Download otosclerosis

OTOSCLEROSIS
BY
PORNTHAPE KASEMSIRI
Aj. SUTHEE (Advisor)
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Introduction
• Otosclerosis : fibrous osteodystrophy of the
human otic capsule
Cause ( suggest ) :
• Hereditary , endocrine , biochemical , metabolic ,
infectious ( eg. Measles ) , traumatic , vascular &
autoimmune
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Embryology
• At 16 th wk : endochondral bone replace
cartilagenous BUT in some people ; incomplete
bone replace
• Fissula ante fenestram (ant to OW ):this region
is affected in 80-90% of pt
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Histology 3 form:
1 otospongiosis ( early phase ) : otoscopic exam
 Schwartz sign
2 transitional phase
3 otosclerosis ( late phase )
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Pathophysiology
• CHL : due to fixation of stapes footplate
• SNHL มี 3 mech :
1 toxic metabolite injury to neuroepithelium
2 vascular compromise
3 direct extension to cochlea
• Dizziness :OS inner ear syn; DDX : Meniere dz
, superior semicircular canal dehiscence (SSCD)
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Epidemiology
• OS  autosomal-dominant hereditary
• Most in Caucasians pt
• Associate FH
( Clayton et al. : OS ass. COL1a1 gene )
• OS advance more progress in female > male ,
female : male 2:1
• Age variable , HL between 15-45 yr ( ~ 33 yr )
• Hormonal factor
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History & Physical examination
• slowly HL over progressive yrs
• Good hearing in noisy situation ( paracusis of
Willis )
• Present tinnitus ~ 75 %
• Otoscopy : TM is normal in most pt , Schwartze
sign may be present
• Weber test : lateralize to greater degree of CHL
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History & Physical examination
• Initial phase  Rinne - ve may be limited to
256 Hz
• Footplate fixation  Rinne - ve at 512 Hz &
1024 Hz
• Rinne – ve :
air-bone gap ~ 10-15 dB at 256 Hz
~ 20-25 dB at 512 Hz
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Audiologic testing
• Audiogram :Cahart notch
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Audiologic testing
• Tympanogram : type A ,As
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Audiologic testing
• Stepedial reflexes : abn or norm depending
degree of fixation
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• Vestibular test should be in pt with
dizziness
• HRCT :
Early Dz : radiolucent areas around Cochlea
 Halo sign
Mature case : diffuse sclerosis
• CT scan helpful R/O middle ear Dz : mass
,vascular anomalies
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Differential diagnosis
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Ossicular chain discontinuity
Mass effect on TM or ossicular chain
Conginital stapedial footplate fixation
Malleus head fixtion : cause infection ,
tympanosclerosis
Paget Dz (osteitis deformans ) :begin involve
periosteal layer & involve endochondral bone last
Osteogenesis imperfecta: autosomal dominant
defect of osteoblast activity ซึ่ งพบว่ามี stapes fixation
& unique blue sclera ~ 40-60%
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Management
Amplification
pt with HL & not be suitable candidate for Sx
• ข้อดี
– Avoid potential risk of profound HL from Sx
• ข้อเสี ย
– Usually not used at night
– Physical sensation of device in EAC  negative
sensation
In pt with severe to profound SNHLcochlear impant
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Medical management
• use sodium fluoride fluoride ion replace hydroxyl
radical form  stable fluorapatite complex resist
osteoclast activites
• Recommended Dose 20-120 mg per day
• Side effect :
– usually minor  GI irritation ( prevent : low dose ,
enteric coat )
– occasional complaint of jt , bone , muscle pain
resolves with temporary discontinuation of therapy
– Rare : fluid retention , cutaneous eruption & eye
problem
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Florical
• Fluoride: 3.75 mg Calcium :145 mg
• Florical can be used three times a day for
otosclerosis. After two years, the dose may be
reduced to one capsule per day
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Surgical management
•
Stapedectomy :
indicated the stapes fix (A-B gap at least 30 dB)
negative Rinne test at 256 , 512 Hz ( Shambaugh)
• Successful stapedectomy :
1. correct CHL
2. remove Carhart’s notch
3. closure pre-op A-B gap
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Factors
• Age :
– In the young pt anomalies of malleus or
incus (congenital OS (25%)> juvenile OS
(3%))
– In older pt post op poorer result in High
frequency range
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• Lifestyle & occupation :
– Repeat exposure barometric pressure change
(scuba diver)  greater risk fistulae , prosthesis
dislocate
– work or hobbies about excellent balance 
questionable candidate for Sx
– work about taste (chef )  recommend to
amplification because risk to stretching or cutting
chorda tympani n.
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• Otologic problem
– Meniere Dz & OS : greater risk of cochlear HL
after stapedectomy
– TM perforate & OS : should be closure TM
before stapedectomy and high incidence
SNHL after stapedectomy
– Severe Eustachian tube dysfunction &
cholesteatoma : not good candidate for
stapedectomy
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Contraindication( Shambaugh)
1. Poor speech discrimination & Hx of vertigo in
recent month (because possibility of
endrolymohatic hydrop labyrinth open)
2. Pt with only hearing ear should be avoid
(relative)
3. Pt with ME infection or effusion (absolute)
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Technique of stapedectomy
Preoperative preparation
 Patient counseling
option :
1. observation
2. fluoride use
3. trial hearing aid
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Informed of risk
– Post op TM perforate ~ 2%
– Dehiscence of fallopian canal over OW จะทาให้มี
expose or prolapsed Facial nerve risk to injury
– Stretching or contusion chorda tympani 
alteration test ( symptom can self limit &
disappear in a few wk or month )
– Post op deafness of less than 2%
– Acute balance disturbance is common after
stapedectomy , can resolve in 3-7 days , long
term is rare
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Preoperative preparation
Anesthesia
– LA : slightly less bleeding & can assess
intraoperative hearing
– GA : pt prefer
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Operative
 obtaining the tissue graft
– vein ; harvested from back of hand
– fat ; harvested from ear lobule
– Temporalis fascia ; harvested through a
small incision above & behind ear
– Perichondrium ; harvested from tragus
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Exposure of the OW
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Stapedectomy
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Stapedotomy
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Tissue Seal of the Oval Window
• Tissue seal : vein , perichondrium , fascia
• No living tissue : Gelfoam
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Prosthesis Placement
Cup piston prosthesis
Original Shea Teflon piston
prosthesis
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McGee/Fisch-type piston prosthesis
House wire prosthesis
Postoperative care
1.
2.
3.
4.
Given adequate analgesic
Avoid straining or blowing nose
Antibiotic are not routine
Keep dry ear until healing TM
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Special Problem During & After Stapedectomy
Floating Footplate Problem
• nonfixed stapedial footplate after the crural arches
have been Fx
• Removing floating footplate : use diamond drill or
laser at promontory edge of OW  Small Rt angle
hook to lift footplate
• If footplate depress into vestibule  graft &
prosthesis place lat to depressed footplate
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Obliterate Otospongiosis
• occur at margins of OW  drill out is required
• approximate locate OW by crura & fallopian
canal
• Use small diamond drill , Argon or CO2 laser is
carefully thinned down until blue area and made
round opening
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Biscuit Footplate
• the thickened footplate with well-defined margins
produced by a primary focus in footplate
• Use small diamond drill , Argon or CO2 laser cut
across center until foot plate  can be extracted
in 2 piece
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Dehiscent , Prolapsed Facial nerve
• facial nerve protruding down over the footplate
• a small fenestra can be made in the footplate
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Postoperative Granuloma
• granuloma develop within the first 2 wk
• postop cause sudden hearing loss &
disturbance of balance
• Hallmark finding: grayish-red mass in the
posterosuperior quadrant of TM
• Should be Early explore & remove and then
cover by living tissue
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Fixed incus and / or Malleus
• If body incus & not
malleus fix
separate incus from head
stapes & remove
• If head malleus & not
incus fix
cut neck malleus &
remove head malleus
• If both body incus&head
malleus fix
remove both and then
insert TORP
remove then insert PORP
• If incus and/or
malleus,not stapes fix
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TORP
PORP
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Round Window Closure
• Partial involvement of margin of round window is
common
• Tiny opening of RW or may be fibrous occlude
RW but not disturb hearing
• Complete bone closure RW with stapes
ankylosis  cause severe MHL ; Stapedectomy
can improve hearing , Attempt open RW should
be avoid
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Fracture of the Long Process of the Incus
• reconstruction by TORP with wire
Acut otitis media
• immediate postoperative period
• culture & appropriate ATB
• post op TM perforate should be treated by ATB &
rare require Sx repair
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Perilymph Gusher ; rare
• profuse flow of perilymph when open vestibule
abn cochlear aqueduct and / or IAC
• elevate headreduce pressure, usually stop flow
of perilymph
• seal OW by tissue graft and then place prosthesis
• bed rest & elevate head 30 degree until dry ear 24hr
• may be lumbar drain is placed
• complicationmay permanent cochlear loss
• further operation on this ear or other ear C/I
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Facial palsy
• facial paralysis of Bell’s palsy can occur 5-7 d
after stapedectomy owing to activation of Herpes
infection
• most case recovery in one to several wk
• Postoperative facial palsy treat with large
coticosteroid as idiopathic Bell’s palsy
• Decompression of nerve might be indicate in
rare case
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Postoperative Perilymh fistula
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rare
symptom: endolymphatic hydrop
should be explore ME , avoid enter to vestibule
found fistulae and then seal by CNT graft
prognosis : vertigo is favorable ; hearing is poor
• Perilymh fistula is more common after wire& Gel
foam or wire fat stapedectomy
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Postoperative Follow-up
• 2-4 wk post op  good fain in hearing &
discrimination
• Best level of hearing ~ 3 mo-1yr (evaluate
by audiogram)
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THANK YOU
Ref
Glasscock-Shambaugh 5 th edition
Bailey 4 th edition
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