Otology & Neurotology
38:e345e353 2017, Otology & Neurotology, Inc.
Cochlear Implantation in Otosclerosis: Surgical and Auditory
Outcomes With a Brief on Facial Nerve Stimulation
Ashish Vashishth, Andrea Fulcheri, Gianluca Rossi, Sampath Chandra Prasad,
Antonio Caruso, and Mario Sanna
Department of Otology and Skull Base Surgery, Gruppo Otologico, Piacenza, Rome, Italy
Objectives: 1) To review the surgical and auditory outcomes
in patients of cochlear implantation in otosclerosis. 2) To
review complications and postimplantation facial nerve
stimulation (FNS). 3) To compare the auditory outcomes
between patients displaying cochlear ossification to the
nonossified ones.
Study Design: Retrospective study.
Setting: Quaternary Otology and Skull base surgery center.
Subjects and Methods: Charts of 36 patients (38 ears) with
otosclerosis undergoing cochlear implantation were reviewed
from the cochlear implant database. Demographic features,
operative findings, auditory outcomes, and postimplantation
FNS were analyzed. Operative findings included extent of
cochlear ossification, approach (posterior tympantomy/subto-
tal petrosectomy), electrode insertion (partial/complete, scala
tympani/vestibuli), and complications. All the patients under-
went implantation using straight electrodes. Auditory out-
comes were assessed over a 4-year follow-up period using
vowel, word, sentence, and comprehension scores. Patients
were divided into two groups (with and without cochlear
ossification) for comparison of auditory outcomes.
Otosclerosis is a progressive temporal bone dysplasia
characterized by bony resorption, vascular proliferation,
cavitation, and sclerotic new bone formation (1). Cochle-
ar histological changes in otosclerosis range from
endolymphatic flow obstructions, spiral ligament degen-
eration and hyalinization, vascular obstruction and de-
crease in endocochlear potentials (2), with correlation
between the extent of hyalinization to degree of sensori-
neural hearing loss (3,4). Advanced otosclerosis is char-
acterized by sensorineural hearing loss and reduced
speech discrimination scores that is amenable to treat-
ment by cochlear implantation (5).
Address correspondence and reprint requests to Ashish Vashishth,
M.S., D.N.B., Department of Otology and Skull Base Surgery, Gruppo
Otologico, Piacenza, Rome, 29121 Italy; E-mail: drashishvashishth@
gmail.com
All the authors take responsibility of the contents of the article.
This article has been sponsored by the Mario Sanna Onlus Foundation.
The authors disclose no conflicts of interest.
DOI: 10.1097/MAO.0000000000001552
e345
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Results: The mean age and duration of deafness of patients
was 59.72 and 28.9 years respectively. Twenty-three of 38
ears had cochlear ossification, with exclusive round window
involvement in 60% of the patients, with the rest having
partial or complete basal turn ossification. 36.8% ears
underwent subtotal petrosectomy for cochlear ossification.
One patient underwent scala vestibuli insertion and two had
incomplete electrode insertion. Patients with no ossification
had no intra or postoperative complications. One patient had
bilateral FNS managed by alterations in programming
strategy. Auditory outcomes in patients without any ossifica-
tion were better than in patients with ossification, though
statistically insignificant in most parameters.
Conclusion: Cochlear implantation in otosclerosis provides
good auditory outcomes, despite high incidence of cochlear
ossification. Patients of FNS can be managed by alterations
in programming strategy, without affecting auditory outcomes.
Key Words: Cochlear implantationFacial nerve stimulation
Ossified cochleaOtosclerosisScala vestibuli.
Otol Neurotol 38:e345e353, 2017.
Despite widespread improvements in cochlear implan-
tation results in varying indications and clinical scenarios
over the years, otosclerosis as a pathology poses unique
challenges. Intraoperative difficulties range from ossifi-
cation and the need for drilling to locate cochlear lumen,
possible false tract and difficult insertions due to cochlear
demineralization and obliteration and incomplete inser-
tion of electrode array (6). Postoperative considerations
can develop because of altered bone properties caused by
progressive bone remodeling leading to altered current
distribution, hence causing higher current levels or max-
imum comfort levels (MCL), increased impedances (7
10), and facial nerve stimulation (712), necessitating
changes in cochlear implant fitting strategies (8,13).
Otosclerosis has been defined as one of the etiological
factors having a negative correlation with postimplanta-
tion speech performance scores, independent of compli-
cations with electrode insertions (14), with a requirement
of frequent alterations of electrical stimulation levels
(T and C scores) postimplantation for 24 months at
least (15).
e346 A. VASHISHTH ET AL.

The current study was conducted to review the patients sentence recognition, and common phrase comprehension)
of otosclerosis who underwent cochlear implantation to with monitored live voice through sound field at a level of
analyze the surgical and postoperative considerations, 70 dB sound pressure level. The protocol used is described
complications, and assess the postimplantation auditory elsewhere (19).
Auditory outcomes were compared between patients dis-
outcomes with a careful insight into evaluation and playing cochlear ossification and the ones without ossification.
management of facial nerve stimulation, including alter-
ations in postimplantation fitting strategies.
RESULTS
METHODS
The mean age of patients was 59.72  12.53 years with
Medical records were analyzed of cochlear implant recipients
21 females and 15 males. The age at implantation ranged
to identify patients with confirmed otosclerosis who underwent
cochlear implantation. Thirty-six patients with confirmed from 25 to 86 years. Twenty-one right ears and 17 left
otosclerosis underwent cochlear implantation until 2016 with ears were operated.
two patients receiving bilateral implants, hence leading to a total The mean duration of deafness was 28.9  13.73 years
of 38 ears. All patients had a 4-year minimum follow-up period and ranged from 1 to 66 years. Figure 1 shows distribu-
postimplantation except one who was implanted in the last year. tion of age at implantation and distribution of duration of
The same patient is excluded from audiological outcomes but is deafness in years.
considered in surgical data. Hence, the surgical dataset consists Twenty-three patients had previous stapedotomies in
of 38 ears (36 patients), whereas the audiological dataset consists the operated ear (cochlear implant side) and one patient
of 37 ears (35 patients). For patients with bilateral implants each had a bone anchored hearing device placed for hearing
ear was analyzed separately.
rehabilitation before cochlear implantation. All stapedot-
One patient with osteogenesis imperfecta, despite having
previous stapedotomy was excluded from the analysis due to omy prosthesis in the current series were Teflon only,
difference in pathology. hence no separate MRI considerations were applied
Patient records were analyzed to evaluate demographic before preoperative scanning.
features, duration, and onset of hearing loss, history of previous
surgical procedures for hearing rehabilitation such as stapes
surgery or bone anchored hearing aids. Preoperative high-
resolution computed tomography (HRCT) scans were obtained
for all the patients and magnetic resonance imaging (MRI)
was obtained to ascertain patency of cochlear lumen when
evidence or doubt of obliteration or ossification was observed
on HRCTs. Operative records were analyzed to ascertain details
of the implantation procedure, particularly to identify instances
when cochlear ossification was encountered necessitating dril-
ling to obtain cochlear lumen, the extent of drilling, approach
used (posterior tympanotomy/subtotal petrosectomy), the
instances of scala vestibuli insertion, extent of electrode array
insertion (complete/partial), and intraoperative complications if
any such as false tract insertions. The technique, indications,
and follow-up of subtotal petrosectomy (STP) patients relevant
to cochlear implantation can be referred to from our previous
publications (6,16,17). Salient features of STP with relevance to
cochlear implantation are blind sac double-layered closure of
the external auditory canal, removal of all possible mastoid cell
tracts after canal wall down mastoidectomy, total removal of
mucosa from middle ear cleft, removal of entire medial canal
skin and tympanic membrane/ossicles, anterior canalplasty, and
drilling of anterior bony annulus, eustachian tube mucosa
removal/coagulation and its obliteration with periosteum, car-
tilage and bone wax, and obliteration of cavity by fat harvested
from abdomen.
Postoperative audiological parameters observed were vowel,
word, sentence, and comprehension measured over serial time
points till 4 years, beginning from activation. Note was made of
revision surgeries due to device failures or implant extrusions
if any. Postimplantation facial nerve stimulation (FNS) was
carefully analyzed and graded as per the grading system
proposed by Kelsall et al. (18) and alterations in programming
strategies needed to alleviate the same were reviewed.
Postoperative auditory performances were evaluated in FIG. 1. (A) Demonstrating the distribution of age at implantation
the auditory-only condition in both closed-set (vowel identifi- and (B) demonstrating distribution of duration of deafness in years
cation) and open-set formats (bisyllabic word recognition, in the study population.

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 COCHLEAR IMPLANTATION IN OTOSCLEROSIS e347

Surgical Results of false tract insertion of electrode array through the

Twenty-three of 38 (60.5%) ears in the study group had
some degree of cochlear ossification observed during
surgery ranging from round window (RW) to complete
basal turn ossification. Fourteen of 38 (36.84%) ears
were implanted using the STP approach following evi-
dence of significant cochlear luminal obliteration or
ossification preoperatively on the HRCT or MRI scans
to obtain a larger and unhindered access. A posterior
tympanotomy or extended posterior tympanotomy ap-
proach was used in the rest of patients with no or minimal
cochlear ossification. Diamond burr was used to drill in
the direction of basal turn scala tympani to obtain co-
chlear lumen. Failure to obtain scala tympani lumen till
the beginning of ascending basal turn was used as an
indication for scala vestibuli insertion. Implants with
straight electrode arrays were used in all the patients
from Cochlear, Medel, and MXM.
Table 1 summarizes the surgical details of patients
exhibiting cochlear luminal obliteration/ossification.
Complications
Table 1 reviews the complications in patients with
ossification. All the patients in the study group had a
successful scala tympani insertion except one patient
in whom scala vestibuli insertion was done due to
near-total ossification of the basal turn. One incidence
vestibule into the superior semicircular canal was
observed intraoperatively and was identified using a
perioperative computed tomography (CT) scan, with
correction of the same and re-insertion into scala
tympani, confirmed radiologically.
Three patients underwent revision implantation, two
due to device failure (one traumatic and other idiopathic)
and another due to implant extrusion.
Only one patient in the study cohort had postimplan-
tation (FNS) (bilateral FNS). Since the case was compli-
cated by FNS and implant extrusion, a short summary is
provided below with the mention of post-FNS program-
ming details.
Patient Details
A fifty-one-year-old male patient with otosclerosis
underwent right-sided cochlear implantation using the
STP approach for modiolar spongiosis using the slim
straight electrode array. Electrode 9 was observed to have
high impedance and was nonfunctional on activation.
One year later, sequential left-sided cochlear implanta-
tion was performed using the STP approach due to RW
obliteration and full scala tympani insertion was done
after drilling to open the scalar lumen using the same
implant type. The right implant exhibited extrusion
through the postaural skin and was subsequently removed

TABLE 1. Elaboration of patients with otosclerosis who displayed cochlear ossification with mention of electrode insertions, scala
inserted into, number of electrodes outside the cochlea, surgical approach used, and extent of ossification
Scala
S.no Age Sex Side Approach Extent of Ossification Inserted Into Extent of Insertion Complications If Any

1 51 M L STP RWO ST Full FNS

2 51 M R STP RWO ST Full Extrusion-revision and FNS
3 66 F R PT RWO ST Full None
4 52 M R PT RWO ST Full None
5 66 M R STP RWO ST Full CSF gusher
6 55 F L PT RWO ST Full None
7 75 F L STP RWO ST Full None
8 61 F R STP Extensive RWO ST Full None
9 38 M L STP RWO partial BTO ST Full None
10 65 M R PT Partial RWO ST Full None
11 53 F R PT RWO ST Full None
12 65 M R STP RWO ST Full None
13 43 M R PT RWO ST Full None
14 71 F L STP RWO partial BTO ST Full None
15 71 M L STP Complete BTO, no ST lumen SV Full None
16 62 M L PT RWO partial BTO ST Full None
17 82 F L STP Extensive RWO ST Full None
18 62 F L STP RWO ST Full None
19 65 M L STP RWO BTO ST Partial, two electrodes out None
20 25 F L PT RWO ST Partial, two electrodes out Device failure and revision
21 50 M L STP RWO ST Full False tract insertion into
vestibule and SSC
22 66 F R PT RWO ST Full None
23 45 F R STP RWO BTO ST Full None

BTO indicates basal turn ossification; FNS, facial nerve stimulation; PT, posterior tympanotomy/extended posterior tympanotomy; RWO,
round window ossification; SSC, superior semicircular canal; ST, scala tympani; STP, subtotal petrosectomy; SV, scala vestibuli.

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e348 A. VASHISHTH ET AL.

with transected array left in the cochlear lumen. One year
later, reimplantation was performed in the right ear with
full insertion using a slim straight electrode. The patient
exhibited signs of FNS on both the sides with grade III on
the left side and grade II on the right side which was
alleviated by switching off electrode 18 on the right
side and electrodes 13, 14, and 15 on the left side.
No significant reduction in speech performance was
observed by switching off electrodes on either side
and no new FNS was noted postprogramming changes.
Auditory Outcomes
Figure 2 displays the serial charting of pre- and
postoperative vowel, word, sentence, and comprehension
scores observed 1, 3, 6, 9, 12, 18, 24, 36, and 48 months
postsurgery. Table 2 demonstrates the scores over
the same follow-up period separately for patients with
cochlear ossification and the ones with no ossification.
All the patients remained motivated implant users and
achieved telephonic conversation.
All the audiological scores demonstrated statistically
significant improvements over the preoperative values
with a p value <0.05 at all time points of follow-up.
Figure 3 displays the comparison of postoperative
hearing outcomes between the patients having cochlear
ossification with those without. Though patients without
any ossification did uniformly better in all the audiologi-
cal parameters with a trend toward significance, the
difference could not reach a p value < 0.05, except in
sentence scores at 24 months ( p 0.03) which were
significantly better in the group without ossification.
Figure 4A and B displays stratification of maximum
word and sentence scores achieved with respect to dura-
tion of deafness in both the ossified and no-ossified
groups.
DISCUSSION
The benefits of cochlear implantation in the rehabili-
tation of severe sensorineural hearing loss in otosclerosis
are now well established and supported by many studies
(711,2026). However, both surgical and auditory out-
comes reported have been heterogeneous, coupled with a
lack of elaboration on operative considerations, particu-
larly in obliterated and ossified cochlear turns. Table 3
presents a brief comparison of the previously published
series of cochlear implantation in otosclerosis in terms of
surgical and auditory results.
Demographics
Long duration of deafness and a higher age at implan-
tation have previously been negatively correlated with
postimplantation speech performance scores (27,28).
However, Matterson et al. (23) in their review of otoscler-
otic patients undergoing cochlear implantation concluded
that the age at implantation and duration of deafness only
make a significant difference in early postoperative speech
outcomes and not in long-term and recommended the
implantation of worst hearing ear despite longer duration
of deafness. In the current study, the worst hearing ear was
always chosen for implantation, given the conditions for
electrode placement were satisfied on radiology. The mean

FIG. 2. Demonstration of serial charting of vowel (A), word (B), sentence (C), and comprehension scores from preoperative period to 1, 3,
6, 9, 12, 18, 24, 36, and 48 months postimplantation. mo indicates months; pre, preoperative.

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 COCHLEAR IMPLANTATION IN OTOSCLEROSIS e349
TABLE 2. Vowel, word, sentence, and comprehension scores over a 48-month follow-up period from preoperative levels for patients
with and without cochlear ossification
Vowel Scores
Follow-up Intervals Preoperative 1 mo 6 mo 12 mo 24 mo 36 mo 48 mo

Ossified 42.1 71.4 78.3 84 75 73 74.2

Nonossified 53 86.5 90.7 80.3 82.6 81.1 81.9
Word Scores
Follow-up Intervals Preoperative 1 mo 6 mo 12 mo 24 mo 36 mo 48 mo

Ossified 13.5 30.7 44 56.5 53.4 52.4 52.6

Non-ossified 17.8 51.4 65 73 76.4 73.2 72.4
Sentence Scores
Follow-up Intervals Preoperative 1 mo 6 mo 12 mo 24 mo 36 mo 48 mo

Ossified 14.9 34.3 47.1 55.4 54.7 59.2 61.2

Non-ossified 26.3 71 79.5 82.9 88 85.5 84.2
Comprehension
Scores
Follow-up Intervals Preoperative 1 mo 6 mo 12 mo 24 mo 36 mo 48 mo

Ossified 20.4 35.2 48.6 70.4 71.8 75.9 75.9

Nonossified 25 68.3 79.1 91.6 90 85 80.8

Scores are provided in percentages.

m indicates months.

age at implantation was 59.7 years, ranging from 25 to
86 years. The mean duration of deafness was 28.9 years,
ranging from 1 to 66 years.
Surgical Considerations
The HRCT scan of the temporal bone plays a vital role
in the preoperative evaluation of otosclerotic patients
planned for cochlear implantation (5,11,25). It lacks
sensitivity as the presence of intraoperative ossification
has been reported frequently even in the absence of any
ossification on the scan (9), but has high specificity with
higher grades of otosclerotic changes on CT (11,25)
associated with difficulties in electrode insertion and
even postimplantation FNS (11,25). Patients with scle-
rotic changes on CT with ossification noted intraoper-
atively displayed some degree of difficulty in electrode
insertion in the current study. A correlation of CT with
postoperative FNS, however, was not noted as only a

FIG. 3. Graphs depicting comparison of auditory outcomes between ossified and nonossified patients. A, Vowel score. B, Word score. C,
Sentence score. D, Sentence comprehension.

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e350 A. VASHISHTH ET AL.
FIG. 4. A, % improvement in maximum word score attained. B, %
improvement in maximum sentence score attained.
single patient had postoperative bilateral FNS (discussed
later).
The proportion of patients in the literature displaying
varying degrees of intraoperative ossification requiring
drilling to obtain scalar lumen ranges from 5% (9) to 51%
(11) (Table 3), whereas it was 60% (23/38) in the current
series. A referral bias could be the reason for a much
higher percentage of ossification cases in the otosclerotic
cohort. In cases of ossification, 16 of 23 ears (69.55%)
had obliteration or ossification limited to the RW region,
6 of 23 ears had extensive partial basal turn ossification,
and 1 patient had complete basal turn ossification,
requiring scala vestibuli insertion.
On suspicion or confirmation of extensive bony
demineralization or basal turn ossification on CT, the
surgical approach used for implantation can be either
posterior tympanotomy or STP. Though limited round
window/basal turn ossification can be managed by an
extended posterior tympanotomy or an anterior posterior
combined transcanal approach (29), over the years, we
have developed a low threshold for STP for patients in
whom significant promontorial drilling is anticipated for
obtaining cochlear lumen (6,16,17). Though developed
and used initially for implanting radical or infected
cavities (6), in our opinion, a wider exposure to the
obliterated round window is one of the most important
indications for the procedure.
In our series, it is a safe approach with comparable
complication rates as closed cavity surgeries, while per-
mitting wider exposure for visualization, drilling, and
instrumentation, all of which cannot be over emphasized.
Two of the patients with RW or basal turn ossification
had incomplete electrode insertion in the current study,
Otology & Neurotology, Vol. 38, No. 9, 2017
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with two electrodes outside the cochlear lumen in both
the instances. Whereas one of these patients had exten-
sive basal turn ossification, the other had RW obliteration
only with successful entry into scala tympani lumen.
While some degree of difficult electrode insertion or even
misplacement into a false demineralized ring in instances
of cochlear ossification (6,8,11), including partial elec-
trode insertion (8,9,11), has been reported, others have
not mentioned any difficulty or incompleteness of inser-
tion if the ossification can be drilled to obtain a patent
cochlear lumen (10,23). In a histopathologic study, Lee
et al. (30) analyzed histopathologic features in instances
of incomplete insertions and found ossification or soft
tissue presence in the cochlear lumen to be the reason in
only minority of the patients. They remarked that the only
statistically significant feature leading to incomplete
insertion was dissection of electrode through spiral liga-
ment to the lateral cochlear wall with a trend toward
incomplete insertion as a function of the number of times
electrode array crossed from one scala to the other (30).
In the current study, despite two cases of incomplete
insertion, in all cases of ossification, a patent lumen could
be obtained after drilling, hence casting doubt over
whether ossification, when adequately managed, has
any association with incomplete insertion.
Scala vestibuli insertion in patients of complete basal
turn ossification occurred in one patient in the current
series, in the presence of no identifiable lumen in scala
tympani. Full electrode insertion was achieved with no
postoperative complications or adverse outcomes. Scala
vestibule insertion has been suggested as a valid insertion
option in patients of totally ossified scala tympani with-
out any adverse outcomes (31). In one study, however,
scala vestibuli insertion in one patient in a series of
otosclerotic patients led to diffuse facial nerve stimula-
tion and had to be switched off (23).
One intraoperative false tract insertion into the supe-
rior semicircular canal was observed in the current study
which was identified using a perioperative CT scan and
was corrected at the same time. No extensive demineral-
ization or ossification was present on preoperative CT,
and basilar membrane damage with slightly superior
trajectory of insertion postdrilling of the obliterated
RW niche could be the reason for the same. This,
however, emphasizes the importance of radiological
confirmation of correct electrode placement postimplan-
tation in otosclerotic patients with ossification.
The prospect of cochlear luminal ossification and
slight difficulty in electrode insertion has even led to
certain authors performing auditory brainstem implants
(32) for cochlear otosclerosis as a spectrum of nontu-
moral indications for the same. This, in our opinion, is
incorrect as it has been proved by our previous multi-
centric review (33) that patients who were previously
implanted using an auditory brainstem implant for co-
chlear otosclerosis with ossification did not obtain any
significant auditory rehabilitation using the same, and
were subsequently rehabilitated with a cochlear implant
with complete electrode insertion, despite ossification.
 TABLE 3. Brief comparison of previous literature on series of cochlear implantation in otosclerosis with surgical and auditory outcomes
% of Patients Electrodes Used Intraoperative Complica-
Number With Cochlear Surgical (Straight/ tions/Extent of Electrode Postoperative Revision Surgery
Studies of Patients Ossification Approach Perimodiolar) Insertions FNS With Indications Auditory Outcomes

Rotteveel et al. 53 32% PT Straight and Three misplaced 38% (5% with Two contralateral Speech performance
2004 perimodiolar insertions (one LSSC, perimodiolar) revisions due to scores negatively
and 2010 one SSSC, one primary single correlated with disease
otosclerotic cavity), channel (later extent on CT and
seven partial ST revised by percentage of inactive
insertions, one partial multichannel partial electrodes
SV insertion, one tip insertion) and partial
rollover electrode insertion
(later revised by
double array
insertion). Two
misplaced and
partial insertions
with 1 eventual
explantation
Psillas et al. 5 40% PT Perimodiolar Full insertions, ST 20% 1 year SC 60.5
Quaranta et al. 9 100% PT Straight Full insertion, ST 44.4% 1 year SC 83.1
2005
Sainz et al. 2007 15 PT Straight One incomplete insertion 13.3% None 1 year SC >80
and 2009
Rama Lopez 30 13.3% PT Straight and Full insertion, ST 0% None 1 year SC 66
et al. 2006 perimodiolar
Matterson et al. 59 18.6% PT Straight and ST and SV (number not 23.7% (0% with No correlation between
2007 perimodiolar mentioned) perimodiolar) speech scores in
obliterated and
nonobliterated cochleae
Mosnier et al. 16 37.5% PT Straight Full insertion, ST 6% 1 year SC 89
2007
Marshall et al. 30 10% PT Straight and Full insertion, ST 17% (0% with 1 year SC 75
2005 perimodiolar perimodiolar)
Semaan et al. 30 29.4% PT Perimodiolar Full insertion, ST 0% 1 year SC 92. No
COCHLEAR IMPLANTATION IN OTOSCLEROSIS

2010 difference in outcomes

between ossification
group and no
ossification group
Castillo et al. 17 5.8% PT (14) and Straight One incomplete insertion, 11.7% None 1 year SC>80
2014 transatical (3) ST
Current study 36 69.5% STP (14 ears) Straight Two incomplete 5.2% Three revisions due to 1 year SC 68 (82 for
and PT insertions (ST), one two device failures patients with no
misplaced insertion and one extrusion ossification, 55 for
(SSSC), one complete patients with

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SV insertion ossification)

FNS indicates facial nerve stimulation; LSSC, lateral semicircular canal; PT, posterior tympanotomy/extended posterior tympanotomy; SC, postoperative sentence scores in %; SSSC, superior
semicircular canal; ST, scala tympani; STP, subtotal petrosectomy; SV, scala vestibuli.
e351

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e352 A. VASHISHTH ET AL.
Out of three revision surgeries, two were due to device
failure and one post-extrusion. All patients underwent
uneventful reimplantation with complete electrode
insertion.
Facial Nerve Stimulation
Varying rates of postimplantation FNS in otosclerotic
patients have been reported in the literature ranging from
none to 38% (711,2024) (Table 3). However, on
analyzing these results closely, they differ when corre-
lated with the kind of electrode used (straight/perimo-
diolar). Although the incidence of FNS with perimodiolar
electrodes has been reported to be close to 0% (10,23,34),
it has varied from 6% (24) to 40% in the subset of patients
implanted using straight array (23) respectively.
In the current series using straight arrays, 1 patient of
36 developed postoperative FNS bilaterally (5%) which
was successfully managed by reducing current levels and
increasing pulse widths initially followed by switching
off 1 electrode (No. 18) on right side and 3 (nos. 13, 14,
15) on the left side. This is since the electrode contacts
most often implicated in FNS are the mid array ones
located in the upper basal turn, being closest to the
labyrinthine facial nerve (18,35). Seyyedi et al. (36) in
a histopathological study concluded that the only micro-
scopic finding significantly associated with FNS was
full-thickness replacement of normal bone by pathologic
bone with spongiosis, neoangiogenesis, cavitation, and
soft tissue deposition with invasion of endosteum of both
the upper basal turn of cochlea and facial nerve canal by
otosclerosis. The specific involvement of this bone can
be sought on preoperative CT scanning to undertake
preemptive measures, such as use of perimodiolar elec-
trodes or alterations in a programming strategy.
In a computer model, the spiral ligament was observed
to have a much higher conductivity to current spread, as
compared with the more resistive organ of corti and
basilar membrane (37). Extensive structural alterations
such as hyalinization of spiral ligament are a hallmark of
otosclerosis (24), possibly leading to the conductance
of current to the facial nerve canal. In another computer
model (38), the same authors concluded that the excita-
tion thresholds for facial nerve remain relatively constant
in otosclerosis, and the main effect seems to be an
increased current required to produce MCLs with higher
bone conductivity. The progressive requirement of
higher MCLs in otosclerosis has been observed by others
as well (7,8).
Auditory Outcomes
Despite some concerns that otosclerosis is associated
with negative postimplantation speech scores (14), most
studies on otosclerosis have uniformly demonstrated
auditory outcomes and speech performance scores that
are comparable to age-matched controls (711,2025).
Although Rotteveel et al. (11,22) found positive corre-
lation between reduced speech performance postimplan-
tation with extent of disease, as observed on CT
or intraoperatively, others did not find so (10). No
Otology & Neurotology, Vol. 38, No. 9, 2017
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significant differences in speech discrimination were
found between otosclerotic patients and nonotosclerotic
controls in many studies (7,9,10), though higher MCLs
and electrical thresholds were observed in otosclerotic
patients (7) that did not correlate with any of the auditory
parameters. Rotteveel et al. (11,12), apart from disease
extent, also attributed the reduced performance to lesser
active electrodes on follow-up, coupled with a high
percentage of electrode deactivation to manage FNS.
Semaan et al. (10) found worse short-term speech
reception thresholds associated with intracochlear ossi-
fication but found no difference in short or long-term
word or sentence scores or long-term speech reception
thresholds. In the current study, all parameters such as
vowel, word, sentence scores, and comprehension im-
proved significantly activation onward and remained
relatively plateaued 1 year onward. Though all patients
without any cochlear ossification fared better in all
auditory scores throughout postoperative period ranging
4 years with a trend toward significance, no statistical
significance could be achieved for any of them except
word scores at 24 months. This seems since a long
duration of hearing loss, such as observed in the current
study itself can lead to a delay in eventual auditory
outcomes, with 2 years being the representative period
for the assessment of the same.
CONCLUSIONS
Cochlear implantation in otosclerosis provides good
auditory rehabilitation despite higher incidence of
cochlear ossification. The completeness of successful
electrode insertion may not depend on ossification, if
patent cochlear lumen can be obtained, and postoperative
radiological confirmation is strongly recommended.
Facial nerve stimulation must be sought on CT changes
preoperatively and use of modiolar hugging electrodes
seem to be preventive. In the event of FNS, alterations in
programming strategy including switching off offending
mid array electrodes may not affect speech outcomes
significantly. Auditory outcomes do not seem to be
significantly worse for patients with ossification in
otosclerosis than in those patients who do not have
any ossification.
Acknowledgments: The authors thank Dr. Charu Shukla for
indispensable assistance in statistical analysis.
REFERENCES
1. Schuknecht HF, Merchant SN, Nadol JB. Schuknechts Pathol-
ogy of the Ear. New York, NY: McGraw-Hill Medical; 2010 .
726 37.
2. Doherty JK, Linthicum FH Jr. Spiral ligament and stria vascularis
changes in cochlear otosclerosis: Effect on hearing level. Otol
Neurotol 2004;25:45764.
3. Parahy C, Linthicum FH Jr. Otosclerosis: Relationship of spiral
ligament hyalinization to sensorineural hearing loss. Laryngoscope
1983;93:71720.
 COCHLEAR IMPLANTATION IN OTOSCLEROSIS
4. Abd el-Rahman AG. Cochlear otosclerosis: Statistical analysis of
relationship of spiral ligament hyalinization to hearing loss.
J Laryngol Otol 1990;104:9525.
5. Merkus P, van Loon MC, Smit CF, Smits C, de Cock AF, Hensen
EF. Decision making in advanced otosclerosis: An evidence based
strategy. Laryngoscope 2011;121:193541.
6. Sanna M, Free R, Merkus P, et al. Surgery for Cochlear and Other
Auditory Implants. Stuttgart: Georg Thieme Verlag; 2016.
7. Sainz M, Garcia-Valdecasas J, Garofano M, Ballesteros JM.
Otosclerosis: Mid-term results of cochlear implantation. Audiol
Neurotol 2007;12:4016.
8. Sainz M, Garcia-Valdecasas J, Ballesteros JM. Complications and
pitfalls of cochlear implantation in otosclerosis: A 6-year follow up
cohort study. Otol Neurotol 2009;30:10448.
9. Castillo F, Polo R, Guiterez A, Reyes P, Royuela A, Alonso A.
Cochlear implantation outcomes in advanced otosclerosis. Am J
Otolaryngol 2014;35:55864.
10. Semaan MT, Gehani NC, Tummala N, et al. Cochlear implant
outcomes in patients with far advanced otosclerosis. Am J
Otolaryngol 2012;33:60814.
11. Rottoveel LJ, Proops DW, Ramsden RT, Saeed SR, van Olphen AF,
Mylanus EA. Cochlear implants in 53 patients with otosclerosis:
Demographics, computed tomographic scanning, surgery and com-
plications. Otol neurotol 2004;25:94352.
12. Polak M, Ulubil A, Hodges AV, Balkany TJ. Revision cochlear
implantation for facial nerve stimulation in otosclerosis. Arch
Otolaryngol Head Neck Surg 2006;132:398404.
13. Ramsden R, Bance M, Giles E, Mawman D. Cochlear implantation
in otosclerosis: A unique positioning and programming problem.
J Laryngol Otol 1997;111:2625.
14. Kraaijenga VJ, Smit AL, Stegeman I, Smilde JJ, van Zanten GA,
Grolman W. Factors that influence outcomes in cochlear implanta-
tion in adults, based on patient-related characteristicsa retrospec-
tive study. Clin Otolaryngol 2016;41:58592.
15. Gajadeera EA, Galvin KL, Dowell RC, Busby PA. The change in
electrical stimulation levels during 24 months postimplantation for
a large cohort of adults using Nucleus cochlear implant. Ear Hear
2017;38:35767.
16. Polo R, Del Mar Medina M, Aristegui M, et al. Subtotal petrosec-
tomy for cochlear implantation: Lessons learnt after 110 cases. Ann
Otol Rhinol Laryngol 2016;125:48594.
17. Free RH, Falcioni M, Di Trapani G, Giannuzzi AL, Russo A, Sanna
M. The role of subtotal petrosectomy in cochlear implant surgery
a report of 32 cases and review on indications. Otol Neurotol
2013;34:103340.
18. Kelsall DC, Shallop JK, Brammeier TG, et al. Facial nerve stimu-
lation after Nucleus 22-channel cochlear implantation. Am J Otol
1997;18:33641.
19. Vincenti V, Pasanisi E, Guida M, D Trapani G, Sanna M. Hearing
rehabilitation in Neurofibromatosis type 2 patients: Cochlear versus
Auditory brainstem implantation. Audio Neurotol 2008;13:27380.
20. Rama Lopez J, Cervera-Paz FJ, Manrique M. Cochlear implantation
of patients with far-advanced otosclerosis. Otol Neurotol 2006;27:
1538.
Copyright 2017 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
e353
21. Quaranta N, Bartoli R, Lopriore A, Fernandez-Vega S, Giagnotti F,
Quaranta A. Cochlear implantation in otosclerosis. Otol Neurotol
2005;26:9837.
22. Rotteveel LJ, Snik AF, Cooper H, Mawman DJ, van Olphen AF,
Mylanus EA. Speech perception after cochlear implantation in 53
patients with otosclerosis: Multicentre results. Audiol Neurotol
2010;15:12836.
23. Matterson AG, OLeary S, Pinder D, Freidman L, Dowell R, Briggs
R. Otosclerosis: Selection of ear for cochlear implantation. Otol
Neurotol 2007;28:43846.
24. Mosnier I, Bouccara D, Ambert-Dahan E, Ferrary E, Sterkers O.
Cochlear implantation and far-advanced otosclerosis. Adv
Otorhinolaryngol 2007;65:3237.
25. Marshall AH, Fanning N, Symons S, Shipp D, Chen JM, Nedzelski
JM. Cochlear implantation in cochlear otosclerosis. Laryngoscope
2005;115:172833.
26. Psillas G, Kyriafinis G, Constantinidis J, Vital V. Far-advanced
otosclerosis and cochlear implantation. B-ENT 2007;3:6771.
27. Blamey P, Arndt P, Bergeron F, et al. Factors affecting auditory
performance of postlinguistically deaf adults using cochlear
implants. Audiol Neurotol 1996;1:293306.
28. Tyler RS, Summerfield AQ. Cochlear implantation: Relationships
with research on auditory deprivation and acclimatization. Ear
Hear 1996;17:38S50S.
29. Balkany T, Gantz BJ, Steenerson RL, Cohen NL. Systematic
approach to electrode insertion in the ossified cochlea. Otolaryngol
Head Neck Surg 1996;114:411.
30. Lee J, Nadol JB Jr, Eddington DK. Factors associated with incom-
plete insertion of electrodes in cochlear implant surgery: A histo-
pathologic study. Audio Neurotol 2011;16:6981.
31. Lin K, Marrinan MS, Waltzman SB, Roland JT Jr. Multichannel
cochlear implantation in the scala vestibuli. Otol Neurotl 2006;27:
6348.
32. Colletti V, Shannon R, Carner M, et al. Outcomes in nontumor
adults fitted with the auditory brainstem implant: 10 years experi-
ence. Otol Neurotol 2009;30:6148.
33. Merkus P, D Lella F, Di Trapani G, et al. Indications and contra-
indications of auditory brainstem implants: Systematic review and
illustrative cases. Eur Arch Otorhinolaryngol 2014;27:313.
34. Aschendorff A, Jaekel K, Klenzner T, Laszig R. Impact of electrode
design on facial nerve stimulation in otosclerosis. Cochlear
Implants Int 2004;5 (suppl 1):635.
35. Kruschinski C, Weber BP, Pabst R. Clinical relevance of the
distance between the cochlea and facial nerve in cochlear implan-
tation. Otol Neurotol 2003;24:8237.
36. Seyyedi M, Herrmann BS, Eddington DK, Nadol JB Jr. The
pathologic basis of facial nerve stimulation in otosclerosis and
multi-channel cochlear implantation. Otol Neurotol 2013;34:
16039.
37. Briaire JJ, Frijns JHM. Field patterns in a 3D tapered spiral model of
the electrically stimulated cochlea. Hear Res 2000;148:1830.
38. Frijns JH, Kalkman RK, BriaIre JJ. Stimulation of the facial nerve
by intracochlear electrodes in otosclerosis: A computer modelling
study. Otol Neurotol 2009;30:116874.
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