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.
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