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The Safety of Flexible Endoscopic Evaluation of

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International Journal of Pediatric Otorhinolaryngology (2006) 70, 591596

www.elsevier.com/locate/ijporl

Endoscopically guided placement of prefabricated

cochlear implant electrodes in a common cavity
malformation
Spiros Manolidis *, Ross Tonini, Jaclyn Spitzer

Department of OtolaryngologyHead and Neck Surgery, Columbia University,

180 Fort Washington Street, HP812, New York, NY 10032, USA

Received 14 March 2005; accepted 9 July 2005

KEYWORDS Summary
Cochlear implant;
Cochleovestibular Objective: To devise a safe and effective method of optimal customized electrode
malformation; placement in the common cavity of children with cochleovestibular malformations.
Common cavity; Methods: Specialized electrodes were manufactured on the basis of three-dimen-
Custom-made cochlear sional data obtained from the high resolution computed tomography (HRCT) scans of
implant electrode; the temporal bones of these two children. Electrode positioning was achieved with
Direct electrode direct endoscopic view of the cavity utilizing a three-hole common cavity technique.
positioning Results: Optimal electrode positioning in apposition to the medial neuroepithelium in
the common cavity was verified visually intraoperatively. Postoperatively, minimal
stable electrical current levels were found to be required.
Conclusions: Custom-designed electrodes have the potential to offer improved
results in children with common cavity malformations. Intraoperative direct position-
ing may further improve these results.
# 2005 Published by Elsevier Ireland Ltd.

1. Introduction duct, and subtle partitioning defects, among others.

Improvements in diagnostic imaging have been able
to specify subtle anatomic abnormalities of the
inner ear in as many as 20% of patients with profound
sensorineural hearing loss at birth [1,2]. Most of
these congenital malformations are mild and rela-
tively common, such as enlarged vestibular aque-
* Corresponding author. Tel.: +1 212 305 5820;
fax: +1 212 305 2249.
E-mail address: sm2397@columbia.edu (S. Manolidis).
With further improvement in diagnostic imaging,
intracochlear anatomic variations and physiologic
changes will begin to be detectable [3,4]. A very
small proportion of infants with congenital profound
sensorineural hearing loss have severe cochleoves-
tibular abnormalities defined as a common cavity
with hypoplasia of the cochleovestibular nerve or
complete aplasia of the cochlea and/or vestibule or
the cochleovestibular nerve.
In one large series of congenital profound sensor-
ineural hearing loss, these infants represented 2% of

0165-5876/$ see front matter # 2005 Published by Elsevier Ireland Ltd.

doi:10.1016/j.ijporl.2005.07.004
592 S. Manolidis et al.

the total sample [5]. These patients present a chal-

lenge in the surgery for cochlear implantation.
Anomalous facial nerves and CSF leak are common-
place [6,7]. In addition, the programming of such
implants requires frequent adjustments due to fluc-
tuation of electrical current requirements [2,6,8].
New cochlar implant electrode designs may over-
come some of these problems. At the same time,
establishing the presence of residual stimulable
neuroepithelium in a severely malformed cochlea
is essential in deciding which side to implant, along
with data from parasagittal MRIs with volumetric
analysis of the internal auditory canals. It follows
that electrode placement in proximity to the iden-
tified neural tissue is highly desirable.
The purpose of this report is to highlight a tech-
nique for the precise placement of a custom-
designed common cavity electrode against the med-
ial neuroepithelia of such malformed cavities.
Fig. 1 HRCT of the left temporal bone in our first
patient. Common cavity malformation with hypoplastic
2. Materials and methods internal auditory canal.

Two patients with severe cochleovestibular malfor-

mations diagnosed by high resolution computed temporal bone HRCT (MED-EL). The patient under-
tomography (HRCT) and MRI of the temporal bone went a cochlear implantation at age of 8 months.
form the basis of this report. Clearance by the
Institutions Review Board was obtained prior to 2.2. Case 2
submitting this data.
The second patient was a healthy girl born following
2.1. Case 1 a normal pregnancy and uncomplicated delivery.
She was first diagnosed with profound sensorineural
The first patient was a healthy 1-month-old girl,
product of a normal pregnancy and an uncomplicated
delivery; she failed neonatal screening by otoacous-
tic emissions and auditory brain stem evoked poten-
tials. Assessment by auditory steady state evoked
potentials produced no stimulation thresholds on
the left but a threshold present at low and mid
frequencies was produced at 125 db on the right.
Routine laboratory screening, Connexin-26 testing
and medical genetics revealed no abnormalities.
Further evaluation by high resolution computed
tomography of the temporal bone revealed a com-
mon cavity on the left and a common cavity with a
vestigial part of the cochlear basal turn on the right
(Figs. 1 and 2). High resolution MRI of the temporal
bone revealed a severely hypoplastic internal audi-
tory canal on the left. On the right there was a slightly
larger IAC and the possibility of a cochlear nerve,
albeit of much diminished diameter (Figs. 35). A
cochlear implant was recommended for this child
after she was approved through a process similar
to what has been described previously [9,10]. A Fig. 2 HRCT of the right temporal bone in our first
custom-made common cavity electrode was manu- patient. Common cavity malformation with presence of
factured based on measurements of the right a fraction of the basal turn of the cochlea.
Endoscopically guided placement of prefabricated cochlear implant electrodes
Fig. 3 Parasagittal T2 weighted MRI image through the
internal auditory canal in our first patient shows presence
of cochleovestibular nerve, albeit of diminished diameter.
hearing loss by behavioral audiometry at age 15
months. Profound bilateral sensorineural hearing
loss was confirmed with auditory steady state
evoked potentials that revealed absent thresholds
on the right and thresholds present in the lower
frequencies at 125 db. Routine serologic screening,
Connexin-26 testing and medical genetics evalua-
tion disclosed no abnormalities. A HRCT of the tem-
Fig. 4 Parasagittal T2 weighted MRI image through the
internal auditory canal in our first patient shows severe
internal auditory canal with absence of the cochlear
nerve.
593
Fig. 5 Axial T2 weighted MRI image displaying a common
cavity on the left and a common cavity with a fraction of a
basal turn of the cochlea on the right.
poral bones revealed a common cavity malformation
on the right with a severely hypoplastic IAC, while
the left had a lesser degree of malformation with a
dilated internal auditory canal (Fig. 6). MR of the
temporal bones corroborated these findings showing
a severely hypoplastic IAC with absent cochlear
Fig. 6 HRCT of the temporal bone on the second patient
displaying a left-sided dilation of the internal auditory
canal with a wide communication to the common cavity.
Incidentally, this patient did not experience a CSF leak
during the implantation.
594
Fig. 7 Axial T2 weighted MRI of our second patient. Wide
IAC and common cavity on the left, common cavity and
severe IAC hypoplasia were present on the right.
nerve on the right, dilated IAC on the left and
possibly a hypoplastic cochlear nerve (Fig. 7). This
patient was evaluated and approved for a cochlear
implant as per evaluation protocols previously
described [9,10]. She underwent a cochlear implant
with a custom electrode as in the first patient.
Fig. 8 Endoscopic view of the common cavity during
electrode positioning. Electrode base pairs can be seen
abutting the neuroepithelium on the medial aspect of the
common cavity. Top part of the photograph is superior;
bottom part of the photograph is inferior.
S. Manolidis et al.
Fig. 9 Postoperative axial high resolution CT scan of the
implanted common cavity, demonstrating proper medial
positioning of the custom electrode.
For both patients, a double posterior labyrinthot-
omy technique was used to place the electrode in
the right common cavity as has been previously
described [11]. A third labyrinthotomy with a drill
bit appropriate for a thin endoscope (2 mm dia-
meter) was placed above the first two labyrintho-
tomies. The electrode was positioned under direct
visualization against the medial neuroepithelium.
Although the endoscope was useful in positioning
the electrode optimally, full panoramic visualiza-
tion of the cavity is not possible due to field of view
restriction for current endoscopes. Hyaluronic acid
was used to fill the common cavity, improve visua-
lization with the endoscope and prevent minor
bleeding with insertion. Small adjustments with
positioning were made by gently rotating the prox-
imal portion of the electrode to achieve optimal
positioning (Fig. 8).
At the time of this report both children are 4
months post-implant. There have been no compli-
cations from the surgery. Initial programming took
place 18 days after implantation. Voice detection
awareness levels were achieved in both infants,
and there has been no requirement for re-adjust-
ment of thresholds since initial programming
(Fig. 9).
3. Discussion
There are 20 reported cases of common cavity
implantation in the literature [12]. These patients
continue to present a challenge for cochlear
implantation. Evaluation by MR imaging in such
Endoscopically guided placement of prefabricated cochlear implant electrodes
children is essential to rule out aplasia of the
auditory nerve. Oblique sagital images, perpendi-
cular to the IAC may provide the best cross sec-
tional views for determination of the presence and
degree of malformation of the cochlear nerve [13].
In these cases auditory brainstem response (ABR)
testing were not adequate in providing information
as to the presence of thresholds. Promontory sti-
mulation ABR has been advocated for determining
the presence of a functioning nerve, but this
method can also be unreliable [14,15]. We used
auditory steady state evoked potentials (ASSR) to
attempt to determine thresholds in both children
in objective fashion. The electrophysiologic results
coincided with the results of imaging studies, mak-
ing the choice of side for implantation an easy one.
However, in an instance where the imaging studies
show symmetric malformations, ASSR may be the
only way to determine the functional aspect of a
cochlear nerve.
The surgical aspects of cochlear implantation in
common cavity malformations have been previously
reported in the literature [2,5,6,8,11,12,1618].
The surgical technique of a double labyrinthotomy
was chosen for our patients as it is perfectly suited
for the placement of the new specialized elec-
trode.18 The specialized electrode were manufac-
tured on measurements of maximum internal
diameter of the common cavity on the HRCT of
the temporal bone. For positioning purposes, the
electrode has an inactive distal carrier tip so that it
can be threaded out of the cavity from the distal
labyrinthotomy. To date, 12 such implants have been
performed worldwide in common cavity malforma-
tions [19]. Outcomes using these implants have not
been published, but appear to be encouraging. In
our patients, we applied a direct endoscopic posi-
tioning of the electrode through a third labyrinthot-
omy of appropriate diameter for a thin rigid
endoscope. We have not observed fluctuating
thresholds, which are typically encountered in
patients with common cavity malformations who
have been implanted though this is a short period
of follow-up time. It is too early to determine
success in terms of language acquisition, but it
appears that these two children are progressing
on par with their congenitally deaf cochlear implant
age peers.
4. Conclusion
Custom-designed electrodes offer new possibili-
ties for rehabilitation in congenital cochlear mal-
formations. Direct positioning of the custom-
designed electrode against the neuroepithelial
595
elements of the malformed cochlea under endo-
scopic guidance is possible and may offer an addi-
tional advantage.
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