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Cochlear implantation or stapes

surgery in far advanced


otosclerosis
Systematic Review/Meta-Analysis
Submitted for Partial Fulfillment of Master Degree in
Otorhinolaryngology
By
Hanaa Sabry Hadaey Abd El-Aziz
M.B.B.Ch, Ain shams university.

Under supervision of
Prof. Dr. Abdelhamid Abdelhamid Elnashar
Professor of Otorhinolaryngology
Faculty of Medicine - Ain Shams University

Prof. Dr. Ehab Kamal


Professor of Otorhinolaryngology
Faculty of Medicine - Ain Shams University

Dr. Ahmed Teaima


Lecturer of Otorhinolaryngology
Faculty of Medicine - Ain Shams University

Faculty of Medicine
Ain Shams University
2021
Acknowledgment
First and foremost, I feel always indebted to ALLAH, the
Most Kind and Most Merciful.
I’d like to express my respectful thanks and profound
gratitude to Prof. Dr. Abdelhamid Abdelhamid
Elnashar, Professor of Otorhinolaryngology, Faculty of Medicine
- Ain Shams University for his keen guidance, kind supervision,
valuable advice and continuous encouragement, which made
possible the completion of this work.

I am also delighted to express my deepest gratitude and


thanks to Prof. Dr. Ehab Kamal, Professor of
Otorhinolaryngology, Faculty of Medicine - Ain Shams University,
for his kind care, continuous supervision, valuable instructions,
constant help and great assistance throughout this work.

I am deeply thankful to Dr. Ahmed Teaima, Lecturer of


Otorhinolaryngology, Faculty of Medicine - Ain Shams University,
for his great help, active participation and guidance.

Hanaa Sabry
‫شكر وعرفان‬

‫إلى أبي أشكرك على كونك مثال أعلى دائما وعلى حثك المستمر لي ألكون أفضل‬
‫وأشكر حبك الدائم ودعمك المستمر وأتمنى لو أحقق لك ما تتمناه لي‪ ..‬أحبك قبل كل‬
‫‪.‬شئ وفوق كل شئ‬

‫إلى أمي حبيبتي التي طالما كانت الضلع الثابت الذي أميل إليه وعليه فكانت خير‬
‫‪.‬سند وخير قلب‪ ..‬شكرا بحجم السماء وأحبك جدا‬

‫إلى زوجي الحبيب شكرا لسندك ودعمك وشكرا ألنك هنا دائما وما كنت أنا ها هنا‬
‫‪.‬لوالك‬

‫‪.‬إلى أخوتي األحباء أدامكم هللا لي سندا وعونا ورفقاء طريق‬

‫‪.‬إلى فريدة ومصطفى أبنائي أحبكم وأتمنى لو تفخروا بي يوما ما‬


List of Contents

Title Page No.

List of Tables................................................................................................i
List of Figures.............................................................................................ii
Introduction.................................................................................................1
Aim of the Work..........................................................................................7
Review of Literature
Anatomy of Oval Window Area.....................................................8
Pathophysiology of Otosclerosis...................................................23
Diagnosis and Clinical Features of Otosclerosis...........................27
Methods.....................................................................................................71
Results.......................................................................................................73
Discussion.................................................................................................93
Summary.................................................................................................109
Conclusion...............................................................................................117
References...............................................................................................118
Arabic Summary........................................................................................––
List of Tables
Table No. Title Page No.

Table (1): Characteristics table for patients in the included articles........92

i
List of Figures
Fig. No. Title Page No.

Figure (1): Schematic drawing of the stapes in the oval


window niche and its relationship with the
underlying saccule and utricle. Stapedial tendon
in red.................................................................10
Figure (2): Endoscopic view of a left middle ear showing
the stapedial tendon(s) rising from the pyramidal
process (p), the tensor tympani muscle (1)
turning around the cochleariform process (*) to
give the tensor tympani tendon (2) that inserts
on the neck of malleus (M); I incus, ET
Eustachian tube................................................12
Figure (3): pp posterior pillar, ap anterior pillar, teg tegmen,
fu fustis, f finiculus, su subiculum, rw round
window, pr promontory,t tunnel of subcochlear
canaliculus, st sinus tympani, po ponticulus, s
stapes................................................................15
Figure (4): Endoscopic picture of left ear round window
niche showing the round window membrane...16
Figure (5): Left lateral view of mesotympanum,
epitympanum, and semicircular canals............17
Figure (6): The audiogram of a patient with bilateral far-
advanced otosclerosis.......................................22
Figure (7): Double halo and narrowed basal turn in a patient
with left retrofenestral otosclerosis..................37
Figure (8): Electrode in cochleostomy (arrow) with excess
electrode coiled in mastoid cavity....................58

ii
List of Figures Cont...

Fig. No. Title Page No.

Figure (9): Opened facial recess.........................................62


Figure (10): Round window membrane where electrode will
be placed. Left ear............................................65
Figure (11): Treatment algorithm of advanced otosclerosis.66
Figure (12): PRISMA flow diagram of the search and review
process..............................................................74
Figure (13): Meta-analysis for satisfaction regarding
stapedectomy VS CI.........................................75
Figure (14): Meta-analysis for satisfaction rate...................76
Figure (15): Meta-analysis for ability to use hearing aids
after stapedectomy...........................................77
Figure (16): Meta-analysis for any postoperative
complications rate............................................78
Figure (17): Meta-analysis for difficult access to area of
cochleostomy rate in CI...................................78
Figure (18): Meta-analysis for difficult insertion of electrode
bundle rate in CI...............................................79
Figure (19): Meta-analysis for dysgeusia rate......................80
Figure (20): Meta-analysis for tinnitus rate..........................80
Figure (21): Meta-analysis for vertigo rate..........................81
Figure (22): Meta-analysis for mean recognition of
monosyllables...................................................82
Figure (23): Meta-analysis for mean recognition of
disyllables.........................................................82
Figure (24): Meta-analysis for mean recognition of phrases83

iii
List of Figures Cont...
Fig. No. Title Page No.

Figure (25): Meta-analysis for facial electrical stimulation


rate in CI...........................................................84
Figure (26): Meta-analysis for family history of otosclerosis
rate....................................................................85
Figure (27): Meta-analysis for hearing loss rate after surgery86
Figure (28): Meta-analysis for history of previous
stapedectomy rate.............................................87
Figure (29): Meta-analysis for retrofenestral extension rate 88
Figure (30): Meta-analysis for revision surgery rate............89
Figure (31): Meta-analysis for mean pure tone average.......90
Figure (32): Meta-analysis for mean speech reception
threshold...........................................................91

iv
Introduction 

INTRODUCTION
Otosclerosis is a process of bone resorption of the
labyrinthine capsule followed by reparative deposition of new,
immature sclerotic bone. It usually occurs during the
postlingual period between the second and fifth decade of life.
The most commonly affected location is around the oval
window (fenestral otosclerosis), which results in conductive
hearing loss due to stapes footplate fixation. As it undergoes a
maturation process, the sclerotic bone increases in size and
depth (Güneri et al., 1996; Adrien et al., 2018).

In approximately 10% of patients, otosclerotic foci


invade deeper into the labyrinth, resulting in retrofenestral
otosclerosis; this process gradually leads to severe mixed
hearing loss and then to profound sensorineural hearing loss
(SNHL) (Liselotte et al., 2004).

Several studies have indicated that retrofenestral sclerotic


foci may lead to hearing loss through disturbance of the ionic
homeostasis of the cochlea by hindering ion recycling and
reducing the endocochlear potential. This leads to dysfunction
or loss of cochlear hair cells (Cureoglu et al., 2010; Doherty et
al., 2004).

Next to the radiological diagnosis, there exists a


functional diagnosis for otosclerosis with severe mixed hearing
loss called far advanced otosclerosis (FAO). Far advanced

1
Introduction 

otosclerosis was first defined by House and Sheehy (House et


al., 1961) in the 1960s as hearing loss in air conduction (AC)
threshold by 85 dB with nonmeasurable bone conduction (BC)
(probably because of limitations in audiometry at that time)
(Mun˜oz et al., 2012).

There is no universally accepted definition for advanced


otosclerosis. Recently, the term advanced otosclerosis is used
when a patient with otosclerosis has severely decreased speech
recognition. Calmels and colleagues described advanced
otosclerosis by its audiologic and radiologic criteria. The
audiologic criteria for diagnosis was the detection of dissyllabic
words less than 30% of the speech discrimination (SD) score at
70 dB, with a well-equipped hearing aid and a blank audiogram
(Calmels et al., 2007).

Various authors have used different CT grading systems


for the classification of advanced otosclerosis. The CT grading
system of Rotteveel (Rotteveel et al., 2004), is partially based
on location and on the type of lesion: solely fenestral (grade1),
retrofenestral: double ring or halo effect (grade 2A), narrowed
basal turn (grade 2B) or both (grade 2C), and diffuse confluent
retrofenestral involvement (grade3). Symons and Fanning
(Marshall et al., 2005), proposed a classification similar to
Rotteveel, except grade 2 is based on anatomic location instead
of the type of lesion: basal turn (2A), middle/apical turns (2B),
both basal and middle/ apical turns (2C).

2
Introduction 

Kabbara and colleagues described another classification


system based on radiologically detected otosclerotic lesions. In
Kabbara's system, stage 1 lesions were characterized by limited
footplate and pericochlear lesions without endosteum
involvement; stage 2 lesions were characterized by significant
pericochlear and endosteum involvement, and stage 3 lesions
were characterized by full obliteration of the round window
and/or basal turn ossification associated with pericochlear
lesions (Kabbara et al., 2015).

Unfortunately, there are no standard guidelines regarding


the rehabilitation of advanced otosclerosis. In advanced
otosclerosis there are three treatment options to propose to
the patient:

(1) no intervention and continue hearing aids, (2) stapes


surgery and hearing aid use, or (3) cochlear implantation (CI). In
some patients with advanced otosclerosis, the decision can be
difficult because of two factors. First, with mixed hearing loss it is
hard to predict the success rate of stapedotomy, especially if
compared to CI as an alternative intervention. Second, extensive
otosclerotic foci around the otic capsule can lead to surgical
complications during cochlear implantation (Liselotte et al.,
2004; Psillas et al., 2007; Ramsden et al., 1997).

Not only the success rate plays a role in the decision;


each intervention has specific advantages and disadvantages.
Stapes surgery is a relatively simple, safe, and lowcost

3
Introduction 

procedure that can accomplish very good results. Stapes


surgery is a suitable treatment option for patients with advanced
otosclerosis, and should be considered mandatory, before
offering cochlear implantation, for those with a demonstrable
conductive component to their hearing loss. A small group of
patients get little benefit from surgery and subsequently a
cochlear implant should be considered (Heining et al., 2017).
However, the results after stapedotomy in severe mixed hearing
loss are unpredictable and variable because stapedotomy is not
applicable for the treatment of sensorineural hearing loss
(Calmels et al., 2007).

Moreover, a feared complication of stapes surgery is an


increase of sensorineural hearing loss, which in advanced
otosclerosis could result in a functionally deaf ear. Cochlear
implantation has yielded excellent results and seems to be an
good treatment for patients with advanced otosclerosis (Rama
et al., 2006; Quaranta et al., 2005; Sainz et al., 2009).

However, CI is an expensive procedure and requires


experienced surgeons because otosclerotic foci can cause
certain surgical problems during implantation. Ossification of
the round window or the basal turn requires extra drilling to
identify the scala tympani. Some patients with severe
osteoneogenesis require a scala vestibuli approach to achieve a
full insertion. Otosclerosis can also lead to obliteration at the
apical regions of the cochlea, which may result in an
incomplete electrode insertion (Rotteveel et al., 2004).

4
Introduction 

Confluent otospongiotic lesions can surround the cochlea,


resulting in pericochlear hypodensity and an osteolytic cavity
(double ring or halo effect). Because this double ring runs parallel
to the basal turn of the cochlea, and the round window has often
vanished in a sclerotic plaque, the halo can resemble an opening
in the basal turn resulting in an electrode misplacement in this
false lumen (Rotteveel et al., 2004; Lee et al., 2009).

It is also possible that an electrode is inserted in the basal


turn, that it penetrates the cochlear endosteum, and eventually
enters the osteolytic cavity or even the internal auditory canal
(Ramsden et al., 1997).

Even after a successful implantation, the rehabilitation of


patients with otosclerosis is challenging because progressive
otosclerotic changes in the cochlea can affect the performance
of the implant.

Reprogramming with higher stimulus levels might be


required to obtain auditory responses, although these high
stimulus levels increase the risk of facial nerve stimulation. In
general, the incidence of facial nerve stimulation in patients
with otosclerosis is high (Toung et al., 2004).

An electrical shunt between the implant and the facial


nerve can cause Facial nerve stimulation (FNS). FNS is an
apprehensive complication of CI occurring on average in 20%
of the patients with otosclerosis. This high occurrence of FNS

5
Introduction 

can be explained by an increased conductivity of the


otospongiotic bone, making it easier to stimulate the facial
nerve. Management of facial nerve stimulation consists of a
reduction in stimulus levels of the cranially located electrodes,
totally deactivating the causative electrodes or reimplantation
(Rotteveel et al., 2004; Sainz et al., 2009).

6
Aim of the Work 

AIM OF THE WORK

To compare the hearing outcomes of stapedotomy vs


cochlear implantation in patients with far advanced
otosclerosis.

7
 Anatomy of Oval Window Area
Review of Literature

Chapter 1
ANATOMY OF OVAL WINDOW AREA
Auditory ossicles

The malleus is hammer shaped and considered the


largest of the middle ear three ossicles. Its length is 8-9 mm and
its weight is about 20-25 mg. It has 4 parts head, neck, handle
and two processes which arise from below the neck (Baily,
2001).

The incus is 5-7 mm long and its weight is about 30 mg.


It has 4 parts a trapezoidal body, short process, long process,
and a rounded lenticular process (Wright, 2001).

The stapes which is the smallest bone of the human


body, is 3.25 mm high and 1.4 mm wide and weighs about 3-4
mg. It lies in a horizontal plane between the lenticular process
and the oval window and below the facial nerve canal. It
consists of 5 parts, a round head, a short neck, anterior and
posterior crura, and an oval footplate (Bruner, 1998).

The head which is the most lateral part of the stapes, is


cylindrical or discoid in shape and its medial part has a glenoid
cavity, the fovea, which corresponds to the articular surface of
the lenticular process. It has a constricted medial end which
forms the neck. It has a smooth anterior edge and a posterior

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 Anatomy of Oval Window Area
Review of Literature

edge which has a small rough surface for the stapedial muscle
tendon insertion (Gerhardt, 1981).

The stapes has two unequal crura, posterior and anterior,


the posterior crus is longer, thicker, and more curved than the
anterior one. The area delimited by the concave arches of the
crura is the obturator foramen, sometimes bridged by a veil of
mucous membrane. The two crura could be very close to the
walls of the niche of the oval window (Wright, 1997).

During stapedectomy, it’s safer to cut the posterior crus


of the stapes rather than to fracture it because the later carries a
risk of footplate luxation. The anterior crus can be fractured
safely because it’s thinner.

The footplate is a thin and oval lamella of bone which is


3 mm long, 1.5 mm wide and about 0.25 mm thick. Its lateral
surface is covered by mucoperiosteum of the middle ear.

It is slightly twisted around its polar axis so that the


anterior half looks to the floor of the vestibule and the posterior
part looks up to the tegmen tympani. The distance from the long
process of the incus to the tympanic surface of the footplate is
about 4 mm. It has a flat medial surface which is lined by the
endosteum of the otic capsule and is in close relation with the
saccule and utricle. The saccule is 1 mm deep from the anterior
part of the vestibular surface of the footplate, and the utricle is at
1.5 mm deep from its posterior part (Lambert, 1990).

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 Anatomy of Oval Window Area
Review of Literature

Figure (1): Schematic drawing of the stapes in the oval window niche and
its relationship with the underlying saccule and utricle. Stapedial tendon
in red. (*) annular ligament, P pyramidal eminence, H head, N neck, AC
anterior crus, PC posterior crus, FP footplate.

Ligaments of the Ossicles

The malleus is kept in place by five ligaments, one


articulation, one tendon, and the tympanic membrane. Three of
the five ligaments are outside the axis of rotation and offer only
a suspensory function. They are: anterior suspensory ligament,
lateral suspensory ligament, superior suspensory ligament,
anterior malleal ligament, posterior malleal ligament (Couter,
1980).

The incus has the least number of ligaments “the


posterior incudal ligament and the superior incudal ligament”
so it’s more susceptible to traumatic dislocation in comparison
to other ossicles (William, 1995).

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 Anatomy of Oval Window Area
Review of Literature

The annular ligament of the stapes is a ring of elastic


fibers which attaches cartilaginous margin of the footplate to the
border of the oval window. Its fibers fuse with the periosteum and
endosteum all around the oval window borders. It’s thinner and
more mobile anteriorly, it acts as a hinge like attachment of the
stapes into the oval window due to the differential thickness
between its anterior and posterior aspects. This attachment allows
a rocking oscillation of the footplate in the oval window, it’s the
essential movement for the high frequency sounds transmission.
The transmission of the low frequency sounds relies on the piston
like movements of the stapes which needs the whole annular
ligament elasticity (Moore, 1997).

Otosclerotic involvement of the anterior aspect of the


annular ligament hinders the piston-like movement of the
stapes rather than the rocking movement, which explains the
low-frequency conductive hearing loss in early stages of
otosclerosis. The posterior part of the annular ligament
conserves its insulator capacity which explains the on/off
stapedial reflex phenomena in early otosclerosis (House, 2004).

The Muscles of the Tympanic Cavity

The tensor tympani: it’s a fusiform muscle which is 20


mm long, its intra-tympanic portion is 2.5 mm long. It arises from
the cartilage of the Eustachian tube, from the walls of its
enveloping bony semi-canal, and from the adjacent portion of the
greater wing of the sphenoid bone. The fibers converge to form a

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 Anatomy of Oval Window Area
Review of Literature

central fibrous core which, proceeding posteriorly, forms the


tendon of the muscle. The most medial fibers of the tendon attach
to the cochleariform process, at which point the main body of the
tendon turns laterally into the cavity to attach to the medial
surface of the junction of the neck and the manubrium of the
malleus. It is innervated by the trigeminal nerve via the nerve to
the medial pterygoid muscle (Baily, 1997).

Its function is drawing the manubrium medially, tensing


the tympanic membrane and damping the movement of the
ossicular chain.

Figure (2): Endoscopic view of a left middle ear showing the stapedial
tendon(s) rising from the pyramidal process (p), the tensor tympani
muscle (1) turning around the cochleariform process (*) to give the tensor
tympani tendon (2) that inserts on the neck of malleus (M); I incus, ET
Eustachian tube.

The stapedius: it’s the smallest skeletal muscle in the


body which measures only 1mm. It lies in a bony cavity in the
posterior wall of the tympanic cavity to emerge from the

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 Anatomy of Oval Window Area
Review of Literature

pyramidal eminence. Its fibers converge into a tendon which


variably attaches to the head and/or posterior crus of the stapes.
It is innervated by the stapedial branch of the facial nerve.

Its contraction provokes a tilting of the stapes by moving


the anterior border of the footplate laterally and the posterior
border medially. This tilting of the stapes stretches the annular
ligament, thus fixing the footplate and damping its movements.
It protects the inner ear from damage caused by loud noise.
Lack of action of this muscle from nerve section or facial nerve
palsy induces hyperacusis (Glasscock, 2003).

Anatomy of round window area

The round window region is rich of anatomical details


which imply important surgical concerns. The round window
area consists of (the round window niche, the round window
skeleton, the round window membrane).

The round window niche

It is located in the posteroinferior aspect of the


promontory in the medial wall of the tympanic cavity. It’s the
bony entrance to the cochlear membrane. It’s around 2 mm
from the inferior margin of the oval window and is separated
from the promontory by the subiculum. It’s 2 mm wide and
around 1 mm high (Marchioni et al., 2016).

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 Anatomy of Oval Window Area
Review of Literature

The round window chamber

The round window chamber is defined as the three-


dimensional space lying between the round window niche and
the round window membrane (Chen et al., 2016).

The round window skeleton

It’s the second opening of the labyrinth into the middle


ear. It’s not always round and is of average (1.91 ± 0.78 mm)
height, and (1.37 ± 0.43 mm) width. It shows the following
bony structures:

– The tegmen of the RW: is defined as the oblique


dorsolateral edge of the promontory forming a convex edge
over the entrance of the RW.

– The posterior pillar: is a pillar located near the bony


edge of the round window niche entrance. In its posterior and
superior aspect, it forms an acute angle with the tegmen.

– The anterior pillar: is a pillar located in the anterior and


superior aspect of the round window niche, fusing with the
anterior portion of the tegmen. The posterosuperior and
posteroinferior walls of the RW meet posteriorly, leading to the
sinus tympani (Chen et al., 2016).

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 Anatomy of Oval Window Area
Review of Literature

Figure (3): pp posterior pillar, ap anterior pillar, teg tegmen, fu fustis, f


finiculus, su subiculum, rw round window, pr promontory,t tunnel of
subcochlear canaliculus, st sinus tympani, po ponticulus, s stapes. (Chen
et al., 2016)

The round window membrane

It has a saddle point shape The visible central portion is


concave toward the cavity, but its edges are convex. The
internal aspect of the RW membrane faces the scala tympani of
the basal turn of the cochlea. The thickness of the RW
membrane is of 70 μm which does not change with advancing
age (Carpenter et al., 1989).

Its length is 1.70 mm and its width is 1.35 mm. Like the
tympanic membrane, the RW membrane is composed of three
layers: an outer epithelium of low cuboidal cells lining the
middle ear, an inner epithelium of squamous cells bordering the

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 Anatomy of Oval Window Area
Review of Literature

inner ear, and a layer of connective tissue between the


epithelial layers. The connective tissue layer consists of
fibroblasts, collagen, and elastic fibers. The distance between
the hinge of the RW niche and the center of the RW membrane
ranges from 1.39 to 2.12 mm (Fugita et al., 2016).

Figure (4): Endoscopic picture of left ear round window niche showing
the round window membrane (Carpenter et al., 1989).

Semicircular canals

The anterior and posterior canals, termed the vertical


canals, most of the anterior canal came to lie above the level of
posterior canal. The membranous canals, much smaller than the
osseous canals, are situated eccentrically in the perilymphatic
space, so that they lie against the convex surface of the
periosteal lining of the bony canals. Here, they are united by
denser connective tissue than the trabeculae, which elsewhere
bridge the perilymphatic spaces of the bony canals. The

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 Anatomy of Oval Window Area
Review of Literature

ampullated ends of the superior and posterior semicircular


canals empty through short, wide continuations into the utricle
(Merchant et al., 2010).

Figure (5): Left lateral view of mesotympanum, epitympanum, and


semicircular canals (Aaron, neurosurgical atlas, 2021).

The utricle

The utricle or utriculus is an elongated portion of the


membranous labyrinth receiving both ends of each semicircular
canal. On the inferior surface of the utricle and extending
slightly onto its lateral surface lies the macula, which contains
sensory endings from the superior division of the vestibular
nerve, the utricular nerve. The macula of the utricle lies almost
in the horizontal plane. The macula is fringed with marginal
fibers. Through the oval window, the macula can be seen above
as a white plaque in the vestibule.

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 Anatomy of Oval Window Area
Review of Literature

The saccule

Unlike the utricular macula, the saccular macula lies in a


shallow bony depression, the spherical recess. The saccular
macula lies at an approximate right angle to the utricular
macula in the vestibule.

The saccular wall consists of mesothelial and epithelial


cell layers. The anterior wall near the stapes is thickened by the
presence of connective tissue between the layers and is called
the reinforced area of the saccular membrane. The saccular duct
together with the utricular duct becomes the utriculosaccular
duct, which communicates with the endolymphatic duct. The
saccule is continuous with the cochlea through the tiny ductus
reuniens.

The cochlea

The promontory is the prominent area between the oval


and round windows. The perilymphatic spaces in the cochlea,
the scala vestibuli, and the scala tympani are compared to a
winding U-tube. One end of the tube is anchored by the stapes
footplate and the other by the round window membrane.
However, the membrane lies not at the end but at the side of the
tube. The tube ends in a short blind sac, the vestibular cecum
(Yasuya Nomura, 2014).

The footplate of the stapes is anchored in the oval


window. Its anterior edge protrudes somewhat into the middle

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 Anatomy of Oval Window Area
Review of Literature

ear cavity, whereas its posterior edge fits to the frame of the
window. Manipulation of the head of the stapes during ear
surgery, with force directed posteriorly, parallel to the stapedial
tendon, can dislocate the stapes from the oval window
(Altmann, 1964).

The upper surface of the vestibular lip of the limbus


spiralis intersects at right angles the furrows produced by tooth
shaped elevations, called Huschke’s teeth. Between the
auditory teeth are the interdental cells, to which the tectorial
membrane is attached. The interdental cells maintain the
integrity of the tectorial membrane (Friedmann et al., 1996).

Otosclerosis

Otosclerosis is an abnormal process of otic and


labyrinthine capsules involving continuous bone osteolysis and
osteogenesis. Typically, otospongiotic lesions occur during the
active phase of otosclerosis but the newly formed and more
compact lamellar bones present during late inactive phase of
otosclerosis (Marchioni et al., 2016). In the active phase, a
reddish tint can be seen through the tympanic cavity “schwatze
sign”, it helps in the diagnosis of otosclerosis and it’s related to
the vascularity of the promontrium which is associated with
otosclerosis active otospongiotic focus (Cureoglu et al., 2010).
Otosclerosis mostly occurs between the second and fifth decade
of life (Rama-lopez et al., 2006). The area adjacent to the oval

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 Anatomy of Oval Window Area
Review of Literature

window is typically affected and results in conductive hearing


loss through stapes fixation (Abdurehim et al., 2016).

96% of affected patients have been reported to have


otosclerosis foci located in the anterior part of the oval window,
and in 30% of clinical otosclerosis round window niche is also
involved (Chole, 2001).

In 60% of patients of advanced otosclerosis treated by


cochlear implantation, ossification of round window membrane
was detected, and in 30% there was scala tympani ossification
(Marchioni et al., 2016).

In otosclerotic patients, once hearing loss starts it usually


worsens, it happens at low frequencies initially then at higher
ones. 10% of otosclerosis patients who have conductive hearing
loss, develop sensorineural hearing loss. Advanced otosclerosis
was defined by House and Sheely in 1961, as hearing loss in air
conduction (AC) threshold by 85 dB with nonmeasurable bone
conduction (BC).

The term advanced otosclerosis is now used when there


is severe decrease in speech recognition in a patient with
otosclerosis. Audiologic and radiologic criteria were used to
describe advanced otosclerosis by Calmels and colleagues. The
audiologic criteria was detecting dissyllabic words less than
30% of the speech discrimination (SD) score at 70 dB, with a

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 Anatomy of Oval Window Area
Review of Literature

well-equipped hearing aid and a blank audiogram (Lachance et


al., 2012).

The most significant points in the diagnosis of far-


advanced otosclerosis are the Following: positive family
history with an onset of a gradually progressive hearing
impairment in early adult life, the wearing of an aid with
apparent great benefit despite the extent of the hearing
impairment, a voice (modulation, pronunciation) that is not
suggestive of a purely sensorineural hearing impairment,
excellent voice quality and articulation, negative ontological
examination, the tympanic membrane is usually intact and
normally mobile. Negative Rinne Tests with Weber
Lateralization to the Poorer Ear (Hildyard, 1972).

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 Anatomy of Oval Window Area
Review of Literature

Figure (6): The audiogram of a patient with bilateral far-advanced


otosclerosis (Lachance et al., 2012).

22
 Pathophysiology of Otosclerosis
Review of Literature

Chapter 2
PATHOPHYSIOLOGY OF
OTOSCLEROSIS
Histology

Otosclerosis is characterized by progressive focal


dysplasia with destruction, remodeling and finally sclerosis of
the endochondrial bone of the labyrinth capsule. Otospongiotic
stage comes first with osteoclastic process, presence of
lymphocytes, histocytes, plasma cells and deposition of
antibody complexes. (Arnold et al., 1996) with increased
activity of proteolytic enzymes and cathepsin is found. Then
comes the otosclerotic stage, which has progressive sclerosis
through a highly mineralized bone of mosaic-like structure
(Altermat et al., 1992).

Early lesions appear as sheets of connective tissue


replacing bone adjacent to the fissula ante fenestram.
Osteoclastic activity absorbs bone and osteocytes deposit new
bone. The otocytes are found at the advancing edge of the
lesion and extends into the otic capsule in fingerlike
projections. These lesions have vascular spaces in the center
which result in disorganized bone rich in osteocytes with
enlarged marrow spaces rich in blood vessels and connective
tissue. These lesions have an affinity for hematoxylin, making
the bone appearing darker. Healthy surrounding bone has few

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 Pathophysiology of Otosclerosis
Review of Literature

viable osteocytes and chondrocytes and is relatively avascular.


The osteoclasts are multinucleated and appear in the center of
the lesion, absorbing the already disorganized bone
(Linthicum, 2002).

Obliterative otosclerosis is a thick ossification of the


whole oval window with severe conducting hearing loss. It can
also happen when the annular ligament is very constricted with
fibrous thickening. It can manifest clinically as “Biscuit”
footplate, with a thick ossified footplate loosely fixed in the
margin of the oval window. In that case, the floating footplate
complication may happen during the perforation of the
footplate (Cherukupally et al., 1998).

Otosclerotic lesions can involve the entire labyrinth


capsule progressively in rare cases with complete occlusion of
the round window. In these cases, there is no aim to gain
hearing using stapedectomy. Despite the advanced otosclerosis
of the labyrinth block, the structure of the inner ear often
remains preserved. However, sometimes, progressive
degenerative changes occur in the inner ear, in particular
atrophy of the spiral ligament, which takes on a hyaline
appearance. Those changes within the labyrinth then bring an
additional inner ear hearing loss (Arnold et al., 2007).

In rare cases, the otosclerotic adhesions affect wide areas


of the petrous bone of the inner ear in addition to the oval and

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 Pathophysiology of Otosclerosis
Review of Literature

round window. This is called malignant otosclerosis and can


lead to progressive deafness (Nadol, 2006).

Pathology

Gross pathology

Gross examination of the immature active focus of


otoscleosis shows a region of bone which is covered by
thickened vascular mucosa. A pinkish hue can be seen on the
promontory when viewed through a translucent tympanic
membrane (schwartze’s sign).

The normal otic capsule is slightly yellow but a mature


disease focus appears white. The normal anatomy of the oval
window niche may be distorted also. The size of the focus
ranges from a few millimeters to a large area involving most or
all of the promontory, and can sometimes reach the round
window niche. (Shambaugh et al., 2008)

The site of onset is usually (80-90%) just anterior to the


oval window niche “fissula ante fenestrum”. The second most
commonly involved region (30-50%) is the border of the round
window niche. The round window involvement is common but
complete obliteration of the niche is rare (1-3%). In order of
decreasing frequency, foci are also found in the apical medial
wall of the cochlea, posterior to the oval window, the posterior
internal auditory canal, the primary footplate, and the
semicircular canals. The incus and malleus involvement is rare.

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 Pathophysiology of Otosclerosis
Review of Literature

There is more than one focus in half of the diseased ears, which
may occur in various stages of activity (Goycoolea, 1991).

Microscopic pathology

Microscopic examination of the otosclerosis immature


active focus shows a porous spongy structure of irregular bony
lamella separated by wide vascular spaces containing an
abundance of histocytes, osteoblasts and some osteoclasts.
Conspicuously missing are the islands of calcified cartilage
characteristic of endochondral bone (Shumrick et al., 1991).

The mature disease focus is relatively acellular, with


narrower vascular spaces and smaller blood vessels. There are
different levels of activity between foci in the same ear but it
tends to be a prevailing level of disease activity within a given
individual. The borders of the focus are irregular and tend to
project into normal bone along blood vessles. These finger-like
projections of disease import a blue-staining border (or mantle)
around the involved blood vessels, known as "blue mantles of
Manasse" (Linthicum 1993, Meyerhoff et al., 2001).

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 Diagnosis and Clinical Features of Otosclerosis
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Chapter 3
DIAGNOSIS AND CLINICAL FEATURES
OF OTOSCLEROSIS
Clinical diagnosis

There is no problem to diagnose the otosclerotic hearing


loss. Typically, there is young adult with bilateral symmetrical
or unilateral progressive loss of hearing, with a positive family
history in 30% of cases. Half of the patients complain of
tinnitus and vertigo (Del et al., 1987; Gersdorf et al., 2000).
The vestibular symptoms tend to be rather mild, except in co-
existing Meniere's disease, in rare cases (in 1.5-3 %) the
otosclerotic hearing loss appears before and during adolescence
(Robinson et al., 1999; Romanet et al., 2004). Although a first
slight loss of hearing had already been noted before the age of
20 years in 15% of patients whose otosclerosis was first
diagnosed when they were adults (Lippy et al., 2006).

These patients hear their own voices by bone conduction


so they speak with a low-volume. Tinnitus occurs
approximately in 75% of patients complaining of otosclerosis.
Associated vestibular symptoms can occur in (25-30%) of
patients and they are attributed to lesions of the horizontal canal
and usually not severe. These symptoms must be carefully
differentiated from those of Meniere’s disease, as hydrops is a
contraindication to stapes surgery. Elderly patients with

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

advanced otosclerosis not infrequently develop progressive


inner ear hearing impairment in addition to the conductive
hearing loss (Vartianinen, 2008).

Ramsay and Linthicum (1994) stated that inner ear


hearing loss eventually occurs in almost 10 % of all patients
with otosclerosis, and in some cases, in so-called "malignant"
otosclerosis, complete deafness develops (Schknecht, 1974). In
recent years, a typical indication for a cochlear implant in the
elderly is bilateral deafness due to malignant otosclerosis
(Kornblut and Ganzer, 2009).

Patients who had been stapedectomized on only one side,


the bone conduction threshold remained significantly better in
the operated ear than in the non-operated ear over an
observation period of ten years in several studies (Conrad
2004). So in bilateral otosclerosis, an early sequential bilateral
stapedectomy should be carried out as recommended by the
authors.

The laser Doppler vibrometry is a new aspect in the


clinical diagnosis of otosclerosis, which is a research tool that
measures middle ear function by registering sound-induced
tympanic membrane velocity (Jakob et al., 2009).

Clinical examination

In otosclerotic patients, physical examination of the ear


is usually normal but it can rule out other diagnoses sharing

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 Diagnosis and Clinical Features of Otosclerosis
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similar symptoms. Occasionally, a reddish blush may be seen


over the promontory and in the oval window niche area. This
happens because of the rich vascular supply of an immature
otospongiotic focus of bone and is called Schwartz’s sign. It
can be identified in only 10% of otosclerotic patients (Dubreuil
et al., 2004).

An important part of the physical exam is the Rinne and


Weber tests with tuning forks with 256, 512, 1024 Hz
frequencies. A negative Rinne test is first noted with the 256
Hz fork and with the disease progression, it progresses up the
frequency spectrum. Patients with a more advanced hearing
loss can be identified with the Weber test which determine the
ear which suffered the greatest insult (Vartiainen et al., 2009).

Patients can present with no measurable hearing or with an


air-bone conduction threshold of 95 to 100 dB. It is important to
consider otosclerosis in these patients as they can obtain
serviceable aided hearing after corrective surgery. Sheehy (1979)
pointed out clues to this diagnosis as: a positive family history
with gradual progression of hearing loss starting in early life;
paracusis noted in an earlier stage of hearing loss; present or
previous wearing of a bone conduction hearing aid; and a
previous audiogram demonstrating a measurable air-bone gap.

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 Diagnosis and Clinical Features of Otosclerosis
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Audiological investigations

Pure tone audiogram

It’s obligatory to have a preoperative audiological


investigation with a classic pure tone audiogram (Hannley,
2008). According to the guidelines of the Committee on
Hearing and Equilibrium of the American Academy in 1995,
the hearing threshold should also be measured at 3 kHz in
addition to the classic octave frequencies 0.25, 0.5,1,2,4 and 8
kHz. A conductive hearing loss of about 40 db in the low
frequencies with a reduction of the gap towards 2 kHz is
typical, because the ankylosis of the stapes joint in the oval
window primarily has functional effects in the low frequencies
(Merchant et al., 1995). Conductive hearing losses of up to 65
db have been measured in obliterative otosclerosis and also in
fibrosis of the annular ligament, but only when this was very
severe (Cherukupally et al., 2002).

Flat conductive hearing losses of between 50 and 70 db


are atypical for otosclerosis and are more indicative of
discontinuity in the chain of the auditory ossicles or a middle
ear malformation (Committee on Hearing and Equilibrium of
the American Academy in 2009).

The speech audiogram

At a raised acoustic pressure, it shows a normal increase


in speech discrimination. Reduced speech discrimination

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 Diagnosis and Clinical Features of Otosclerosis
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indicates additional inner ear involvement, which may or may


not be otosclerotic in nature and which should be investigated
further.

Acoustic reflex morphology

Is a very sensitive indicator of otosclerosis. In contrast to


the normal configuration in which middle ear compliance is
reduced for the duration of a stimulus, in otosclerosis there is a
characteristic diphasic response or "on-off" phenomenon. With
this response there is an increase in compliance at both the onset
and termination ("offset") of the sound stimulus. This "on-off"
response can often be seen prior to the development of a
detectable air-bone gap. Advancing stapedial fixation affects both
the ipsilateral and contralateral acoustic reflexes (Hannley, 2008).

It’s unclear why this happens but it may result from


inherent elasticity in the otosclerotic anterior footplate and
crura allowing the non-affected posterior footplate to move
with stapedius contraction and relaxation. The amplitudes of
the acoustic reflex are reduced as the stapes fixation progresses.
followed by elevation of ipsilateral, then contralateral
thresholds, and finally, disappearance of the reflexes altogether
(Roland and Meyerhoff, 2001).

Forty percent of the normal population can have a brief


compliance increase at the onset of a stimulus but the offset
increase (at the termination of the signal) is virtually

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

pathognomonic of early stapedial fixation. It is often seen in the


first 5 years of disease progression but is rarely seen in
otosclerosis of 10 years or longer (Hall et al., 2012).

The various test results in the clinical and audiological


investigations should coincide. The cause of any discrepancy
must be elucidated. Not infrequently it is then found that
instead of classic otosclerotic conductive hearing loss, a
sensorineural hearing loss is present with a small, additional
conductive component (Jerger, 2011).

Imaging procedures

It is controversial whether imaging studies are needed,


beyond the physical exam, tuning forks, and audiogram, in
diagnosing and managing patients with otosclerosis. Those in
favor of computed tomography (CT) scanning of the temporal
bone in these patients site a number of advantages. CT can
characterize the extent of the otosclerotic focus at the oval
window and can be used when the clinical diagnosis is in doubt
(patients with profound mixed hearing loss). It is also indicated
by some when patients have significant mixed hearing loss to
determine if capsular involvement is present. An enlarged
cochlear aqueduct may be seen which increases the index of
suspicion for a potential perilymph gusher during footplate
fenestration or removal (Alan, 2006).

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 Diagnosis and Clinical Features of Otosclerosis
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Radiographic investigations in patients with otosclerosis


were published for the first time in 1928, by Graham
Hodgson, from the 1960s onwards; it became possible to
visualize foci of otosclerosis of up to 1 mm in diameter with the
use of the polytomographic technique (Valvassori, 2008). High
resolution computed tomography is currently the best method
for obtaining precise information about the bone structure of
the petrous bone. Constrictions and ossifications of the oval
window can be recognized radiologically via computerized
tomography in 90 % of cases of surgically confirmed
otosclerosis (Swartz et al., 2012; Miura et al., 2008).

The demineralization in active otospongiosis lesions is


seen as a characteristic "double ring structure" or as a halo
effect" (Valvassori, 2008; Marc et al., 2007), inactive, highly
sclerotic lesions present as a uniform hyperdense mass and are
sometimes difficult to distinguish from the normal compact
labyrinth capsule.

In cases with a histological-radiological correlation, even


massive otosclerosis lesions have remained unrecognized in a
preliminary CT- investigation (Thiers et al., 2014). CT scanning
of the temporal bone should be taken in the axial and coronal
oblique planes. The axial projection is obtained by having the
patient lay supine with the cantho-meatal line perpendicular to the
table top. 1 mm cuts are then taken from the top of the superior
semi-circular canal to the inferior aspect of the cochlea. The
coronal oblique view should be taken with the patient supine with

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

the head overextended so that the X-ray beam passes


perpendicular the Frankfurt’s line (Swartz et al., 2012).

Types of otosclerosis

According to the localization of the disease there are two


types of otosclerosis: fenestral and retrofenestral.

Fenestral otosclerosis types: A- Anterior focus, the


fissula ante fenestram, which lies in front of the oval window,
is the principal place where otosclerosis appears. B- Posterior
focus, in which otosclerosis may appear behind the oval
window. C- circumferencial, in which the disease process
spreads around the margin of the stapes footplate. D- biscuit
type, in which the disease process involves the footplate but the
annular ligament is free. E- Obliterative type, where the disease
process completely obliterates the oval window niche.

Retrofenestral involvement occurs much more frequently


due to the progression of fenestral otosclerosis with
encroachment on the bone surrounding the membranous
labyrinth than as an isolated manifestation. Depending on the
stage of the disease, more or less dense, spotty or band-shaped
plaques are found in the os petrosum. The cochlea may have a
double-ring appearance (Valvassori sign). The otosclerotic
lesions can reach all structures of the membranous labyrinth
(cochlea, vestibule, semicircular canals, endo- and

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

perilymphatic duct), the facial or internal auditory canal, but


they can also lie isolated in the bone (Naumann et al., 2005).

Rotteveel and colleagues described a classification


system for advanced otosclerosis based on CT evaluation of the
otic capsule involvement. The CT grading system of Rotteveel
is partially based on location and on the type of lesion: solely
fenestral (grade1), retrofenestral: double ring or halo effect
(grade 2A), narrowed basal turn (grade 2B) or both (grade 2C),
and diffuse confluent retrofenestral involvement (grade3)
(Rotteveel, 2004). Symons and Fanning (Marshall et al., 2005),
proposed a classification similar to Rotteveel, except grade 2 is
based on anatomic location instead of the type of lesion: basal
turn (2A), middle/apical turns (2B), both basal and middle/
apical turns (2C).

Kabbara and colleagues described another classification


system based on radiologically detected otosclerotic lesions. In
Kabbara’s system, stage 1 lesions were characterized by limited
footplate and pericochlear lesions without endosteum
involvement; stage 2 lesions were characterized by significant
pericochlear and endosteum involvement, and stage 3 lesions
were characterized by full obliteration of the round window
and/or basal turn ossification associated with pericochlear
lesions (Kabbara et al., 2015).

Clinical history, audiologic tests, previous otosclerosis


surgery, and high-resolution computed tomography (CT) scans

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

can aid in the diagnosis of advanced otosclerosis.16 Because of


the recent prevalence of cochlear implantation, SD scores have
been more commonly used than pure-tone thresholds for the
diagnosis of advanced otosclerosis (Merkus, 2011).

Temporal bone CT and/or MRI can aid in the detection


of advanced otosclerosis. CT imaging studies have reported
that SNHL correlated with the severity of otosclerosis. High-
resolution CT can detect oval window abnormalities in 80% to
90% of otosclerosis cases. Bone densitometry can show the
disease severity and help to measure the grade of otospongiosis.
On CT scan, the presence of pericochlear lucency is specific to
cochlear otosclerosis. This finding is usually referred to as a
ring or double halo. Rings emerge in cases whereby the
pericochlear confluent foci surround the cochlear lumen. On
MRI, a ring with an intermediate signal is usually detected in
the pericochlear area, with mild to moderate enhancement of
the gadolinium in T1-weighted images. MRI can also detect
cochlear duct patency (Marshall et al., 2005; Rotteveel et al.,
2004).

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

Figure (7): Double halo and narrowed basal turn in a patient with left
retrofenestral otosclerosis (type 2a based on Rotteveel classification
system) (Merkus, 2011).

Management of otosclerosis

There are many factors which help in determining the


best method of treatment for otoscleosis patient, such as the
results of audiometry and tuning fork method, surgeon skills,
the patient’s medical condition and patient wishes. Patients
undergoing surgical treatment of otosclerosis have an increased
satisfaction rate than those who use hearing aids. Once the
diagnosis is made, treatment options including hearing aids and
surgery with their advantages and disadvantages should be
extensively discussed with the patient. (Porter et al., 2004).

The patients are advised to visit the clinic again after a


week to have enough time to think and not to rush. The best
surgical candidate is a patient in good health with a socially un
acceptable ABG, a negative Rinne test, excellent

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

discrimination, and the acceptance for surgery after an


appropriate period of time for deliberation. Other factors that
should be considered when counselling patients about surgery
are the age of the patient occupation, and the history of prior
stapes surgery (De Bruijn et al., 2006).

Treatment options

- No treatment - Medical treatment

- Hearing aid - Surgical treatment

1- No treatment

Otosclerosis does not have to be treated. It is usually


advisable to have a hearing test repeated once a year (or earlier
if hearing drops) (Timothy, 2009).

2- Medical treatment

The only proposed medical treatment has been sodium


fluoride, which is a dietary supplement. Sodium fluoride taken
orally has been shown to stabilize the hearing loss associated
with otosclerosis in 80% of patients. The fluoride ion replaces
the usual hydroxyl ion in periosteal bone, forming fluorapatite,
instead of the usual hydroxyapatite. In addition, bone resorption
is reduced, and calcification of new bone is enhanced. Actively
expanding foci of otosclerosis are inactivated, as has been
documented by computed tomography. In addition, tinnitus and
imbalance are reduced, and schwartz’s sign frequently becomes
negative (Shambaugh, 2008).

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 Diagnosis and Clinical Features of Otosclerosis
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Side effects of fluoride (Florical and Monocal are the


two preparations available over the counter) include occasional
stomach upset, allergic itching, and increased joint pains. If
aggravation of arthritis occurs, the fluoride is stopped and the
joints return to their previous state in a few weeks. In such a
situation, patients can "pace themselves", taking as much of the
medication as can be tolerated. Typical doses are one tablet
three times a day (florical) and one-two tablets about 20-40 mg
of fluoride a day three times a day (Monocal) (Shambough,
2008; Causse et al., 2008).

In pregnant women and children this dosage is decreased.


After two years of fluoride treatment, the efficacy of the treatment
is evaluated. Schwartz’s sign, and the degree of tinnitus and
imbalance are reassessed, and a CT scan is repeated. If overall
stabilization of the disease has occurred, the dose of fluoride is
reduced from three times a day to once a day (about 25mg).

In theory, avoidance of estrogens or use of estrogen


blockers might be helpful in individual with otosclerosis as
otosclerosis frequently worsens during pregnancy, suggesting
hormonal modulation (Causse et al., 2008).

3- Hearing aid

Patients who are not fit for surgery or don’t want to


undergo surgery, will use a hearing aid. Certainly, the option of
amplification as an alternative to surgery should be discussed
with all patients. The benefit over surgery is the avoidance of
the risk of significant SNHL (House, 2001).

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 Diagnosis and Clinical Features of Otosclerosis
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Patients who undergo successful surgery have a higher


satisfaction rate than those wearing hearing aids. This is
probably due to couple of reasons. The first is the canal
occlusion effect in which the hearing aid gives the patient the
sensation they are hearing in a barrel. The other is the fact that
amplification is not used at night (Roland et al., 2001).

In ADVANCED OTOSCLEROSIS the following


treatment options are in recent use: (1) hearing aids without
surgery, (2) hearing aids with stapes surgery, (3) the direct
acoustic cochlear stimulation implant, and (4) cochlear
implantation.

A new implantable hearing system, the direct acoustic


cochlear stimulator (DACS) is presented. This system is based
on the principle of a power-driven stapes prosthesis and
intended for the treatment of severe mixed hearing loss due to
advanced otosclerosis. It consists of an implantable
electromagnetic transducer, which transfers acoustic energy
directly to the inner ear, and an audio processor worn externally
behind the implanted ear. The device is implanted using a
specially developed retromeatal microsurgical approach. After
removal of the stapes, a conventional stapes prosthesis is
attached to the transducer and placed in the oval window to
allow direct acoustical coupling to the perilymph of the inner
ear. In order to restore the natural sound transmission of the
ossicular chain, a second stapes prosthesis is placed in parallel
to the first one into the oval window and attached to the

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

patient's own incus, as in a conventional stapedectomy


(Häusler et al., 2008).

4- Surgical treatment

For a successful surgical outcome, one must begin


planning treatment with the initial physical examination. This
begins with an overall observation of the patient. The age of the
patient alone should not be a contraindication to performing
surgery. In patients less than 16 years of age, there is a greater
chance that the conductive impairment is secondary to
congenital anomalies rather than otosclerosis. Likewise, very
active diffuse obliterativeotosclerosis may be found in a young
patient, predisposing to a higher incidence of complications.
Yet surgery should be considered in the young because hearing
acuity is essential during the formative years of development.
Patients older than 75 years are still surgical candidates if they
are in good health. Hearing acuity becomes more important as
other senses decline with age (Glasscock et al., 2003).

A- Stapes surgery

When surgery is decided upon, the worst hearing ear


should be approached first. The basic order of steps in stapes
surgery for otosclerosis has remained relatively constant over
the past 30 years. What have changed are subtleties in
technique and style in performing each of these basic steps.
This starts with the anesthetic choice. This is determined by the

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

surgeon’s preference, patient wishes, and medical condition of


the patient (Cokkeser et al., 2008).

Surgeons who use local anesthesia with sedation tend to


be experienced otologic surgeons in a non-teaching
atmosphere. The advantages sited are that hearing restoration
can be immediately evaluated and that the patient can report
symptoms of vertigo. In addition, local anesthesia is useful in
patients with pre-existing medical conditions that preclude
them from general anesthesia. General anesthesia is useful in
teaching institutions where Hearing results are similar for both
methods. The patient is seen initially in holding and the correct
ear to be operated on is confirmed with the patient and by the
audiogram and CT (if available). The ear is then marked
externally to ensure that there is no confusion with the rest of
the OR staff (Roland et al., 2001).

The CT scan and audiogram are then placed in a visible


location in the OR for ease in intra-operative reviewing. Once
anesthesia has been attained, the patient’s ear is prepped, usually
with Betadine (if there is no history of allergy to iodine), and the
ear is draped in a sterile fashion (Roland et al., 2001).

Anaesthesia

Stapedotomy is performed under local anesthesia &


General anesthesia.

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

The external auditory canal is infiltrated with 1% lidocaine


(Xylocaine) and with 1:200,000 epinephrine (House, 2002).

Raising the tympanomeatal flap

The tympanomeatal incision is carried out using a variety


of cutting devices (roller knife, round knife, or various straight
blades). The canal skin is incised at the six and twelve o’clock
positions and are carried out approximately 6-8 mm lateral to
the annulus. A lateral curved cut parallel to the annulus
connects these incisions. It is important to make the
tympanomeatal incision lateral enough to the annulus to allow
enough skin to drape back over the scutum, which will be
partially removed (Valsalva, 2004).

If the flap is too big, however, it will become difficult to


retract it out of the way of the operating field. The flap is
elevated with a round knife anteriorly to the annulus. Cotton
soaked with adrenaline can be helpful in with elevation and
controlling bleeding (Bhardwaj et al., 2008).

The annulus is then removed from its sulcus with a


Rosen needle. The chorda tympani nerve is identified and is
separated from the TM. The nerve should be preserved if
possible. Releasing its attachments to the medial surface of the
malleus can increase the slack on the nerve and allow it to be
move out of the way without stretching it. In some cases, it is in
the way of the operating field, despite maneuvers to increase its

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 Diagnosis and Clinical Features of Otosclerosis
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mobility. In these cases, it should be cut rather than stretched


out of the way. Patients develop more severe and prolonged
dysgeusia from stretching the nerve than from cutting it. In
addition, there is a risk of retrograde inflammation to the facial
nerve with possible delayed facial nerve paralysis
(Hammerschlag et al., 2009).

Exposure

Removal of a portion of the scutum is almost always


necessary for adequate visualization of the stapes footplate,
oval window niche, and surrounding structures. A curette is
used to remove bone just lateral to the edge of the scutum first.
This thins the scutum and allows for easy removal. Attempts to
remove the edge first may result in slipping of the instrument
and injury to surrounding structures (Valsalva et al., 2001).

It is important to remove enough bone posteriorly to allow


for visualization of the pyramidal eminence and superiorly to
allow visualization of the inferior border of the facial nerve. A
small amount of bone should be left over the incus superiorly to
prevent retraction of the tympanic membrane onto the lateral
surface of the ossicles (Sheehy et al., 2009).

Middle ear examination

All three ossicles should be palpated for fixation prior to


and after division of the incudostapedial joint. If malleus or

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 Diagnosis and Clinical Features of Otosclerosis
Review of Literature

incus fixation is encountered, these must be addressed as well


to attain optimal hearing results (Lippy et al., 2003).

Sometimes a very small focus of active otosclerosis is


found, usually at the anterior pole of the stapes footplate.
Gentle palpation of the footplate will sometimes mobilize it.
The patient can then be followed and treated with sodium
fluoride to convert the focus into the sclerotic phase. In general,
re-fixation usually occurs, making this technique less appealing
(Rosen, 1955).

The tympanic segment of the facial nerve should be


examined and overhang into the oval window niche as well as
any bony dehiscence should be noted. Dehiscence usually
occurs on the medial and inferior aspect of the nerve
(Bhardwaj and Kacker, 1988).

The distance between the footplate and the medial


surface of the lower aspect of the long process of the incus is
then measured (usually 4.5 mm). About 0.25 to 0.50mm is
added to the measured distance to accommodate entrance of the
prosthesis slightly in to the vestibule. Measurement before
disarticulation of the incudostapedial joint may be more
accurate, since movement of the incus is possible with joint
disarticulation. Some surgeons still prefer to perform the
measurement after disarticulation (De La Cruz et al., 2000).

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Laser method

In 1978, the first laser stapedotomy was performed by


Perkins and has since become a widely accepted method for
fenestrating the footplate. The three most commonly used
lasers for stapedotomy are the potassium titanyl phosphate
(KTP/532), argon, and carbon dioxide lasers (Perkins, 1980).

The KTP/532 and argon lasers have similar biosurgical


effects. They both are visible beams of light and therefore; do
not require a separate laser to act as an aiming beam. This
allows for far greater target accuracy because they act as their
own aiming beam (Di Bartolbmeo, 1980).

The CO2 laser is an invisible surgical beam and must be


used with a separate aiming beam, usually a red helium-neon
beam, which produces an ill-defined fuzzy border. The CO2
beam must be perfectly lined up with the aiming beam or the
target will be off center (Lesiniski et al., 1989).

Both the KTP/532 and the argon lasers are absorbed by


hemoglobin making them better coagulators than the CO2
laser. The CO2 laser is less expensive and requires less
maintenance. When using the laser, it is important to evaluate
all equipment in the pre-operative period to ensure proper
function (Silverstein, 1998).

The CO2 laser in continuous wave mode is effective for


removing soft tissue, and it can vaporize thin bony structures

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when focused to a small spot. One of the main advantages of


the far-infrared emission of the CO2 laser is its strong
absorption by water, resulting in a shallow penetration depth of
only 0.01mm from the irradiated surface. This property of CO2
laser light is particularly useful in stapes surgery. During a
stapedotomy, the perilymph completely absorbs the CO2 laser
energy and thus protects the inner ear structures from direct
injury (Lesinski, 1989).

Operative laser technique

As in conventional surgery, the external auditory canal is


infiltrated with 1% lidocaine (Xylocaine) and with 1:200,000
epinephrine, and the tympanomeatal flap is elevated to enter the
middle ear. The canal bone covering the oval window niche is
removed with a sharp House curette or diamond bur, preserving
the chorda tympani. sufficient access to the oval window is
gained when the pyramidal process and tympanic segment of
the facial nerve are clearly visible (House, 2002).

Before the CO2 laser is used, test firings are made on a


wooden spatulaor other suitable object to check for any mis
alignment between thehelium neon (HeNe) aiming beam and
the invisible CO2 laser beam.

Then the stapedial tendon, incudostapedial joint, and


crura are vaporized and the footplate is perforated with the CO2
laser beam using non-contact technique (Haberkamp, 1999).

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The stapedial tendon is vaporized with two or three


separate pulses of 0.05 sduration at 2 W (power density 8000
W/cm2). In some cases, it may be possible to preserve the tendon
if anatomic conditions are favourable (Laske et al., 2011).

The incudostapedial joint is generally separated by


conventional means incases with complete stapes fixation. If
the footplate is only partially fixed, laser-assisted separation of
the joint is performed. The joint is opened with 8–14 pulses of
0.05 s duration at 6W (power density 24,000 W/cm2),
vaporizing the stapes capitulum. Since the CO2 laser beam
often does not strike the joint precisely at a perpendicular
angle, the joint should also be probed with amanual instrument,
which is used to clear any remaining connections between the
lenticular process and stapes capitulum (Jovanovic, 2004).

The posterior crus, which is generally thicker, longer,


and more curved, is transected close to the footplate with
conventional technique or with laser four to eight pulses of 0.05
s duration at 6W (power density 24,000W/cm2), the same
settings usedon the incudostapedial joint. When this relatively
high wattage is used to vaporize the joint and posterior crus,
care should be taken that the beam does not accidentally strike
middle ear structures that lie in the path of the beam
(footplate,facial canal). This can be prevented by filling the
middle ear with physiologic saline solution or covering these
structures with moist gelatin sponge (Gelfoam). If the posterior
crus remnant is still too long after the suprastructure has been

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removed, it can be vaporized to the level of the footplate using


the same laser parameters to obtain better posterior exposure of
the footplate (Buchman, 2000).

The anterior crus of the stapes is fractured with a small


hook using conventional technique. If all or part of the anterior
crus is still visible, it is vaporized with the CO2 laser beam using
the same parameters as for the posterior crus.If this does not
completely transect the crus, the vaporized site can be fractured
using controlled pressure on the small hook. This virtually
eliminates the danger of mobilizing the footplate or even partially
or completely extracting it. The stapes superstructure is then
extracted with a small forceps (Moscillo, 2006).

After the suprastructure has been removed, the


stapedotomy opening is created, usually placing it in the
posterior half of the footplate. The goal is to create an
approximately round, reproducible fenestra 0.5–0.7 mm in
diameter,applying the beam either in a single application (one-
shot technique) 2- to 3 W laser application of 0.03–0.05 s
duration or in aslightly overlapping pattern (multi-shot
technique), without causing significant thermal alteration of the
peripheral zones (Silverstein, 2014).

Care should be taken that the vestibule is filled with


perilymph to ensure adequate protection for inner ear structures
and prevent damage from direct irradiation. If the perilymph is
inadvertently suctioned from the vestibule, no additional laser

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energy should be applied to the footplate. Lasing of the


footplate is continued only after additional fluid has seeped into
and adequately filled the vestibule. It may be necessary in some
cases to fill the vestibule with physiologic saline solution
(Lescanne, 2010).

A platinum-fluoroplastic piston 0.4–0.6 mm in diameter


is then inserted into the fenestra and connected to the long
process of the incus.

Sealing the oval window and placement of the piston

Multiple substances can be used with equal efficacy in


sealing the oval window. Causse (1980) is a proponent of vein
grafts. He made the argument that it is important to reconstruct
the annular ligament. The function of the annular ligament is to
provide both resistance (dissipation force) to protect the inner
ear from acoustic trauma and elasticity for acoustic transfer
(disrupted by otosclerosis). Because of the inherent elastic
properties of vein, it is better suited to recreate this physiologic
function. Vein, fat, and perichondrial grafts require additional
incisions unlike temporalis fascia or blood.

Once the oval window has been sealed the appropriately


sized prosthesis is placed into the fenestra and around the incus.
Multiple stapes prostheses have been developed over the past 30
years. The sharp or beveled polyethylene strut developed by
Shea (1985) is associated with higher incidence of postoperative

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fistula, so it is rarely used today. Similarly, the wire prosthesis


attached to compressed gel foam has been associated with higher
incidence of postoperative fistula, due to reparative granuloma
formation. Stapedotomies are usually repaired with piston type
prosthesis of 0.6 mm or 0.8 mm diameter, which again rests on a
connective tissue graft. The prosthesis usually attaches to the
long process of the incus, either with a wire that is crimped into
place, or a bucket handle that fits under the lenticular process of
the incus (Schuknecht, 1993).

After placement of the prosthesis, the malleus is palpated


to ensure appropriate movement of the repaired ossicular chain,
one consideration when choosing the diameter of the prosthesis
to use is how wide the oval window niche is. A deep narrow
niche may require a thinner prosthesis such as a wire. If the
patient is awake, hearing can be tested by whispering into the
patient's ear. The tympanomeatal flap is then redraped and the
ear canal is packed with gel foam (Nadol, 1998).

Contraindications

In the past, stapedectomy was not performed on those


who experience frequent changes in barometric pressure, such
as pilots and divers. When gelfoam and wire prostheses were
commonly used, postoperative fistula and prosthesis
displacement were more common in these patients. However,
with small fenestra stapedotomy and piston reconstruction,
displacement is uncommon and perilymph fistula is very rare,

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even with flying and scuba diving. According to House (1993),


he allows his patients to fly after five days, resume physical
exercise after 10 days and play football or scuba dive after
three weeks. A conservative approach would be to discuss the
patient's hobbies and job requirements, and encourage the
patient to make the final decision.

Since postoperative disequilibrium sometimes occurs,


elderly patients with a baseline imbalance or those whose jobs
demand excellent balance (e.g., iron workers) may want to
consider non-surgical treatment. However, with small fenestra
surgery, the risk of prolonged postoperative dizziness is very
low (Albera et al., 2004).

Known endolymphatic hydrops is an absolute


contraindication. Stapedectomy in these patients will often lead
to a permanent profound sensorineural loss. Patients with
vertigo and poor speech discrimination may have
endolymphatic hydrops, and therefore are not surgical
candidates (Shea et al., 2004).

Tympanic membrane perforation with middle ear


infection is a contraindication to stapedectomy due to the
increased risk of suppurative labrynthitis. Stapedectomy in
these patients is delayed until the infection is treated, and the
tympanic membrane has been repaired (Lundy, 2006).

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Recent stapes operation (within 6-12 months) usually


precludes operating on the opposite ear, due to the risk of
delayed sudden profound hearing loss in the operated ear.
Similarly, operating on an only hearing ear is generally
contraindicated (Glasscock et al., 2003).

Patients with a substantial down sloping sensorineural


loss as part of a mixed loss usually have a poor speech
discrimation, which does not improve after surgery. In addition,
these patients are considered to have a "compromised cochlea"
with sensorineural loss often worsening after any degree of
intraoperative trauma (Conrad, 2004).

B- Cochlear implantation

As the cochlear implant has evolved since its inception,


so has the cochlear implant incision. The original incisions
were based on the concept that wide exposure of the internal
receiver stimulator (R/S) was necessary for placement and
fixation. It was believed that the incision should not cross the
implant or electrode array. Because of these concepts and the
early practice of “thinning” the flap over the magnet, initially
the majority of cochlear implant complications were flap
related, sometimes necessitating implant removal (Cohen et al.,
1991; Gibson et al., 1995). Despite the evolution in incision
design, the underlying principles of the incision have remained
the same including the following:

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1- Planned incision should not be near the internal R/S to


prevent potential extrusion or pain.

2- Blood supply must not be compromised.

3- Linea temporalis, mastoid tip, and spine of Henle should


be accessible without undue retraction.

The original, anteriorly based C-shaped postauricular


incision worked well with single- channel implants but had to
be modified to a larger C-shaped incision when multichannel
devices came into use due to the increased size of the R/Ss.
There was a high rate of device extrusion with the larger c-
shaped incisions. The c-shaped incisions preserved blood
supply from branches of the superficial temporal artery but
transected occipital artery branches. This incision was thought
to be incompatible with patients who had a pre-existing
postauricular incision due to compromised blood supply
(Hoffman et al., 1993). Because of the complications occurred
with the C-shaped incision, a newer incision was developed and
widely used in Europe “extended endaural incision” which is
smaller incision with lower risk. The endaural incision was
abandoned, however, due to a high incidence of skin
breakdown at the external auditory meatus and scalp numbness.
In Australia, an inferiorly based inverted U-shaped incision was
developed to replace the C-shaped incision. The inverted U-
shaped incision, which was later modified into an inverted J-
shaped incision, maximized the blood supply from both the

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superficial temporal and occipital arteries but still had similar


complications to the C-shaped incision, including scalp
numbness. A benefit of the inverted J-shaped incision was that
it could incorporate a pre-existing postauricular incision
(O’Donoghue et al., 2002). The inverted J-shaped incision has
been modified and shortened over time into the standard
postauricular incision, which is the most commonly used
incision at this time. Many centers (including New York
University [NYU] and Vanderbilt University) now use a
minimal access incision, which is a 2-cm to 4-cm, oblique,
straight postauricular incision. The advantages of this incision
are that there is minimal hair shaving, less tissue
elevation/manipulation, shorter operative times, faster healing,
less swelling, and the potential for earlier activation.
Disadvantages include decreased visibility, need for more skin
retraction, and limited access for drilling the bony well for the
implant (Flint et al., 2010; O’Donoghue et al., 2002).

Securing the cochlear implant

There are different methods of securing the internal R/S


and the cochlear implant electrode have been proposed because
device migration can lead to infection, extrusion and the need
for revision surgery.

Traditionally, the R/S has been secured using tie-down


sutures that were passed through monocortically drilled holes

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on each side of the R/S (Cohen et al., 2002). Other techniques


for securing the R/S include:

- Drilling two 4-mm titanium screws on either side of the


well and connecting them with a 3-0 nylon suture (Lee et
al., 2005)

- Applying polypropylene mesh over the R/S and securing


the mesh with titanium screws (Davis et al., 2004).

- Cementing the R/S with ionomeric bone cement (Rudel


et al., 1994).

- Securing the proximal portion of the electrode by placing


it in a drilled-out groove connecting the well and
mastoid, thus eliminating the need for fixation of any
type (Loh et al., 2008).

- Sewing the periosteum together over the implant


(Molony et al., 2010).

In 2009, Balkany and colleagues described the


temporalis pocket technique obviating drilling a well or fixation
of any type. The theory behind this technique is based on the
anatomic limitations of the temporalis pocket, which is
bounded “anteriorly by dense condensations of pericranium
anteriorly at the temporal-parietal suture, posteroinferiorly at
the lamboid suture, and anteroinferiorly by the bony ridge of
the squamous suture (Balkany et al., 2009).

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At Vanderbilt University and NYU, no effort is made to


fix the electrode at the fantail or at the facial recess. Attempts
are made to coil the redundant electrode array in the mastoid
cavity, usually securing the coil in against the tegmen (Mangus
et al., 2012).

A tight pocket technique for securing the R/S, snugged


up with or without periosteal sutures, is currently preferred.
This technique has shortened operative times, eliminating the
need for (potentially biofilm-forming) additional foreign
material. No significant R/S migrations have occurred at either
center (Mangus et al., 2012).

Manimally invasive techniques

Mastoidectomy with posterior tympanotomy approach

In 1961, Dr House introduced the mastoidectomy with


posterior tympanotomy approach (MPTA) for cochlear
implantation. Since then, the MPTA has stood the test of time
and become the most commonly used approach. As the name
implies a mastoidectomy is performed followed by a posterior
tympanotomy, which opens the facial recess exposing the
round window. Several techniques have been developed and
explored to try to minimize the extent of surgery needed to
place the implant and the risk to the facial nerve and chorda
tympani associated with the MPTA (House et al., 1976).

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Figure (8): Electrode in cochleostomy (arrow) with excess electrode


coiled in mastoid cavity (arrow head). Left ear (Postelmans et al., 2010).

Suprameatal route

In 1999, Kronenberg and colleagues (Kronenberg et al.,


2004) developed a technique that avoids a mastoidectomy
altogether and introduces the electrode into the middle ear via a
suprameatal route. This suprameatal approach is based on a
retroauricular tympanotomy approach to the middle ear in
which the facial nerve is protected by the body of the incus.
Drawbacks to the suprameatal approach include the following:
(Postelmans et al., 2010)

- The electrode is stretched during insertion into the


cochleostomy.

- Low-lying dura is a relative contraindication.

- A round window insertion and inferior cochleostomy is


difficult.

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- Additionally, the revision surgery rate is much higher


with this technique (Thomas Roland Jr, MD, personal
communication, 2011).

Endural approach

Another nonmastoidectomy technique uses an endaural


approach for access to the cochleostomy and a superoposterior
transcanal wall approach for the electrode. This endaural
approach, also known as the Veria operation, requires a special
perforator for drilling a direct tunnel and a safety electrode
forceps for inserting the electrode (Kiratzidis et al., 2002).

Minimal access incision techniques

Minimal access incision techniques (O’Donoghue et al.,


2002). Mann et al. (2006) have also been described. A
percutaneous cochlear implant technique that involves a single,
image-guided drill passed from the mastoid cortex through the
facial recess to access the cochlea has been developed. The
percutaneous cochlear implant technique uses an intraoperative
CT scan and three fiducial markers in the bone surrounding the
mastoid to plan a safe trajectory for the drill and has been
validated in vitro (Labadie et al., 2009; Balachandran et al.,
2010) and in vivo (Labadie et al., 2010). Access to correct
cochleostomy or round window insertion may also be limited
and the 3-D approach to scala tympani insertion is limited.

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Cochleostomy versus round window insertion

Because the human brain has the capability to integrate


both acoustic and high frequency electrically processed
information, (Von llberg C et al., 1999) much attention has
been paid to the possibility and benefit of electroacoustic
stimulation (EAS). The goal of EAS is to use the cochlear
implant for high-frequency loss and use a hearing aid to
improve the residual low-frequency hearing. Benefits of EAS
when compared with electrical stimulation only or acoustic
stimulation only include improved listening to speech in quiet,
in noise, or in competition with another speaker. Other benefits
of EAS include improvement in identification of melodies and
reception of musical sounds (Gantz et al., 2005; Kong et al.,
2005; Gfeller et al., 2006). With the benefits of EAS in mind,
many investigators have sought the least traumatic way to
insert the electrode array in hopes of preserving residual low
frequency hearing.

Traditional Cochleostomy Technique

- The traditional way to drill the cochleostomy is through


the promontory anterior and inferior to the round window
membrane using a 1-mm to 1.5-mm diamond burr.

- The round window membrane is usually 1-mm to 1.5-


mm inferior to the stapes tendon (Flint et al., 2010).

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- If necessary, the round window niche is removed to


identify the round window. Meticulous drilling with a 1-
mm diamond burr is then used and continued until the
“blue” lining of the endosteum is visible, taking care to
avoid inadvertent penetration of the endosteum because
this may expose the inner ear to significant acoustic
trauma, up to 130 dB (Pau et al., 2007) The endosteum
is at the same level and is continuous with the round
window membrane.

- The size of the cochleostomy is determined by the size of


the electrode array, which ranges from 1.0 mm to 1.4 mm.

- Once the endosteum is exposed, great care is taken to


prevent bone dust or blood from entering into the
cochleostomy. Some centers encourage the use of
hyaluronic acid or dilute surgical-grade glycerin at this
point to prevent entrance of blood and bone dust (James et
al., 2005). These substances have a buoyant density greater
than bone dust and blood, thus preventing ingress to the
scala tympani.

- At this point, a straight pick is used to open the


endosteum and the electrode is inserted.

- Suction is prohibited at this stage to avoid loss of


perilymphatic fluid. Systemic and/or topical intratympanic
steroids may be used in hearing preservation.

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The use of the traditional cochleostomy approach in


combination with a short/hybrid electrode in patients with
residual low-frequency hearing has resulted in improved
hearing in noise and music perception (Gantz et al., 2006;
Gfeller et al., 2006). Although temporal bone studies have
shown that the basal turn structures can be damaged with the
traditional cochleostomy approach (Dahm et al., 2000; Briggs
et al., 2001), this approach is preferred by many surgeons to
avoid the complicated negotiation of the hook region of the
cochlea and initiate insertion at an appropriate angle up the
scala tympani in the proximal basal turn of the cochlea (pars
inferior).

Figure (9): Opened facial recess. (B, incus buttress; C, cochleostomy; E,


stapes; *, facial nerve). Left ear (Dahm et al., 2000).

The round window approach

The round window approach to electrode insertion has


gained much attention due to the potential for reduced damage

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to intracochlear structures, as demonstrated in by several


temporal bone studies (Roland et al., 2007).

To avoid insertion into the wall of the scala tympani, the


electrode is inserted into the round window at an
oblique/anterior angle to the surface. The electrode itself seals
the insertion incision, and further sealing is accomplished with
muscle and/ or periosteum.

Good visualization of the round window may be


achieved in most cases by removing the bony round window
niche with a 1-mm diamond as well as performing an adequate
facial recess with drilling away of the bone anterior to the
descending facial nerve over the stapedius muscle.

Use of the round window approach with a standard


electrode has resulted in preservation in residual low-frequency
hearing and the benefit of EAS in children (Skarzynski, 2007).
In addition to avoiding the potential trauma that the inner ear
experiences from the 130 dB produced from drilling the
traditional cochleostomy (Pau et al., 2007) the round window
approach may reduce postoperative vertigo (Skarzynski et al.,
2007; Todt et al., 2008).

Concerning the cochleostomy versus round window


debate, James and colleagues (James et al., 2006) concluded,
“it appears that the correct approach to opening the cochlea,
whether via the round window or. via an anterior-inferior

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cochleostomy, is vital to avoid basal trauma, whether a long or


short electrode is used.” Even if hearing is not preserved, it is
expected that minimally invasive techniques and preservation
of fine structures will optimize postoperative performance.

Postoperative radiograph/telemetry

The technique of (Mangus B, David S. Haynes, Thomas


Roland Jr, Alejandro Rivas and Betty S. Tsai) is to only obtain
intraoperative plain radiographs in unique or suspect cases.
Many centers, including the NYU group, routinely perform
intraoperative radiographs to verify the absence of tip rollover,
verify intracochlear insertion, and act as a baseline for
postoperative analysis should electrode extrusion be suspected.
Intraoperative CT scanning has recently been available at the
authors’ center. The authors have found the scanner useful for
complex cases of severe malformations or in cases of
significant osteoneogenesis. Scanning in the operating room
allows making intraoperative decisions regarding electrode
placement in difficult cases, eliminates the need for
postoperative scanning, and potentially reduces revision
surgical cases. Intraoperative fluoroscopy is commonly used by
the NYU team in cases of cochlear anomalies and obstructed
cochleas. This technique has been shown to prevent intrameatal
internal auditory canal (IAC) electrode insertions and verify an
insertional stop point in common cavities and hypoplastic
cochlea cases (Coelho et al., 2008; Fishman et al., 2003).
Neural-response telemetry and impedence testing obtained

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intraoperatively can also help confirm proper functioning of the


device and correct placement. Spread of excitation testing can
also detect electrode tip rollover. Intraoperative measurements,
fluoroscopy, and intraoperative radiographs may alert surgeons
to a malfunctioning or misplaced device allowing a surgeon to
replace the implant at that time, saving a patient from a future
operation.

Figure (10): Round window membrane where electrode will be placed.


Left ear (Fishman et al., 2003).

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Figure (11): Treatment algorithm of advanced otosclerosis (Merkus et


al., 2011).

Complications of cochlear implantation

Facial nerve stimulation (FNS) during CI use occurs with


some regularity in patients with advanced otosclerosis. FNS
may be a minor, self-resolving, or major complication that can
usually be resolved by reprogramming or deactivation of the
problematic electrodes in a multichannel CI. However, when
many electrodes require deactivation, the performance of the
device may be negatively impacted. In some cases, FNS cannot
be resolved through deactivation and reimplantation may be

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necessary (Polak M, et al 2006). FNS is thought to occur


because of current leaks, especially in cases where the electrode
array has been partially inserted. It has been asserted that
electrode design can influence the incidence of FNS, with
perimodiolar configurations less leaky and, therefore, less
likely to cause FNS. The effect of electrode design on FNS in
advanced otosclerosis has not been confirmed using rigorous
clinical trials (Flook, 2010).

The incidence of FNS has been reported to vary from 0.9%


to 14.9% in the general CI population. In a study that based its
findings on reports from a large database on cochlear
implantation, incidence of FNS was found to be 2.71% in adults
and 0.94% in children (Gold et al., 1998). FNS is most commonly
associated with otosclerosis, cochlear malformation, and temporal
bone fractures. Symptoms of FNS include tingling, visible facial
spasms, and facial pain (Seyyedi et al., 2013).

According to a relevant study, FNS, when it occurs,


begins on average approximately 6.8 months after cochlear
implantation (Rayner et al., 2003).

FNS affects many patients with advanced otosclerosis


who have undergone cochlear implantation (Fernandez-Vega
et al., 2008).

Flook and colleagues reported that 21 patients who had


undergone cochlear implantation with perimodiolar electrodes

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developed no FNS, but 6 of 14 (43%) patients with straight


electrodes did develop FNS (Flook, 2010).

In addition, Matterson and colleagues indicated that none of


24 patients who had undergone cochlear implantation with contour
electrodes experienced FNS, whereas 14 of 35 patients with
straight electrodes experienced FNS (Matterson et al., 2007).

Reprogramming or deactivation of the electrodes that


cause FNS can aid in the treatment of FNS. Another treatment
modality for refractory FNS after cochlear implantation is the
injection of botulinum toxin. Gold and colleagues reported the
benefit of oral fluoride treatment in cases refractory to the
previously described options (Gold et al., 1998).

Cochlear ossification or pericochlear hypodensity


findings on imaging can be associated with incorrect or partial
electrode placement (Marshall et al., 2005). As otosclerosis
progresses, demineralization can cause the formation of
cavitation around the cochlea with resultant perilymphatic
gusher during cochleostomy (Kabbara et al., 2015).

Device failure is the second most common complication


encountered in patients with CIs, Failure of the CI device is of
particular concern in children because they may not be able to
articulate problems with the device. A patient with a suspected
device failure often undergoes clinical, audiologic, and
radiographic workups in addition to device testing.

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Unfortunately, many of the patients will present with normal


results of device integrity testing. Once explanted, all devices
are sent to the manufacturer for ex vivo device analysis. Most
causes of device failure involve fracture of the casing and loss
of the hermetic seal (Marlowe, 2010).

Device migration complications range from


asymptomatic occurrences to a requirement of revision surgery
for explantation. Methods to secure the device adequately
include creation of a smaller or tighter perichondrial pocket, a
bony well for stabilization, or suture of the device to bone
(Black, 2011).

Surgical site infection is one of the complications that


worries the cochlear implant team. According to the literature,
the incidence of infection ranges from 1.7 to 16.6%. The
majority of the cases were managed with local wound care.
Debridement and primary closure were performed for the
treatment of partial necrosis and wound dehiscence (Ramos,
2006). Postoperative otitis media was attributed to wound or
flap infections, and some patients subsequently required
reimplantation (Ikeya et al., 2013).

Because meningitis is a potentially serious complication


after CI surgery, and may be fatal in some cases, the FDA
recommends use of prophylactic antibiotics preoperatively.
This recommendation will decrease the risk of both meningitis
and wound infection. Vaccination with pneumococcal

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conjugate vaccine (PCV13) and Haemophilus influenzae type


B (Hib) should be given prior to implantation, especially in
cases of inner ear anomalies with the aim of reducing the
incidence of meningitis (Qin et al., 2016).

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Methods 

METHODS
The current review followed the guidelines of preferred
reporting items for systematic reviews and meta-analysis
statement 2009 (PRISMA). The detailed steps of methods were
described elsewhere as well as PRISMA checklist.
Quality assessment:
The quality of relevant studies was assessed using NIH
quality assessment tool for observational cohort studies.
(“Study Quality Assessment Tools | National Heart, Lung, and
Blood Institute (NHLBI),” 2019) Regarding cohort studies,
each study was given a score out of 14 based on answering
each question (Yes= 1, No= 0, NA= 0). A score of 10-14
indicated a good quality article, 5-9 for fair, and 1-4 for poor
quality article. Regarding case series studies, total evaluation
score was 9, a score from 7-9 indicated good quality article,
whereas score from 4-6 for fair, and 1-3 for poor quality article.
Statistical analysis:
We made pairwise meta-analysis of our outcomes using
Comprehensive Meta-Analysis software (CMA version 3.9).
Odds ratio (OR) with the corresponding 95% confidence
intervals (95%CI) was also be calculated for categorical data.
While dichotomous variables with one group were assessed by
event rate and its corresponding 95%CI. A fixed-effects model
was used when there was no heterogeneity. Heterogeneity was

71
Methods 

assessed with Q statistics and I2-test considering it significant


with I2 value > 50% or P-value < 0.10.

72
Results 

RESULTS
Literature search and study characteristics:
Electronic search using the search terms: (Otosclerosis
OR Stapedectomy OR (Stapes surgery) OR Stapedotomy OR
(Cochlear implantation) OR (cochlear implant)) AND (far
advanced otosclerosis) in different combinations, yielded
hundreds of articles from which 51 articles met our criteria,
from seven databases (Figure 12). After duplicates removal, 35
articles were screened in title/abstract screening, while 30
articles were screened in full text screening for inclusion.
Finally, 28 articles were included finally in qualitative analysis
and 24 in quantitative meta-analysis (Figure 12). The manual
search resulted in no additional studies. online databases
included; PubMed, SCOPUS, Web of science, Cochrane
Central Register of Controlled Trials, (CENTRAL), Science
Direct, and Google Scholar. Detailed characteristics of the
included studies are shown in (Table 1).

73
Results 

Figure (12): PRISMA flow diagram of the search and review process

74
Results 

Risk of bias assessment:


With regard to quality assessment, from 28 studies, 19
were evaluated with good quality, eight were fair, and one was
poor (Table 1).
Outcomes:
1.1. Satisfaction:
Meta-analyses of relevant studies showed that CI
approach was significantly higher satisfaction ratio than
stapedectomy in patients with far-advanced otosclerosis [Odds
ratio = 0.243, 95% CI (0.080–0.739), p-value=0.013] (Figure
13).
Fixed model was used due to absence of heterogeneity
with I^2<0.001 and P-value=0.791.

Figure (13): Meta-analysis for satisfaction regarding stapedectomy VS CI

75
Results 

1.2. Satisfaction rate:


Meta-analyses of relevant studies showed that CI was
significantly higher satisfaction rate than stapedectomy in
patients with far-advanced otosclerosis [Event rate = 86.3%,
95% CI (55.6%–96.9%), p-value=0.026]. While satisfaction
rate of stapedectomy was [Event rate = 69.5%, 95% CI
(55.2%–80.8%), p-value=0.009] (Figure 14).
Random model was used due to presence of
heterogeneity with I^2=65.955 and P-value<0.001.

Figure (14): Meta-analysis for satisfaction rate

76
Results 

2. Ability to use hearing aids after stapedectomy:


Meta-analyses of relevant studies showed that
stapedectomy had significant high rate for ability to use hearing
aids after surgery in patients with far-advanced otosclerosis
[Event rate = 71.3%, 95% CI (54%–84%), p-value=0.017]
(Figure 15).
Random model was used due to presence of
heterogeneity with I^2=50.183 and P-value=0.111.

Figure (15): Meta-analysis for ability to use hearing aids after


stapedectomy

3. Any postoperative complications:


Meta-analysis of relevant studies showed that CI had
significant lower rate of any postoperative complications in
patients with far-advanced otosclerosis [Event rate = 13.6%,
95% CI (9.7%–18.6%), p-value<0.001]. While any
postoperative complications rate of stapedectomy was [Event
rate = 21.5, 95% CI (12.7%–34%), p-value<0.001] (Figure 16).
Fixed model was used due to presence of heterogeneity
with I^2=13.440 and P-value=0.296.

77
Results 

Figure (16): Meta-analysis for any postoperative complications rate

4. Difficult access to area of cochleostomy:


Meta-analysis of relevant studies showed that CI had
significant low rate of difficult access to area of cochleostomy
in patients with far-advanced otosclerosis [Event rate = 24.9%,
95% CI (13.4%–41.4%), p-value=0.004] (Figure 17).
Random model was used due to presence of
heterogeneity with I^2=78.836 and P-value<0.001.

78
Results 

Figure (17): Meta-analysis for difficult access to area of cochleostomy


rate in CI

5. Difficult insertion of electrode bundle:


Meta-analysis of relevant studies showed that CI had
significant low rate of difficult insertion of electrode bundle in
patients with far-advanced otosclerosis [Event rate = 14.8%,
95% CI (10.2%–21%), p-value<0.001] (Figure 18).
Fixed model was used due to presence of heterogeneity
with I^2=41.675 and P-value=0.071.

Figure (18): Meta-analysis for difficult insertion of electrode bundle rate


in CI

6. Dysgeusia:
Meta-analysis of relevant studies showed that CI had
significant lower rate of dysgeusia in patients with far-
advanced otosclerosis [Event rate = 1.4%, 95% CI (0.1%–
18.7%), p-value=0.003]. While dysgeusia rate of stapedectomy

79
Results 

was [Event rate = 3.6%, 95% CI (0.5%–21.4%), p-


value=0.001] (Figure 19).
Fixed model was used due to presence of heterogeneity
with I^2=22.158 and P-value=0.267.

Figure (19): Meta-analysis for dysgeusia rate

7. Tinnitus:
Meta-analysis of relevant studies showed that CI had
lower rate of tinnitus in patients with far-advanced otosclerosis
[Event rate = 32.7%, 95% CI (17.1%–53.4%), p-value=0.099].
While tinnitus rate of stapedectomy was [Event rate = 52.5%,
95% CI (13.3%–88.8%), p-value=0.001] (Figure 20).
Fixed model was used due to presence of heterogeneity
with I^2=22.158 and P-value=0.267.

Figure (20): Meta-analysis for tinnitus rate

80
Results 

8. Vertigo:
Meta-analysis of relevant studies showed that
stapedectomy had significant lower rate of vertigo in patients
with far-advanced otosclerosis [Event rate = 8.8%, 95% CI
(3.5%–20.3%), p-value<0.001]. While vertigo rate of CI was
[Event rate = 12.8%, 95% CI (2.3%–47.8%), p-value=0.040]
(Figure 21).
Fixed model was used due to presence of heterogeneity
with I^2=22.158 and P-value=0.267.

Figure (21): Meta-analysis for vertigo rate

9. Recognition of monosyllables:
Meta-analysis of relevant studies showed that
stapedectomy had a higher significant mean for recognition of
monosyllables in patients with far-advanced otosclerosis [Mean
= 34%, 95% CI (16.4%–51.6%), p-value<0.001]. While mean
recognition of monosyllables of CI was [Mean = 28.1%, 95%
CI (5.1%–61.3%), p-value=0.097] (Figure 22).
Random model was used due to presence of
heterogeneity with I^2=97.200 and P-value<0.001.

81
Results 

Figure (22): Meta-analysis for mean recognition of monosyllables

10.Recognition of disyllables:
Meta-analysis of relevant studies showed that
stapedectomy had a higher significant mean for recognition of
disyllables in patients with far-advanced otosclerosis [Mean =
56.6%, 95% CI (45.2%–68%), p-value<0.001]. While mean
recognition of disyllables of CI was [Mean = 55.2%, 95% CI
(21.4%–89%), p-value=0.001] (Figure 23).
Random model was used due to presence of
heterogeneity with I^2=98.095 and P-value<0.001.

Figure (23): Meta-analysis for mean recognition of disyllables

82
Results 

11.Recognition of phrases:
Meta-analysis of relevant studies showed that CI had a
high significant mean for recognition of phrases in patients
with far-advanced otosclerosis [Mean = 65.7%, 95% CI
(49.1%–82.4%), p-value<0.001] (Figure 24).
Random model was used due to presence of
heterogeneity with I^2=98.444 and P-value<0.001.

Figure (24): Meta-analysis for mean recognition of phrases

83
Results 

12.Facial electrical stimulation:


Meta-analysis of relevant studies showed that CI had a
significant low rate of facial electrical stimulation in patients
with far-advanced otosclerosis [Event rate = 12.4%, 95% CI
(8.4%–18%), p-value<0.001] (Figure 25).
Fixed model was used due to presence of heterogeneity
with I^2=7.630 and P-value=0.371.

Figure (25): Meta-analysis for facial electrical stimulation rate in CI

84
Results 

13.Family history of otosclerosis:


Meta-analysis of relevant studies showed that CI had
high rate of family history of otosclerosis in patients with far-
advanced otosclerosis [Event rate = 60.7%, 95% CI (43.4%–
75.7%), p-value=0.224] (Figure 26).
Fixed model was used due to presence of heterogeneity
with I^2=11.763 and P-value=0.339.

Figure (26): Meta-analysis for family history of otosclerosis rate

85
Results 

14.Hearing loss after surgery:


Meta-analysis of relevant studies showed that CI had
significant lower rate of hearing loss after surgery in patients
with far-advanced otosclerosis [Event rate = 16.4%, 95% CI
(4.9%–42.9%), p-value=0.017]. While hearing loss rate after
surgery of stapedectomy was [Event rate = 21.2%, 95% CI
(11.1%–36.7%), p-value<0.001] (Figure 27).
Random model was used due to presence of
heterogeneity with I^2=53.150 and P-value=0.046.

Figure (27): Meta-analysis for hearing loss rate after surgery

86
Results 

15.History of previous stapedectomy:


Meta-analysis of relevant studies showed that
stapedectomy had higher rate of history of previous
stapedectomy in patients with far-advanced otosclerosis [Event
rate = 87.5%, 95% CI (46.3%–98.3%), p-value=0.069]. While
history of previous stapedectomy rate of CI group was [Event
rate = 57.9%, 95% CI (40.3%–73.7%), p-value=0.380] (Figure
28).
Random model was used due to presence of
heterogeneity with I^2=69.259 and P-value<0.001.

Figure (28): Meta-analysis for history of previous stapedectomy rate

87
Results 

16.Retrofenestral extension:
Meta-analysis of relevant studies showed that
stapedectomy had a significant lower rate of retrofenestral
extension in patients with far-advanced otosclerosis [Event rate
= 3.6%, 95% CI (0.5%–21.4%), p-value=0.001]. While
retrofenestral extension rate of CI was [Event rate = 15.8%,
95% CI (6.7%–33%), p-value=0.001] (Figure 29).
Random model was used due to presence of
heterogeneity with I^2=57.063 and P-value=0.040.

Figure (29): Meta-analysis for retrofenestral extension rate

88
Results 

17.Revision surgery:
Meta-analysis of relevant studies showed that CI had a
significant lower rate of revision surgery rate in patients with
far-advanced otosclerosis [Event rate = 8.1%, 95% CI (4.3%–
14.9%), p-value<0.001]. While revision surgery rate of
stapedectomy was [Event rate = 16.4%, 95% CI (7.9%–31%),
p-value<0.001] (Figure 30).
Random model was used due to presence of
heterogeneity with I^2=51.023 and P-value=0.031.

Figure (30): Meta-analysis for revision surgery rate

89
Results 

18.Postoperative Pure tone average:


Meta-analysis of relevant studies showed that CI had a
better mean for pure tone average in patients with far-advanced
otosclerosis [Mean = 29.1 dB CI (29.1–32.5), p-value=0.096].
While mean pure tone average of stapedectomy was [Mean =
52.3, 95 dB CI (39.9–64.8), p-value<0.001] (Figure 31).
Random model was used due to presence of
heterogeneity with I^2=98.850 and P-value<0.001.

Figure (31): Meta-analysis for mean pure tone average

90
Results 

19.Speech reception threshold:


Meta-analysis of relevant studies showed that
stapedectomy had a higher significant mean for speech
reception threshold in patients with far-advanced otosclerosis
[Mean = 62.6%, 95% CI (33.6%–91.5%), p-value<0.001].
While mean speech reception threshold of CI was [Mean =
43.7%, 95% CI (30.5%–56.9%), p-value<0.001] (Figure 32).
Random model was used due to presence of
heterogeneity with I^2=98.130 and P-value<0.001.

Figure (32): Meta-analysis for mean speech reception threshold

91
Results 

Table (1): Characteristics table for patients in the included articles


Age (years)
Sample Follow-up Sex (Female) QA
Reference ID Type of Study Type of surgery [mean
size period (years) n (%) tool
(SD)]
Castillo/2014/Spain Prospective Cohort 17 0.5, 1, 2, 3, 5 CI 55.6 13 (76.5) Good
Lopez/2006/Spain Prospective Cohort 30 5.8 CI 51 (41) 24 (80) Good
Dumas/2018/USA Retrospective Cohort 35 1 CI 59 (8) 16 (45.7)
Good
Psillas/2007/Greece Retrospective Cohort 5 NA CI 60.2 3 (60)
Luca/2021/Italy Retrospective Cohort 11 0.5, 1, 3 Stapedectomy 69.5 5 (45.5) Good
7 Stapedectomy 70.9 3 (42.9) Good
Calmels/2007/France Retrospective Cohort 2 months
7 CI 63.9 5 (71.4) Fair
Redfors/2011/Sweden Retrospective Cohort 65 30 Stapedectomy NA NA Good
Dejaco/2018/Austria Case report 1 31 days CI NA NA Poor
Frattali/1992/USA Retrospective Cohort 9 NA Stapedectomy NA NA Fair
Ghonim/1997/Egypt Retrospective Cohort 8 NA Stapedectomy 49 (4.75) 3 (37.5) Fair
Glasscock/1996/USA Retrospective Cohort 15 0.25, 1 Stapedectomy 62 8 (53.3) Good
HEINING/2017/UK Retrospective Cohort 28 NA Stapedectomy NA NA Fair
Lurato/1985/Italy Retrospective Cohort 34 1 Stapedectomy NA NA Good
32 1 Stapedectomy
Kabbara/2014/France Retrospective Cohort 59 (11.9) NA Good
34 1 CI
Khalifa/1998/Egypt Retrospective Cohort 8 NA Stapedectomy 61 5 (62.5) Fair
Lachance/2012/Canada Retrospective Cohort 16 1 Stapedectomy NA NA Good
Lovato/2020/Italy Retrospective Cohort 5 1 CI 59.6 3 (60) Good
Marshall/2005/Canada Retrospective Cohort 25 0.5, 1 CI 4.7 NA Good
Mosniera/2007/France Retrospective Cohort 16 0.5, 8 CI 61 9 (56.3) Good
Rotteveel/2004/UK Retrospective Cohort 53 NA CI NA NA Fair
Rotteveel/2009/UK Retrospective Cohort 53 NA CI NA NA Fair
Ruckenstein2001/USA Retrospective Cohort 8 1 CI 62 2 (25) Good
Sainz/2009/Spain Prospective Cohort 15 6 CI 32.6 (8.6) NA Good
Semaan/2012/USA Retrospective Cohort 30 1 CI 72 (5) 16 (53) Good
8 Stapedectomy 56 7 (36.8)
Bajin/2020/Turkey Retrospective Cohort 2.3 Good
13 CI
Vashishth/2017/Italy Retrospective Cohort 38 4 CI 59.72 11 (29) Good
Vincent/2006/UK Prospective Cohort 2525 14 Stapedectomy NA NA Good
Wiet/1987/USA Case report 2 NA CI NA NA Fair

92
Discussion 

DISCUSSION
The treatment of far advanced otosclerosis has evolved
over the past 20 years with the improvement in hearing aid
technology and the advent of cochlear implantation as an
alternative of treatment. Nevertheless, cochlear implantation is
not without disadvantages. It requires implication in a
reeducation program, is more invasive and is much more
expensive than a simple stapedotomy. Moreover, the electrical
stimulation provided through a CI is less than optimal, with a
limited dynamic range and a lack of ability to deliver detailed
spectral information that helps define the pitch and timbres of
music. This leads to poor music appreciation, extremely poor
melody perception, and worse discrimination in loud
environments (Kabbara et al., 2015).

The challenge in FAO is to determine which patients will


no longer benefit from acoustic stimulation and will require
cochlear implantation. In addition, multiple factors can affect
the surgical decision such as the degree of residual hearing in
the contralateral ear, duration of hearing deprivation, and
patient preference (Bajin et al., 2020).

The results of this meta-analysis show that comparing the


outcomes and complications of cochlear implantation and
stapes surgery in far advanced otosclerosis patients have
different results, in some of them there was a highly favorable

93
Discussion 

and recommended procedure than the other, other results


declared no significant difference in postoperative outcomes.

This meta-analysis showed that CI had a significantly


higher satisfaction ratio (p-value= 0.013) & rate (P-value=
0.009) than stapedectomy in patients with far advanced
otosclerosis.

Many patients who have suffered hearing difficulty for


years tend to choose CI-the fastest route to hearing-even if that
meant unilateral hearing. Six patients (31%) made such a
decision (Bajin et al., 2020).

Published Speech Recognition (SR) scores of


Otosclerosis patients with CI range from 45% to 98%. Many
studies report better hearing results with CI than with stapes
surgery (Roland, 1197; Van loon et al., 2014; Calmels et al.,
2007; Sainz et al., 2009).

Lahance et al reported that they obtained a 94%


satisfaction and telephone use rate after the surgery of 1 (13
patients) or 2 ears (3 patients). Thirteen patients were no longer
candidates for cochlear implant after stapes surgery, and the
information was impossible to obtain for 1 patient who spoke
neither English nor French. This gives a success rate of 87%.
The 2 patients who remained candidates according to
audiologic criteria were not interested in receiving an implant
(Lahance et al., 2012).

94
Discussion 

Postoperative ability to use hearing aid was very high in


relevant studies (event rate= 71.3%) and this confirms that
stapes surgery can give very good results in patients with FAO.
This result is consistent with the literature.

Patients may be treated with stapedotomy and then fitted


with hearing aids. Stapedotomy is a relatively simple, safe and
low-cost procedure and can give acceptable outcomes in FAO.
Stapes surgery enables the acoustic stimulation of cochlea,
providing a more natural sound perception than electrical
stimulation of CI and does not require a long
rehabilitation/fitting period which is crucial for CI users to
benefit (Sainz et al., 2009; Heining et al., 2017).

lahance et al reported that the patients in their study only


needed adjustment of their hearing aids after surgery, and re-
education was therefore very simple for them, which is an
advantage over cochlear implant especially in the older
patients. Their study was designed to determine if stapes
surgery gave enough improvement, so these patients would no
longer need a cochlear implant (Lahance et al., 2012).

According to our meta-analysis CI has lower rate of any


postoperative complications than stapedectomy in FAO
patients. Access to area of cochleostomy was less difficult in CI
(even rate= 24.9%), CI had also a significant low rate of
difficult insertion of electrode bundle (even rate= 14.8%).

95
Discussion 

Placing a CI in a cochlea with a degree of ossification is


certainly a challenge, however, is not a contraindication. Fayad
et al reported that the presence of cochlear ossification does not
affect the CI audiological results. In Castillo’s study they had
one case of cochlear ossification previously suspected by
radiology out of 17 patients with FAO who were fitted with a
CI. The patient also had partial insertion of the electrode bundle
induced by ossification, with long-term results similar to the
other patients (Castillo et al., 2014).

Cochlear ossification has been postulated as a cause of facial


electrical stimulation, which is another common complication in
placing a CI in FAO. Cochlear implantation in FAO studies report
facial electrical stimulation of 7% to 75%, with an average of 20%
(Rotveel et al., 2004; Berrettini et al., 2004).

Despite the theoretic positive aspects already mentioned,


the use of a cochlear implant in a patient affected by far-
advanced otosclerosis can present certain difficulties given that
the evolution of the illness can produce phenomena of
obliteration at the different levels of the cochlea (Fayad et al.,
1990). However, in Rama-Lopez study, the degree of
obliteration does not reach the same intensity as that observed
in cases of ossification after ossifying labyrinthitis. As such,
they were able to achieve the insertion of the electrodes without
compromising in any way the subsequent results. It is also
possible to obtain information from the HRCT regarding the
permeability of the cochlea such that in the four cases that

96
Discussion 

presented a degree of cochlear obliteration, this could be seen


in the radiologic images. This obliteration was confirmed
during the surgery and they were able to perform the
cochleostomy (Rama-López et al., 2006).

Rotteveel and colleagues reported that the insertion of a


multichannel electrode array was problematic for 10 of 53 of
the patients with advanced otosclerosis in their study. Three of
these patients experienced misplacement, and 7 of them
experienced partial insertion of the electrode array (Rotteveel et
al., 2004). Kabbara and colleagues stated that advanced
otosclerotic lesions can result in significant difficulties during
surgery. On the other hand, Semaan and colleagues revealed
that complete electrode insertion was achieved in all 34 of the
patients in their study with advanced otosclerosis who had
received CIs (Kabbara et al., 2015).

Dysgeusia was of a lower rate in patients underwent CI


(even rate= 1.4%) than those who had stapes surgery (even
rate= 3.6). Tinnitus also occurred at a lower rate in CI (even
rate= 32.7) than in stapes surgery (even rate= 52.5%), regarding
vertigo results were close in both CI and stapes surgery but it
was of a lower rate in stapedectomy (event rate= 8.8%) than CI
(event rate= 12.8%) this difference in event rate was due to
varying number of patients in each study.

In their series, Sainz and colleagues reported that 2 of 15


(13.3%) patients with advanced otosclerosis experienced

97
Discussion 

progressive episodes of intense tinnitus, dizziness, and headaches


after cochlear implantation. All patients were treated with the
deactivation of the offending electrodes (Sainz et al., 2009).

In another series, Semaan and colleagues reported that 2


of 30 patients with advanced otosclerosis experienced new
tinnitus after cochlear implantation (Semaan et al., 2012).

Vestibular problems can also be identified in patients


with advanced otosclerosis. Vertigo can be recurrent,
positional, or spontaneous. Benign positional vertigo may
be associated with advanced otosclerosis, and vestibular
problems can be detected regardless of cochlear involvement
(Cureoglu et al., 2010).

Our meta-analysis revealed that Monosyllables and


disyllables recognition was surprisingly of a higher mean in
stapedectomy (mean= 34%, 56.6%) than in CI (mean= 28.1%,
55.2%), statistically monosyllables and disyllables recognition
is better in stapedectomy but in fact there is nearly no
significant difference but maybe they are better in CI in some
studies, this results were due to the varying number of studies
included in each procedure and the varying number of patients
in different studies. Regarding recognition of phrases, it was
only mentioned in CI studies and it was of a high significant
mean (65.7%).

98
Discussion 

In Calmel’s study, after stapedotomy surgery, seven


patients’ hearing was improved (64%), four patients (36%)
have a disyllabic word recognition at 70 dB of /60%, five
patients (45%) have a percentage of global satisfaction/50%
and three of them can use the telephone. Some studies have
also reported results of stapedotomy for far advanced
otosclerosis. Shea et al. demonstrated that 42% of 60 patients
with absent preoperative bone conduction thresholds had
measurable thresholds after the surgery and the hearing was
restored to an audible level (Calmels et al., 2007).

One of the most important complications of CI is Facial


electrical stimulation, in our meta-analysis it was of a low rate
in FAO patients who underwent CI with even rate (12.4%).

FNS is a well-known adverse effect of cochlear implants.


In far advanced otosclerosis, the reported incidence of FNS in
cochlear implant recipients varies widely from 17% to 78%.
According to Psillas 2007, 4 (2.3%) out of 170 cochlear
implantees experienced FNS and among them one patient had
otosclerosis. FNS has been reported to occur as a result of an
electrical shunt between the cochlear ducts and the facial nerve.
The massive bone resorption in otosclerosis and a gradual
thinning of the bone between the facial nerve and cochlea
might contribute to this shunt (Bigelow et al., 1998).

Marshall and colleagues compared 30 patients with


advanced otosclerosis treated with CIs and 30 patients without

99
Discussion 

otosclerosis who had CI as control. FNS occurred in 17% of the


patients in the otosclerotic group, whereas none of the patients
in the control group had FNS. Treatment of these patients
required deactivation of one or more of the electrodes in their
implants. This study also showed that there was no difference
between the radiologic extent of otosclerosis and the
performance with CIs. The risk for FNS in grade 3 (diffuse
confluent retrofenestral involvement) advanced otosclerosis is
significant and may be related to electrode configuration
(Marshall et al., 2005).

In ruckenstein’s study, in studying a specific group of


patients with otosclerosis does confirm a higher incidence of
facial nerve stimulation in this patient group. In both the
patients who experienced facial nerve stimulation in this group,
deactivation of the offending channels was done without
detriment to their auditory function. Neither of these patients
has required adjunctive treatment to manage this problem, such
as fluoride administration (Ruckenstein et al., 2001).

Heining et al reported that one patient (3 per cent), who


had tinnitus, hemifacial spasm, balance disturbance and a dead
ear, was diagnosed with a middle-ear granuloma which had
eroded into the inner ear and facial nerve. This was removed
via petrosectomy and blind sac closure of the ear. Post-
operatively, the facial nerve symptoms resolved and normal
facial nerve function was maintained (Heining et al., 2017).

100
Discussion 

In Rama-López’s study no FNS was noted among 30


patients and their strategy consisted of inactivation of the
implicated electrodes. However, this may result in decreased
performance if too many electrodes must be inactivated. A
technique called variable mode programming can also solve
FNS, while the non-offending electrodes receive normal pulses,
the offending electrodes receive wider pulses (Rama-López et
al., 2006).

Most of FAO patients who underwent CI had a family


history of otosclerosis with event rate= (60.7%).

In Rama-Lopez’s 2006 study, the incidence of familial


precedents of otosclerosis was in the region of 35% and 75% of
these patients offered a radiologic aid to diagnosis. The
proportion of patients with radiologic signs did not differ with
respect to those patients without any familial precedents in
contrast to what has been indicated in other series of patients in
which a greater incidence of indicative radiologic signs was found
in those patients with a family history (Rama-Lopez et al., 2006).

Regarding postoperative hearing loss, CI had a lower rate


of hearing loss than patients of FAO who had a stapes surgery
with even rates (16.4%, 21.2%) respectively.

Heining et al. stated that Five patients (17 per cent) had
hearing loss that satisfied criteria for cochlear implantation
after stapes surgery, three of these patients underwent cochlear

101
Discussion 

implantation. One patient (3 per cent) had a poor audiological


outcome, with no hearing gain; they did not pursue any further
intervention and were discharged following surgery (Heining
et al., 2017).

Bajin et al stated that the only intraoperative


complication in the stapedotomy group was perilymph oozing
in one patient that led to total sensorineural hearing loss and
eventually necessitated CI (Bajin et al., 2020).

The need of a revision surgery was higher in


Stapedctomy with (even rate=16.4%), and was much lower in
CI with (even rate=8.1%).

According to Heining et al. Twenty-two patients (76 per


cent) had successful primary stapes surgery, with hearing
improvement, and did not undergo further surgery. Two
patients (7 per cent) required revision of stapes surgery; both
had a successful outcome, with audiological improvement
(Heining et al., 2017).

In Baijin’s study, CI was performed in 13 patients (65%).


Seven had prior failed stapes surgeries and six chose CI as the
initial procedure. Full electrode insertion was successful in all
cases (Bajin et al., 2020).

The performance after stapedotomy can sometimes be


disappointing because of the difficulty in predicting the
outcome of stapedotomy in patients with FAO. All patients that

102
Discussion 

benefitted from stapedotomy were later operated on their other


ears and also had satisfying results (Roland, 1997; Sainz et al.,
2009).

Some authors (Iurato et al., 1992; Glasscock et al.,


1996) declared that they prefer to perform a stapedectomy once
a patient is disgnosed as Far advanced otosclerosis patient. In
Rama-Lopez’s study, they had a different opinion, in their
study, 22 of the patients had had a stapes surgery but they
generally presented a severe, profound sensorineural
hypoacusis with little discrimination of the spoken word, which
was disappointing. Rama-Lopez also did not consider the use of
hearing aids as a definitive treatment because they did not
improve these results (Rama-Lopez et al., 2006). Ruckenstein
et al. (2001), also prefer not considering Stapes surgery or
hearing aids as primary treatment, they consider that this group
of patients should be included as adequate candidates for a
cochlear implantation.

In ruckenstein’s study, one patient underwent a primary


stapedectomy and another a revision stapedectomy before
cochlear implantation, and neither derived any benefit from these
procedures. In the other cases in his series, primary or revision
stapedectomies were not attempted because these patients
exhibited no clinical or audiometric response to bone stimulation.
On the basis of the experience described here, it might be
reasonable to attempt stapedectomy in patients with documented
otosclerosis who have a non-vibrotactile response to bone

103
Discussion 

stimulation, i.e., a response at least at the limits of the equipment


at frequencies above 500 Hz. However, given the excellent
performance of these patients with cochlear implants, they
certainly should be considered as strong candidates for cochlear
implantation if stapedectomy fails (Ruckenstein et al., 2001).

Regarding postoperative pure tone average, CI had a better


mean for PTA in patients with FAO (mean= 29.1 dB), while the
mean of PTA in unaided stapes surgery was (52.3 dB). This is a
very important item in comparison between CI and stapedectomy.
Speech recognition threshold in our meta-analysis is statistically
of a higher mean in FAO patients who had stapes surgery
(62.6%), and of a lower mean in CI (43.7%). Relevant studies had
a different number of patients, so although this rate seemed more
often associated with CI failures, the difference between the two
subgroups was not found to be significant.

A speech recognition score of 30% on an open set


dissyllabic word test is considered an appropriate upper limit for
preoperative performance in determining cochlear implant
candidacy. Patients in the stapedotomy group showed a
statistically significant postoperative improvement of speech
discrimination after surgery (p B/0.001), and 36% of them had
useful hearing after the surgery with a well fitted hearing aid
(Calmels et al., 2007).

When Bajin et al compared the SR test scores of 13


patients who had CI and six patients who benefitted from

104
Discussion 

stapedotomy and hearing aids, there was no statistically


significant difference (p=0.368). Similarly, there was no
difference when they compared the SR results of the six
patients who preferred CI as the primary treatment and the
eight patients who had stapedotomy as the primary treatment
and back-up CI if necessary (p=0.414) (Bajin et al., 2020).

In Lovato’s 2020 study, one years after unilateral CI, the


mean SRT and WRS of the five FAO considered patients were
respectively 36 dB and 94%; these results are consistent with
those available in the literature.

In Rama-lopez’s study, they observed that in the


bysilables test and CID sentence tests how from some fairly
basic levels of discrimination before the implantation,
significant improvement differences were obtained in a short
period of time. Moreover, these results that were maintained
stable from the sixth month onward (Rama-López et al., 2006).

In Psillas’s 2007 study, Otosclerotic patients achieved


excellent speech perception after implantation and obtained
almost similar results to those achieved by the control group.
According to their protocol, speech perception skills of the
otosclerotic patients gradually improved with time in a period
of one year. All these patients were able to converse over the
telephone (Psillas et al., 2007).

Twelve months after surgery, the mean speech


perception scores in patients with otosclerosis (disyllable words
105
Discussion 

70 ± 21%, sentences 89 ± 11%, n 9) were similar to those of


patients with other causes (disyllable words 74 ± 28%,
sentences 86 ± 22%, n 55). the recent challenge was to restore
binaural hearing. In patients with non-measurable bone
conduction levels on both ears and no benefit from hearing
aids, they proposed a bilateral implantation. The follow-up was
6 months for 1 patient and 1 year for 2 patients. All 3 patients
reported better speech perception with bilateral implants
compared to a unilateral implant. especially in noisy
environments. In case of measurable bone conduction on one
side, a stapedotomy on one side and a cochlear implantation on
the other side were performed during the same surgical time in
2 patients. Stapedotomy improved the hearing level in both
patients, who obtained serviceable hearing with a hearing aid.
Six months after surgery, binaural hearing improved disyllable
word and sentence scores, compared to performances with a
cochlear implant alone (Mosnier et al., 2007).

Unfortunately, the exact method of testing is often


unclear. Even with comparable methods of testing, variations
are present. Glasscock et al., Khalifa et al., and Calmels et al.
described the poorest postoperative stapes surgery outcomes
with mean speech recognition of 33%, 43%, and 54%,
respectively. A possible explanation for these poor results is the
poor preoperative performance of the majority of patients in
these studies (67%-80% had immeasurable speech recognition).
Most of these patients did not use hearing aids, and it is likely
that their hearing loss was present for a long period, increasing
the chance of a poor postoperative performance. On the other
106
Discussion 

hand, Iurato et al. also included patients with a mean


preoperative speech recognition of less than 10%, yet they
reported good postoperative speech recognition scores. This
might be explained by the study design because they presented
three individual patients in which stapedotomy was very
successful instead of a group of patients, thereby possibly
introducing selection bias.

Disyllabic WRS at 60 dB were obtained 12 months


postoperatively with a well-fitted ipsilateral hearing aid for the
stapedotomy group and without any contralateral hearing aid
for CI patients. All groups showed significant improvement
after the surgery (Wilcoxon test, p G 0.001). The mean WRS
for Groups I (Primary stapedectomy), II (Primary CI), and III
(secondary CI) were 50.6, 75, and 72%, respectively. The
cochlear implant groups (II + III) had better overall results with
a mean WRS of 72.8% (T19) compared to 50.6% (T34) for the
stapedotomy group (p G 0.01). The success rate, defined as a
postoperative WRS greater than 50% with a well fitted
ipsilateral hearing aid, was also compared. Nineteen patients in
the stapedotomy group (60%) had a successful outcome
compared to 29 patients with cochlear implantation (85%). The
success rate was significantly higher in CI users (Groups II and
III) (Fisher’s exact test, p = 0.027) (Kabbara et al., 2015).

In Castillo’s study, 100% of patients implanted with a


diagnosis of FAO improved their hearing with a CI. Patients with
FAO had certain parameters with better yields than the UOHL
(unknown origin hearing loss) group (PTA and monosyllables in

107
Discussion 

general, ant particularly PTA in a year, monosyllables at 6 months


and a year, and disyllables at 6 months); they believe that this
may be due to a small size sample, or predictable behavior of this
pathology compared with other of unknown cause and behavior
(Castillo et al., 2014).

Both Berrettini et al. and Calmels et al. demonstrated that


CI leads to statistically better mean speech recognition scores
than stapedotomy when the whole group of CI patients was
compared with the whole group of stapedotomy patients.
However, stapedotomy yielded excellent results in a
considerable subgroup (in four of six and four of seven of the
patients). These successfully treated patients achieve mean
postoperative speech recognition scores of 80% to 82%, which
is comparable to or even better than the performance after CI
(Berrettini et al., 2004; Calmels et al., 2007).

Both stapedotomy and CI have different advantages and


disadvantages, and thus the treatment modality should be
tailored according to the patient’s history, clinical signs and
symptoms, audiological results (tuning fork tests, pure tone
audiogram, speech threshold testing, SR), HRCT findings,
ECoG/promontory stimulation and individual expectations.

108
Summary 

SUMMARY
Otosclerosis is a process of bone resorption of the
labyrinthine capsule followed by reparative deposition of new,
immature sclerotic bone. It usually occurs during the
postlingual period between the second and fifth decade of life.
The most commonly affected location is around the oval
window (fenestral otosclerosis), which results in conductive
hearing loss due to stapes footplate fixation. As it undergoes a
maturation process, the sclerotic bone increases in size and
depth (Güneri et al., 1996; Adrien et al., 2018).

There is no universally accepted definition for advanced


otosclerosis. Recently, the term advanced otosclerosis is used
when a patient with otosclerosis has severely decreased speech
recognition. Calmels and colleagues described advanced
otosclerosis by its audiologic and radiologic criteria. The
audiologic criteria for diagnosis was the detection of dissyllabic
words less than 30% of the speech discrimination (SD) score at
70 dB, with a well-equipped hearing aid and a blank audiogram
(Calmels et al., 2007).

Various authors have used different CT grading systems


for the classification of advanced otosclerosis. The CT grading
system of Rotteveel (Rotteveel et al., 2004), is partially based
on location and on the type of lesion: solely fenestral (grade1),
retrofenestral: double ring or halo effect (grade 2A), narrowed
basal turn (grade 2B) or both (grade 2C), and diffuse confluent

109
Summary 

retrofenestral involvement (grade3). Symons and Fanning


(Marshall et al., 2005), proposed a classification similar to
Rotteveel, except grade 2 is based on anatomic location instead
of the type of lesion: basal turn (2A), middle/apical turns (2B),
both basal and middle/ apical turns (2C).

In advanced otosclerosis there are three treatment options


to propose to the patient:

(1) No intervention and continue hearing aids, (2) stapes


surgery and hearing aid use, or (3) cochlear implantation (CI). In
some patients with advanced otosclerosis, the decision can be
difficult because of two factors. First, with mixed hearing loss it
is hard to predict the success rate of stapedotomy, especially if
compared to CI as an alternative intervention. Second, extensive
otosclerotic foci around the otic capsule can lead to surgical
complications during cochlear implantation (Liselotte et al.,
2004; Psillas et al., 2007; Ramsden et al., 1997).

Not only the success rate plays a role in the decision;


each intervention has specific advantages and disadvantages.
Stapes surgery is a relatively simple, safe, and lowcost
procedure that can accomplish very good results. Stapes
surgery is a suitable treatment option for patients with
advanced otosclerosis, and should be considered mandatory,
before offering cochlear implantation, for those with a
demonstrable conductive component to their hearing loss. A
small group of patients get little benefit from surgery and

110
Summary 

subsequently a cochlear implant should be considered. Heining


et al. (2017) however, the results after stapedotomy in severe
mixed hearing loss are unpredictable and variable because
stapedotomy is not applicable for the treatment of sensorineural
hearing loss. (Calmels et al., 2007)

However, CI is an expensive procedure and requires


experienced surgeons because otosclerotic foci can cause
certain surgical problems during implantation. Ossification of
the round window or the basal turn requires extra drilling to
identify the scala tympani. Some patients with severe
osteoneogenesis require a scala vestibuli approach to achieve a
full insertion. Otosclerosis can also lead to obliteration at the
apical regions of the cochlea, which may result in an
incomplete electrode insertion (Rotteveel et al., 2004).

It is also possible that an electrode is inserted in the basal


turn, that it penetrates the cochlear endosteum, and eventually
enters the osteolytic cavity or even the internal auditory canal.
(Ramsden et al., 1997)

An electrical shunt between the implant and the facial


nerve can cause Facial nerve stimulation (FNS). FNS is an
apprehensive complication of CI occurring on average in 20%
of the patients with otosclerosis. This high occurrence of FNS
can be explained by an increased conductivity of the
otospongiotic bone, making it easier to stimulate the facial
nerve. Management of facial nerve stimulation consists of a

111
Summary 

reduction in stimulus levels of the cranially located electrodes,


totally deactivating the causative electrodes or reimplantation
(Rotteveel et al., 2004; Sainz et al., 2009).

Patients who are not fit for surgery or don’t want to


undergo surgery, will use a hearing aid. Certainly, the option of
amplification as an alternative to surgery should be discussed
with all patients. The benefit over surgery is the avoidance of
the risk of significant SNHL (House, 2001).

A new implantable hearing system, the direct acoustic


cochlear stimulator (DACS) is presented. This system is based
on the principle of a power-driven stapes prosthesis and
intended for the treatment of severe mixed hearing loss due to
advanced otosclerosis. It consists of an implantable
electromagnetic transducer, which transfers acoustic energy
directly to the inner ear, and an audio processor worn externally
behind the implanted ear. The device is implanted using a
specially developed retromeatal microsurgical approach. After
removal of the stapes, a conventional stapes prosthesis is
attached to the transducer and placed in the oval window to
allow direct acoustical coupling to the perilymph of the inner
ear. In order to restore the natural sound transmission of the
ossicular chain, a second stapes prosthesis is placed in parallel
to the first one into the oval window and attached to the
patient's own incus, as in a conventional stapedectomy
(Häusler et al., 2008).

112
Summary 

Electronic search using the search terms: (Otosclerosis


OR Stapedectomy OR (Stapes surgery) OR Stapedotomy OR
(Cochlear implantation) OR (cochlear implant)) AND (far
advanced otosclerosis) in different combinations, yielded
hundreds of articles from which 51 articles met our criteria, from
seven databases. After duplicates removal, 35 articles were
screened in title/abstract screening, while 30 articles were
screened in full text screening for inclusion. Finally, 28 articles
were included finally in qualitative analysis and 24 in
quantitative meta-analysis. The manual search resulted in no
additional studies. online databases included; PubMed,
SCOPUS, Web of science, Cochrane Central Register of
Controlled Trials, (CENTRAL), Science Direct, and Google
Scholar.

The results of this meta-analysis show that comparing the


outcomes and complications of cochlear implantation and
stapes surgery in far advanced otosclerosis patients have
different results, in some of them there was a highly favorable
and recommended procedure than the other, other results
declared no significant difference in postoperative outcomes.

This meta-analysis showed that CI had a significantly


higher satisfaction ratio (p-value= 0.013) & rate (P-value=
0.009) than stapedectomy in patients with far advanced
otosclerosis.

113
Summary 

Postoperative ability to use hearing aid was very high in


relevant studies (event rate= 71.3%) and this confirms that
stapes surgery can give very good results in patients with FAO.
This result is consistent with the literature.

According to our meta-analysis CI has lower rate of any


postoperative complications than stapedectomy in FAO
patients. Access to area of cochleostomy was less difficult in CI
(even rate= 24.9%), CI had also a significant low rate of
difficult insertion of electrode bundle (even rate= 14.8%).

Dysgeusia was of a lower rate in patients underwent CI


(even rate= 1.4%) than those who had stapes surgery (even
rate= 3.6). Tinnitus also occurred at a lower rate in CI (even
rate= 32.7) than in stapes surgery (even rate= 52.5%), regarding
vertigo results were close in both CI and stapes surgery but it
was of a lower rate in stapedectomy (event rate= 8.8%) than CI
(event rate= 12.8%) this difference in event rate was due to
varying number of patients in each study.

Our meta-analysis revealed that Monosyllables and


disyllables recognition was surprisingly of a higher mean in
stapedectomy (mean= 34%, 56.6%) than in CI (mean= 28.1%,
55.2%), statistically monosyllables and disyllables recognition
is better in stapedectomy but in fact there is nearly no
significant difference but maybe they are better in CI in some
studies, this results were due to the varying number of studies
included in each procedure and the varying number of patients

114
Summary 

in different studies. Regarding recognition of phrases, it was


only mentioned in CI studies and it was of a high significant
mean (65.7%).

One of the most important complications of CI is Facial


electrical stimulation, in our meta-analysis it was of a low rate
in FAO patients who underwent CI with even rate (12.4%).

Regarding postoperative hearing loss, CI had a lower rate


of hearing loss than patients of FAO who had a stapes surgery
with even rates (16.4%, 21.2%) respectively.

Regarding postoperative pure tone average, CI had a


better mean for PTA in patients with FAO (mean= 29.1 dB),
while the mean of PTA in unaided stapes surgery was (52.3
dB). This is a very important item in comparison between CI
and stapedectomy. Speech recognition threshold in our meta-
analysis is statistically of a higher mean in FAO patients who
had stapes surgery (62.6%), and of a lower mean in CI (43.7%).
Relevant studies had a different number of patients, so although
this rate seemed more often associated with CI failures, the
difference between the two subgroups was not found to be
significant.

Both stapedotomy and CI have different advantages and


disadvantages, and thus the treatment modality should be
tailored according to the patient’s history, clinical signs and
symptoms, audiological results (tuning fork tests, pure tone

115
Summary 

audiogram, speech threshold testing, SR), HRCT findings,


ECoG/promontory stimulation and individual expectations.

Both Stapes surgery and CI are reliable treatment options


for FAO with very close success rates. Statistics of CI are
greater than Stapes surgery and CI has a consistent
improvement in speech discrimination scores in comparison to
Stapes surgery. Patients must receive adequate counseling
regarding all the factors mentioned above and the decision must
be made by surgeons and the informed patients.

116
Conclusion 

CONCLUSION
Both Stapes surgery and CI are reliable treatment options
for FAO with very close success rates. Statistics of CI are
greater than Stapes surgery and CI has a consistent
improvement in speech discrimination scores in comparison to
Stapes surgery. Patients must receive adequate counseling
regarding all the factors mentioned above and the decision must
be made by surgeons and the informed patients.

117
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136
‫الملخص العربى ‪‬‬

‫الملخص العربي‬
‫م‪££‬رض تص‪££‬لب األذن ه‪££‬و عملي‪££‬ة ارتش‪££‬اف عظمي لغش‪££‬اء الِّتي‪ُ££‬ه يليه‪££‬ا ترس‪££‬ب‬
‫تعويضي لتصلب عظمي جدي‪£‬د غ‪£‬ير ناض‪£‬ج وه‪£‬و يح‪£‬دث ع‪£‬ادًة خالل الف‪£‬ترة بين الِع ق‪£‬د‬
‫الثاني والخامس من العمر‪ .‬الموق‪£‬ع األك‪£‬ثر ت‪£‬أثرا ه‪£‬و ح‪£‬ول الناف‪£‬ذة الَبيَض ِوَّية ( تص‪£‬لب‬
‫األذن النافذي) مما ي‪£‬ؤدي إلى فق‪£‬دان الس‪£‬مع التوص‪£‬يلي بس‪£‬بب تث‪£‬بيت الص‪£‬فيحة القدمي‪£‬ة‬
‫للركابي‪ .‬في حوالي ‪ ٪10‬من المرضى ‪ ،‬تغزو بؤر تصلب األذن أعم‪£‬ق في الِّتي‪ُ£‬ه‪ ،‬مم‪£‬ا‬
‫يؤدي إلى تصلب األذن العظمي الخلف ناف‪££‬ذي‪ ,‬ه‪££‬ذه العملي‪££‬ة ت‪££‬ؤدي ت‪££‬دريجيا إلى فق‪££‬دان‬
‫السمع الشديد المختلط ثم إلى فقدان السمع الحسي العص‪££‬بي العمي‪££‬ق‪ .‬أش‪££‬ارت العدي‪££‬د من‬
‫الدراسات إلى أن ب‪£‬ؤر التص‪££‬لب ال‪£‬رجفي الخلفي ق‪££‬د ت‪£‬ؤدي إلى فق‪£‬دان الس‪£‬مع من خالل‬
‫اضطراب التوازن األيوني للقوقعة عن طريق إعاقة إعادة التدوير األيوني وتقليل الجهد‬
‫الداخلي للقوقعة وهذا يؤدي إلى ضعف أو فقدان خاليا الشعر السمعية للقوقعة‪.‬‬
‫ال يوج‪££‬د تعري‪££‬ف مقب‪££‬ول عالمي‪ً£‬ا لم‪££‬رض تص‪££‬لب األذن المتق‪££‬دم‪ ,‬في اآلون‪££‬ة‬
‫األخيرة يتم استخدام مصطلح تصلب األذن المتقدم عن‪££‬دما يك‪££‬ون الم‪££‬ريض المص‪££‬اب‬
‫بتصلب األذن يعاني من انخفاض شديد في التع‪££‬رف على الكالم‪ .‬وق‪££‬د وص‪££‬ف الع‪££‬الم‬
‫كالمل وزمالئه تصلب األذن المتقدم عن طريق خصائصه السمعية واإلشعاعية‪ .‬وقد‬
‫كانت الخصائص السمعية الالزمة للتشخيص هي اكتشاف كلم‪££‬ات غ‪££‬ير مألوف‪££‬ة أق‪££‬ل‬
‫من ‪ %30‬من درجة تمييز الكالم عند ‪ 70‬ديسيبل‪ ,‬مع استخدام ُم عينة سمعية مجه‪££‬زة‬
‫جيدًا ومخطط سمعي فارغ‪.‬‬
‫وق‪££‬د اس‪££‬تخدم م‪££‬ألفون مختلف‪££‬ون أنظم‪££‬ة تص‪££‬نيف مقطعي‪££‬ة مختلف‪££‬ة لتص‪££‬نيف‬
‫مرض تصلب األذن المتقدم‪ .‬ويعتمد نظ‪££‬ام التص‪££‬نيف المقطعي لروتفي‪££‬ل جزئي ‪ً£‬ا على‬
‫موق‪££‬ع ون‪££‬وع الض‪££‬رر‪( :‬الدرج‪££‬ة األولى)‪ :‬ناف‪££‬ذ فق‪££‬ط‪( ,‬الدرج‪££‬ة الثاني‪££‬ة)‪ :‬وتنقس‪££‬م إلى‬
‫قسمين القسم األول‪ :‬خلف نافذي َح َلقي مزدوج والقسم الثاني‪ :‬ضيق في اللفة الس‪££‬فلية‬
‫أو كالهما الدرجة الثانية القسم الثالث‪( ,‬الدرجة الثالثة)‪ :‬اكتناف خلف نافذي منتش‪££‬ر‪.‬‬
‫وقد تقدم العلماء سايمونز وفاننج بتصنيف مماثل لتصنيف روتفيل فيم‪£‬ا ع‪£‬دا الدرج‪£‬ة‬
‫الثانية والذي يعتمد على الموقع التشريحي بدًال من ن‪£‬وع الض‪££‬رر‪ ,‬وتنقس‪£‬م إلى ثالث‪£‬ة‬

‫‪1‬‬
‫الملخص العربى ‪‬‬

‫أقسام‪ ,‬القسم األول إذا شمل الدوران السفلي‪ ,‬والقسم الثاني إذا شمل الدوران األوسط‬
‫أو األعلى‪ ,‬والقسم الثالث إذا شمل كليهما‪.‬‬
‫لسوء الحظ ال توجد مبادئ توجيهية قياسية فيما يتعلق بإعادة تأهيل مرض‪££‬ى‬
‫تصلب األذن المتق‪££‬دم‪ ,‬يوج‪££‬د ثالث خي‪££‬ارات عالجي‪££‬ة بالنس‪££‬بة لم‪££‬رض تص‪££‬لب األذن‬
‫المتقدم يتم اقتراحها على الم‪££‬ريض وهي‪ ,‬أواًل ‪ :‬ع‪££‬دم الت‪££‬دخل واالس‪££‬تمرار باس‪££‬تخدام‬
‫سماعات األذن‪ ,‬ثانًيا‪ :‬جراحة الرك‪££‬ابي م‪££‬ع اس‪££‬تخدام س‪££‬ماعات األذن‪ ,‬ثالًث ا‪ :‬زراع‪££‬ة‬
‫القوقعة‪ .‬في بعض مرضى تصلب األذن المتقدم يكون اتخ‪££‬اد الق‪££‬رار ص‪££‬عًبا لس‪££‬ببين‪,‬‬
‫أواًل ‪ :‬مع فق‪££‬دان الس‪££‬مع المختل‪££‬ط من الص‪££‬عب التنب‪££‬ؤ بمع‪££‬دل نج‪££‬اح جراح‪££‬ة الرك‪££‬ابي‬
‫خاصًة مقارنًة بزراعة القوقعة كتدخل ب‪£‬ديل‪ ,‬ثانًي ا‪ :‬ب‪£‬ؤر تص‪£‬لب األذن البالغ‪£‬ة ح‪£‬ول‬
‫محفظة األذن الداخلية يمكن أن تؤدي لمضاعفات جراحية خالل زراعة القوقعة‪.‬‬
‫ال يعتمد القرار فق‪££‬ط على مع‪££‬دل النج‪££‬اح‪ ,‬فك‪££‬ل ت‪££‬دخل ل‪££‬ه مم‪££‬يزات وعي‪££‬وب‬
‫محددة‪ ,‬فجراحة الركابي نس‪££‬بًيا بس‪££‬يطة وآمن‪££‬ة وذات تكلف‪££‬ة بس‪££‬يطة ويمكن أن تحق‪££‬ق‬
‫نتائج جيدة للغاية‪ ,‬فُتعتَب ر جراح‪££‬ة الرك‪££‬ابي خي‪££‬ار عالجي مناس‪££‬ب لمرض‪££‬ى تص‪££‬لب‬
‫األذن المتقدم ويجب إعتبارها إلزامية قبل عرض خيار زراعة القوقعة ألولئك ال‪£‬ذين‬
‫لديهم عنصر موصل يمكن إثباته لفقدان السمع لديهم‪ .‬مجموعة صغيرة من المرضى‬
‫تحص‪£££‬ل على اس‪£££‬تفادة قليل‪£££‬ة من الجراح‪£££‬ة ل‪£££‬ذا يجب أخ‪£££‬ذ زراع‪£££‬ة القوقع‪£££‬ة في‬
‫اإلعتبار‪.‬ومع ذلك فإن النتائج بعد جراحة الركابي في فقدان السمع الح‪£‬اد المختل‪£‬ط ال‬
‫يمكن التنبؤ بها ومتغيرة ألن جراحة الركابي ال يمكن تطبيقه‪££‬ا لعالج ض‪££‬عف الس‪££‬مع‬
‫الحسي العصبي‪.‬‬
‫عالوًة على ذل‪£‬ك‪ ,‬ف‪£‬إن المض‪£‬اعفات ال‪£‬تي نخش‪£‬اها من جراح‪£‬ة الرك‪£‬ابي هي‬
‫زيادة في فقدان السمع الحسي العصبي والذي في حالة تصلب األذن المتقدم قد يؤدي‬
‫إلى أذن صماء وظيفية‪ .‬وق‪££‬د حققت زراع‪£‬ة القوقع‪££‬ة نت‪££‬ائج ممت‪££‬ازة ويب‪££‬دو أن‪££‬ه خي‪££‬ار‬
‫عالجي جيد لمرضى تصلب األذن المتق‪££‬دم‪ .‬وم‪££‬ع ذل‪££‬ك ف‪££‬إن زراع‪££‬ة القوقع‪££‬ة إج‪££‬راء‬
‫مكل‪££‬ف ويتطلب ج‪££‬راحين ذوي خ‪££‬برة ألن ب‪££‬ؤر تص‪££‬لب األذن يمكن أن تس‪££‬بب بعض‬
‫المشكالت الجراحية أثناء الزرع‪ .‬يتطلب تعظم النافذة المستديرة أو الدوران القاعدي‬
‫إجراء حفر إضافي للتعرف على الِس قاَلة الطبلية‪ .‬بعض المرض‪££‬ى ال‪££‬ذين يع‪££‬انون من‬

‫‪2‬‬
‫الملخص العربى ‪‬‬

‫هشاشة العظام الح‪££‬ادة يحت‪££‬اجون إلى ال‪££‬دخول ع‪££‬بر الِس قاَلة الدهليزي‪££‬ة للوص‪££‬ول إلى‬
‫إدخال كامل‪.‬‬
‫يمكن أن يؤدي تصلب األذن إلى إنسداد في المناطق القممية من القوقعة مما‬
‫قد يؤدي إلى إدخال غير مكتمل للقطب الكهرب‪££‬ائي‪ .‬يمكن أن تحي‪££‬ط اآلف‪££‬ات العظمي‪££‬ة‬
‫السفلية المتموج‪££‬ة بالقوقع‪££‬ة‪ ,‬مم‪££‬ا ق‪££‬د ي‪££‬ؤدي إلى انخف‪£‬اض الكثاف‪££‬ة المحيط‪££‬ة بالقوقع‪££‬ة‬
‫وتجويف العظام (الحلق‪££‬ة المزدوج‪££‬ة)‪ .‬ألن ه‪££‬ذه الحلق‪££‬ة المزدوج‪££‬ة موازي‪££‬ة لل‪££‬دوران‬
‫القاعدي للقوقعة وغالًبا ما تختفي النافذة المستديرة في لويحة ص‪££‬لبة‪ .‬يمكن للهال‪££‬ة أن‬
‫ُتشِبه فتح‪££‬ة في ال‪££‬دوران القاع‪££‬دي مم‪££‬ا ق‪££‬د ي‪££‬ؤدي إلى إدخ‪££‬ال خ‪££‬اطئ للقطب في ه‪££‬ذا‬
‫التجوي‪££‬ف الخ‪££‬اطئ‪ .‬من الممكن أيًض ا أن يتم إدخ‪££‬ال قطب كهرب‪££‬ائي في ال‪££‬دوران‬
‫القاعدي وأن يخترق الطبقة العظمية للقوقعة وي‪££‬دخل في نهاي‪££‬ة المط‪££‬اف في تجوي‪££‬ف‬
‫العظام أوحتى القناة السمعية الداخلية‪.‬‬
‫حتى بعد نجاح عملية الزرع فإن إع‪££‬ادة تأهي‪££‬ل المرض‪££‬ى ال‪££‬ذين يع‪££‬انون من‬
‫تص‪££‬لب األذن ص‪££‬عبة للغاي‪££‬ة ألن التغ‪££‬يرات التص‪££‬لبية العص‪££‬بية في القوقع‪££‬ة يمكن أن‬
‫تؤثر على أداء الزرع‪ .‬قد تكون هناك حاجة إلعادة البرمجة ذات مس‪££‬تويات التحف‪££‬يز‬
‫العالية للحصول على استجابات سمعية‪ ,‬على الرغم من أن مستويات التحفيز العالي‪££‬ة‬
‫هذه تزيد من خطر تحفيز العص‪£‬ب ال‪£‬وجهي‪.‬بش‪£‬كل ع‪£‬ام ف‪£‬إن مع‪£‬دل تحف‪£‬يز العص‪£‬ب‬
‫الوجهي لدى مرضى تص‪££‬لب األذن مرتف‪££‬ع‪ .‬تحويل‪££‬ة كهربائي‪££‬ة بين ال‪££‬زرع وعص‪££‬ب‬
‫الوجه يمكن أن تس‪£‬بب تحف‪£‬يز العص‪££‬ب ال‪£‬وجهي‪ .‬تحف‪£‬يز العص‪££‬ب ال‪£‬وجهي ه‪£‬و أح‪£‬د‬
‫المض‪££‬اعفات الخاطئ‪££‬ة لزراع‪££‬ة القوقع‪££‬ة ال‪££‬تي تح‪££‬دث في المتوس‪££‬ط في ‪ %20‬من‬
‫المرضى الذين يع‪£‬انون من م‪£‬رض تص‪£‬لب األذن‪ .‬يمكن تفس‪£‬ير ه‪£‬ذا التواج‪£‬د الع‪£‬الي‬
‫لتحفيز العص‪££‬ب ال‪££‬وجهي بزي‪££‬ادة التوص‪££‬يل للعظ‪££‬ام اإلس‪££‬فنجية لألذن مم‪££‬ا يس‪££‬هل من‬
‫تحفيز العصب الوجهي‪ .‬تت‪£‬ألف إدارة تحف‪£‬يز العص‪££‬ب ال‪£‬وجهي من خفض مس‪£‬تويات‬
‫التحف‪£‬يز لألقط‪££‬اب الموج‪££‬ودة في الجمجم‪££‬ة ‪ ،‬مم‪££‬ا ي‪££‬ؤدي إلى إلغ‪££‬اء تنش‪££‬يط األقط‪££‬اب‬
‫الكهربائية المسببة أو إعادة زرعها‪.‬‬
‫الهدف من الدراسة‬

‫‪3‬‬
‫الملخص العربى ‪‬‬

‫االس‪£££‬تعراض المنهجي للمقارن‪£££‬ة بين نت‪£££‬ائج الس‪£££‬مع ومض‪£££‬اعفات جراح‪£££‬ة‬


‫الركابي مقابل زراعة القوقعة في المرضى الذين يعانون من تصلب األذن المتقدم‪.‬‬
‫النتائج والمناقشة‬
‫تظهر نتائج هذا التحليل التلوي أن مقارنة نتائج ومضاعفات زراع‪££‬ة قوقع‪££‬ة‬
‫األذن وجراحة الركاب في مرضى تص‪££‬لب األذن المتق‪££‬دمين له‪££‬ا نت‪££‬ائج مختلف‪££‬ة ‪ ،‬في‬
‫بعضهم كان هن‪££‬اك إج‪££‬راء مفض‪££‬ل للغاي‪££‬ة وموص‪££‬ى ب‪££‬ه عن اآلخ‪££‬ر ‪ ،‬بينم‪££‬ا أظه‪££‬رت‬
‫النتائج األخرى عدم وجود اختالف كبير في نتائج ما بعد الجراحة‪.‬‬
‫أظهر هذا التحليل التلوي أن زراعة قوقعة األذن كان لديها نسبة رضا أعلى‬
‫بشكل ملحوظ (قيمة ‪ )p = 0.013‬ومعدل (قيمة ‪ )P = 0.009‬من استئصال عظمة‬
‫الركاب في المرضى الذين يعانون من تصلب األذن المتقدم‪.‬‬
‫كانت القدرة بع‪£‬د الجراح‪£‬ة على اس‪£‬تخدام المعين‪£‬ات الس‪£‬معية عالي‪£‬ة ج‪ً£‬دا في‬
‫الدراسات ذات الصلة (معدل الحدث = ‪ )٪71.3‬وهذا يؤكد أن جراحة عظم الركاب‬
‫يمكن أن تعطي نتائج جيدة جًدا لمرضى تصلب األذن المتقدم وهذه النتيجة تتف‪££‬ق م‪££‬ع‬
‫الدراسات المنشورة سابقا‪.‬‬
‫وفًقا للتحليل التلوي الذي أجريناه ‪ ،‬فإن زراعة قوقعة األذن لديها معدل أق‪££‬ل‬
‫من أي من مضاعفات ما بعد الجراحة مقارنة باستئصال الركاب في مرضى تصلب‬
‫األذن المتقدم‪ .‬كان الوصول إلى منطقة فغر القوقعة أقل صعوبة في زراع‪££‬ة القوقع‪££‬ة‬
‫(معدل متساٍو = ‪ ، )٪24.9‬كان لدى زراعة القوقع‪£‬ة أيًض ا مع‪£‬دل منخفض ج‪ً£‬دا من‬
‫صعوبة إدخال حزمة اإللكترود (المعدل الزوجي = ‪.)٪14.8‬‬
‫كان عسر التذوق منخفًضا في المرض‪££‬ى ال‪££‬ذين خض‪££‬عوا لـ زراع‪££‬ة القوقع‪££‬ة‬
‫(معدل متساٍو = ‪ )٪1.4‬من أولئك الذين خض‪££‬عوا لجراح‪££‬ة رك‪££‬اب (مع‪££‬دل متس‪££‬اٍو =‬
‫‪ .)3.6‬ح‪££‬دث ط‪££‬نين األذن أيًض ا بمع‪££‬دل أق‪££‬ل في زراع‪££‬ة القوقع‪££‬ة (مع‪££‬دل متس‪££‬اٍو =‬
‫‪ )32.7‬مقارنة بجراحة الركاب (معدل متساٍو = ‪ ، )٪52.5‬فيما يتعلق بنتائج ال‪££‬دوار‬
‫كانت قريبة في كل من جراحة زراعة القوقعة وجراحة الرك‪££‬اب ‪ ،‬لكنه‪££‬ا ك‪££‬انت أق‪££‬ل‬
‫في عملية استئصال الركاب (مع‪££‬دل الح‪££‬دث) = ‪ )٪8.8‬من زراع‪££‬ة القوقع‪££‬ة (مع‪££‬دل‬

‫‪4‬‬
‫الملخص العربى ‪‬‬

‫الح‪££‬دث = ‪ )٪12.8‬ك‪££‬ان ه‪££‬ذا االختالف في مع‪££‬دل الح‪££‬دث بس‪££‬بب اختالف ع‪££‬دد‬


‫المرضى في كل دراسة‪.‬‬
‫كش‪££££‬ف التحلي‪££££‬ل التل‪££££‬وي ال‪££££‬ذي أجرين‪££££‬اه أن التع‪££££‬رف على المق‪££££‬اطع‬
‫الصوتيةاألحادية والمق‪£‬اطع الص‪££‬وتيةالثنائية ك‪£‬ان بش‪£‬كل م‪£‬دهش أعلى في استئص‪££‬ال‬
‫الركاب (المتوسط = ‪ )٪56.6 ، ٪34‬منه في زرعةالقوقعة (المتوسط = ‪، ٪28.1‬‬
‫‪ ، )٪55.2‬التعرف اإلحص‪££‬ائي على المق‪££‬اطع الص‪££‬وتيةاألحادية والمق‪££‬اطع الص‪££‬وتية‬
‫الثنائية يكون أفضل في استئصال الرك‪££‬اب‪ .‬في الواق‪££‬ع ‪ ،‬ال يوج‪££‬د ف‪££‬رق كب‪££‬ير تقريًب ا‬
‫ولكن ربما يكونون أفضل في زراعةالقوقعة في بعض الدراسات ‪ ،‬كانت هذه النتائج‬
‫بسبب العدد المتف‪£‬اوت من الدراس‪£‬ات المدرج‪£‬ة في ك‪£‬ل إج‪£‬راء والع‪£‬دد المتف‪£‬اوت من‬
‫المرضى في الدراسات المختلفة‪ .‬فيما يتعلق بالتعرف على العبارات ‪ ،‬فقد تم ذكره‪££‬ا‬
‫فقط في دراسات زراعة القوقعة وكان لها متوسط مرتفع (‪.)٪65.7‬‬
‫يعد التحفيز الكهربائي للوجه من أهم مضاعفات زراع‪£‬ة قوقع‪£‬ة األذن ‪ ،‬ففي‬
‫تحليلنا التلوي كان معدل منخفًض ا لدى مرضى تصلب األذن المتق‪££‬دم ال‪££‬ذين خض‪££‬عوا‬
‫لزراعة القوقعة بمعدل (‪.)٪12.4‬‬
‫فيما يتعلق بفقدان السمع بعد الجراح‪££‬ة ‪ ،‬ك‪££‬ان ل‪££‬دى جراح‪££‬ة زراع‪££‬ة القوقع‪££‬ة‬
‫معدل فقدان سمع أقل من مرضى تصلب األذن المتقدم الذين خضعوا لجراحة ركاب‬
‫بمعدالت (‪ )٪21.2 ، ٪16.4‬على التوالي‪.‬‬
‫فيما يتعلق بمتوسط النغمة النقية بعد العملي‪££‬ة الجراحي‪££‬ة ‪ ،‬ك‪££‬ان ل‪££‬دى زراع‪££‬ة‬
‫قوقعة األذن متوسط أفض‪££‬ل لـ ‪ PTA‬في المرض‪££‬ى ال‪££‬ذين يع‪££‬انون من تص‪££‬لب األذن‬
‫المتقدم (المتوسط = ‪ 29.1‬ديسيبل) ‪ ،‬بينما ك‪£‬ان متوس‪£‬ط ‪ PTA‬في جراح‪£‬ة الرك‪£‬اب‬
‫بدون مساعدة (‪ 52.3‬ديسيبل)‪ .‬هذا عنصر مهم للغاية بالمقارنة بين جراح‪££‬ة زراع‪££‬ة‬
‫القوقعة واستئصال عظمة الركاب‪ .‬التعرف على الكالم في تحليلن‪££‬ا التل‪££‬وي إحص‪££‬ائًيا‬
‫لديه متوسط أعلى لدى مرضى تصلب األذن المتقدم الذين خضعوا لجراحة رك‪££‬اب (‬
‫‪ ، )٪62.6‬ومتوسط أقل في جراحة زراعة القوقع‪£‬ة (‪ .)٪43.7‬ك‪£‬ان للدراس‪£‬ات ذات‬
‫الصلة عدد مختلف من المرضى ‪ ،‬لذلك على الرغم من أن هذا المعدل يبدو مرتبًط ا‬

‫‪5‬‬
‫الملخص العربى ‪‬‬

‫في كث‪£££‬ير من األحي‪£££‬ان بفش‪£££‬ل زراع‪£££‬ة القوقع‪£££‬ة ‪ ،‬إال أن الف‪£££‬رق بين المجموع‪£££‬تين‬
‫الفرعيتين لم يكن مهًم ا‪.‬‬
‫لكل من شق عظمة الركابي و زراعة قوقعة األذن مزايا وعي‪££‬وب مختلف‪££‬ة ‪،‬‬
‫وبالتالي يجب تصميم طريقة العالج وفًق ا لت‪££‬اريخ الم‪££‬ريض والعالم‪££‬ات واألع‪£‬راض‬
‫السريرية والنتائج السمعية (اختبارات الشوكة الرنانة ‪ ،‬مخطط السمع النقي ‪ ،‬اختبار‬
‫عتبة الكالم ‪ ، )SR ،‬نتائج ‪ / HRCT ، ECoG‬التحفيز الرعن والتوقعات الفردية‪.‬‬
‫تع‪££‬د ك‪££‬ل من جراح‪££‬ة عظم الرك‪££‬ابي و زراع‪££‬ة قوقع‪££‬ة األذن من الخي‪££‬ارات‬
‫العالجية الموثوقة لمرض تصلب األذن المتقدم م‪£‬ع مع‪£‬دالت نج‪£‬اح قريب‪£‬ة ج‪ً£‬دا‪ .‬تع‪£‬د‬
‫إحص‪£‬ائيات جراح‪£‬ة زراع‪£‬ة قوقع‪£‬ة األذن أك‪£‬بر من جراح‪£‬ة عظم الرك‪£‬اب ‪ ،‬كم‪£‬ا أن‬
‫زراعة القوقعة لديها تحسن ثابت في درجات تمييز الكالم مقارنة بجراح‪££‬ة الرك‪££‬اب‪.‬‬
‫يجب أن يتلقى المرضى المشورة الكافية فيما يتعلق بجميع العوام‪££‬ل الم‪££‬ذكورة أعاله‬
‫ويجب أن يتم اتخاذ القرار من قبل الجراحين والمرضى المطلعين‪.‬‬

‫‪6‬‬

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