decided
more correctly. Finally, proper classification also will create
a common language around the world, where clinicians can
understand each other better.

Financial Disclosure: No financial disclosure was declared by

the authors.

Conflict of Interest: No conflict of interest was declared by

the author.
FIG. 9k. A hypoplastic cochleovestibular nerve (CVN) entering common
cavity (CC). Size of the facial nerve (FN) is much larger than the CVN.
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