A kursus, 2009, indre øre
Sudden Deafness og
Otosklerose
Mads Sølvsten Sørensen
ØNH-Kir F2074, RH
Sudden deafness, definition:
Sudden (sensorineural) hearing loss
SSNHL
develops overnight or within three days
>30dB BC loss
over at least 3 contiguous audiometric
frequencies
Sudden deafness, forekomst:
Incidens: 22-(20)/100.000/år ~
100--(1000)/år i DK
100
Hyppigst ml. 5050-60år
M=F
2% bilaterale
The inner ear
Dual action basic mechanism: Passive and active
Inner hair cells = microphone
Outer hair cells = motor (weak sounds) / brake (loud sounds)
Sudden deafness, årsager:
Infektion: meningitis, herpes, varicel, parotit, CMV, lues mm
Autoimmun: Wegeners g., SLE, PN, Cogan mm
Traumatisk: basisfraktur, PLF, dekompression, stapedotomi
Vaskulær: cerebrovasculær insuff/thrombose, DM, seglcelle
Neurologisk: multipel sclerose, migræne
Neoplasi: vest. Schwannom, metastase, myelomatose mm
Idiopatisk: ISSNHL
Sudden deafness, undersøgelser:
Anamnese + alm klinisk US
ØNH-US inkl kranienerver
Audiometri
Evt vestibulær US
Evt ABR
(Evt Hgb, leuko+diff, glucose, CRP, ANCA, mm, lues-,
HIV-,borrelia-, diverse virus-serologikun hvis anamnese, symptomer eller kliniske fund kan
begrunde det)
MR
Sudden deafness, slutresultat:
(Afhænger at evt. grundlidelse)
ISSNHL: spontan remission 50-65% inden for 4 uger
Bedst prognose ved:
Ingen ledsagende vertigo
Let høretab/ bevaret diskant-mellemtone
Alder ml 15-60år
Kort sygdomshistorie !
Sudden deafness, SSNHL eller PSNHL?
Sudden (sensorineural) hearing loss SSNHL
happens overnight or within three days,
>30dB BC loss over at least 3 contiguous
audiometric frequencies
Progressive sensorineural hearing loss PSNHL
– develops over many days or weeks
Autoimmune sensorineural hearing loss ASNHL
– SSNHL or PSNHL responsive to
corticosteroids
What Brian McCabe described in 1979
18 patients with PSNHL, 5 of which had facial paralysis,
at least 2 had granulomatous lesions in the middle
ear and one of these had vasculitis and granulomas
in the biopsy
All were treated with corticosteroids and
cyclophophamide for a year like you would treat
patients with Wegener’s granulomatosis with good
results
There was no mentioning of pulmonary XX-ray
examinations or renal function tests.
A lymphocyte inhibition assay using human inner ear
tissue extract was positive in 6 of 6 tested. Controls
were negative.
ANCA testing was not available at that time.
What happened since 1979 ?
The message of Brian McCabe was taken out
of its context. Based on theoretical
considerations the presumption evolved that
all cases of SSNHL and PSNHL were
assumed to be due to autoimmunity and
should be treated with corticosteroids.
corticosteroids.
Sudden deafness, behandling:
Afhænger at evt. grundlidelse
ISSNHL:
Steroid, cyklofosfamid mm
Diuretika, dextran, hydroxyethylcellulose mm
Carbogen, papaverin, nikotinsyre mm
Acyklovir
Calcium antagonister
Hyperbar oxygen
Diverse: akupunktur, slangegifte mm
Sudden deafness, behandling:
ISSNHL: sjælden tilstand/ spontan remission 50-65%
inden for 4 uger => vanskeligt at vurdere behandling
Ingen evidens for behandlingseffekt !!!
Conlin AE, Parnes LS. Treatment of sudden
sensorineural hearing loss. 1 A systematic review. Arch
Otolaryngol Head Neck Surg 2007; 133: 573-81.
Conlin AE, Parnes LS. Treatment of sudden
sensorineural hearing loss. 2 A meta-analysis. Arch
Otolaryngol Head Neck Surg 2007; 133: 582-6.
Otosclerosis = pathological bone remodeling in the otic capsule
Otosclerotic focus invading the stapes footplate
Otosclerotic focus
Otosklerose, diagnose:
Anamnese:
høretab
Objektivt:
disposition, debutalder > 20år
progredierende (ensidigt)
normal otoskopi, malleus mobil
Rinne neg. Weber lat.
Carhart´s notch
stapediusrefleks mangler
(evt. on/off)
Kompliance nedsat
evt. high resolution CT:
hypodense områder
Otosklerose, diverse karakteristika:
Klinisk (fenestral/cochleær) otosklerose: 0,3-1%
Histologisk otosklerose:
Kvinder/mænd =
Etnisk variation
2/1
1/1
10%
(klinisk)
(histologisk)
(Afrika, Europa, Indien)
50% har familiær disposition
tvillingestudier +
Debutalder oftest 20 – 40 år
debut/exacerbation under graviditet
Otosklerose, ætiologi:
Osteodysplasi
Callus dannelse i mikrofissurer
Mekaniske kræfter ifm tygning
Metaplasi i embryonale bruskrester
Osteocytær osteolyse
Enzymatisk defekt
Collagen type 2 autoimmunitet
Lokaliseret mb. Paget/ osteogenesis imperfecta
Virus infektion – morbilli, hundesyge
Genetisk – OTSC1-8
Det osteodynamiske koncept
Perilabyrinthine bone resorption is low
Effect: Perilabyrinthine drift barrier
Perilabyrinthine bone remodeling in vivo
bone turnover is 10% per year in long bones
bone turnover is 0,13% per year in perilabyrinthine bone
Perilabyrinthine bone remodeling in vivo
bone turnover is 10% per year in long bones
bone turnover is 0,13% per year in perilabyrinthine bone
bone turnover is distributed centrifugally around the inner ear
Extensive lacuno-canalicular
network …
Lacuno-canalicular pathway, bulk stain, basic fuchsin
The effect:
When bone matrix gets older – it cracks!
When osteocytes get old – they degenerate and die!
Accumulation of microfissures and osteocyte apoptosis
An osteo-dynamic concept of
the pathogenesis of otosclerosis:
1. Perilabyrinthine bone resorption is normally suppressed
by a local inner ear signal via the lacuno-canalicular
pathway.
2. Resulting accumulation of perilabyrinthine microfissures
and osteocyte apoptosis may interrupt the bone signaling
pathway, and upset capsular suppression locally.
3. When the suppression/remodelig balance fails locally and
a resorptive threshold is transgressed – otosclerosis may
result.
An osteo-dynamic concept of
the pathogenesis of otosclerosis:
Microfissures as well as otosclerosis accumulate in
perilabyrinthine bone, where bone resorption is low
(inverse relation).
An osteo-dynamic concept of
the pathogenesis of otosclerosis:
Microfissures increase with age (from 16 years)…
followed by a similar increase in otosclerosis.
Treatment:
Hearing aid
or
stapedotomy
Different techniques
and prosthesis types
Otosclerosis – Stapes prostheses
Material:
q Platinum / teflon.
q Gold
q Titanium
q Nitinol
Diameter:
0.4 – 0.6 – 0.7
ABC, female, 34 years old (1995)
Pre opr. Dec 1995
Post opr. control Oct. 1997
Dec 1995: Stapedotomia dx (Argon Laser)
Stapes Surgery – Hearing results
Air Bone Gap
closure, dB
-10
0
11
21
>
0
10
20
30
30
Sensorineural
hear.loss
Anacusis
Fistula
200 consec.
stapedect.
78 consec.
stapedot.
(pick, handdrill)
%
29 
28  83%
26 
7
5
%
13 
44  90%
33 
4
4
4
0,5
0,5
2
0
0
84 consec.
Argon--laser
Argon
stapedot.
%
29 
55  98%
14 
1
1
0
0
0
BC tærsklen forbedres når stapes mobiliseres ved
operation.
Hvis præoperativ BC benyttes ved beregning af postoperativ
ABG vil den kirurgiske succes blive overvurderet
ABG skal altid findes ud fra de aktuelle måleværdier
Realistisk
postoperativ
ABG:
90% kommer
under 20dB
Næsten
20% har
alligevel
SRT>30dB
og dermed
behov for
HA
Tinnitus før og efter stapedotomi
tinnitus, % af total
tinnitus 2-6mdr postop
præoperativ status
ingen
mere tinnitus end
præop.
mindre tinnitus end
præop.
som
præoperativt
I alt
ingen tinnitus
36%
1%
0%
2%
38%
tinnitus
26%
3%
15%
18%
62%
I alt
62%
4%
15%
20%
100%
38% har ingen tinnitus før operation,
heraf får 3% tinnitus efter stapedotomi
62% har tinnitus før operation,
heraf får 5% mere tinnitus efter stapedotomi,
2 ud af 3 får mindre eller ingen tinnitus.
Komplikationer efter stapedotomi
chorda
2-5mdr postop
>1år postop
11/ 112
2/ 77
2-5%
n. Facialis
1/ 112
0/ 77
<1%
Vertigo
0/ 112
0/ 77
0-2%
Anacuse
0/ 112
0/ 77
0-2%
Tilbyd altid
patienten et
HA først !
Fremtid - øget accept af
HA ?
Mindre accept af risiko
ved operation ?
Årsager til Døvhed. %
CI-patienter OUH
Arv (Progred.)
7
6 4
Infektion
Blandet
13
52
Trauma
18
Otosclerose
Streptomycin
Ørets virtuelle anatomi i 3-D og stereoskopi
Mads Sølvsten Sørensen
Øre-næse-halskirurgisk klinik F 2074
Rigshospitalet, Danmark
Haobing Wang, M.A.
Saumil Merchant, M.D
Massachusetts Eye and Ear Infirmary
Harvard Medical School, Boston, USA
https://research.meei.harvard.edu/Otopathology/3dmodels//3Dviewer.html
VES. The Visible Ear Simulator
Sorensen MS, Mosegaard J, Trier P. The Visible Ear Simulator: A Public PC
Application for GPU-Accelerated Haptic 3D Simulation of Ear Surgery Based on
the Visible Ear Data. Otol Neurotol 2009; 30: 484-7.
http://www.alexandra.dk/ves/index.htm.