Literature: Hans Behrbohm, Oliver Kaschke, Tadeus Nawka,Andrew Swift: Ear, Nose, and Throat Diseases: Founding Authors W. Becker, H.H. Naumann, C.R. Pfaltz (Paperback) Publisher: Thieme Publishing Group; 3rd Revised edition edition (12 Aug 2009). 471 pages , Language English. ISBN-10: 313671203X, ISBN-13: 978-3136712030. Med Servis Mgr. Jaroslava Wilhelmová, Všetičkova 29, 602 00 Brno. Tel./fax. 05/43241146 . Ear Cochlear cross-section Cochlear duct cross-section Conductio of vibration from tymp.membr. through cochlea, Organon Corti OHC = servomechanism for IHC Cochlear septum ENT investigation  history of disorder, in otology: hearing, tinnitus, dizzines, otorrhea (discharge), pain...  inspection, palpation and using investigation instrumentarium (otoscopy, otomicroscopy )  Radiography, ultrasound – Sumation x-ray: Schüller, Stenvers, Meyer – CT : axial and coronar sections  functional investigation „classical hearing test“, audiometry, tympanometry, brain-stem electric response audiometry (BERA), spontáneus otoacustic emission  endoscopic investigation  otoneurology  other: laboratory... History of disorder Physician itself is a „remedy“ (Michael Balint) History of disorder – ear disease symptomatology • Hearing • Tinnitus • Dizzines • Otorrhea (discharge) • Pain Vertigo (dizziness)  Periferal type – feeling of rotation of itself body or surroundings, direction of rotation is usually into healthy part, loss of stability or feeling of swimming  Central type – ineptitude by walk, inability of walk, vertigines with aura (EPI), disorder of vision "black outs" – diplopia is seen by disorder of oculomotory. Otoscopy eye evaluation of deeper parts of external meatus and ear drum Normal otoscopy: Bezold´s trias: 1. Prominentia mallearis 2. Stria mallearis 3. Light reflex Otoscopy – tympanic membrane quadrants and zones Normal ear drum Basic patologic finding on tympanic membrane  Injection of the vessels of the tympanic membrane  (position) bulgging due to exudate - hyperemia, moist infiltration and opacity of the surfice, the contours of the handle of mellaeus and short process disappear  retraction - injection of blood vessels  (integrity) perforations – – after injury – inflammatory - acute - chronic - central (mesotympanic) - marginal (peripheral)  (changes after infamm.) thickening of the tympanic membrane, scars  changes behind the ear-drum: middle ear efussion, fluid level, air bubbles Various types of perforations of ear drum Schema of CT of ossicular chain and middle ear cavity Canalis Fallopi Disorder of the ear congenital anomalies inflammations tumors injuries Microotia III. St. Microotia Treacher-Collins syndrome Most affected individuals have underdeveloped facial bones, particularly the cheek bones, and a very small jaw and chin (micrognathia). Hearing loss occurs in about half of all affected individuals; hearing loss is caused by defects of the three small bones in the middle ear, which transmit sound, or by underdevelopment of the ear canal. People with Treacher Collins syndrome usually have normal intelligence. Apendices praeauriculares Apostasis auriculae Erysipelas bullosa auricullae Herpes zoster oticus (part of Ramsey-Hunt syndrome) acute finding– after 3 days – after 10 days Perichondritis Chronic polychondritis – alergy- cauliflower ear Spinocellular cancer of auricle Othematoma (čerstvý úraz, po 14 dnech, po několika měsících) Cerumen Foreign body in external meatus - insect Ventilační trubička k drenáži středouší (nezávislý nález) Foreign body in external meatus bead, piece of wood, blood Exostosis in ext. meatus right Furunculus of external meatus Tympanojugular chemodectoma Central perforation Central perforation in anteroinferior quadrant Velký stopkatý polyp u chro. zánětu středouší Defekt epitympanálníh prostoru po odstranění cholesteatomu Hemotym panum, vpáčený bubínek Traumat. perforace Otitis media acuta Otitis med. ac. l. sin. – progressive inflam. changes Otitis med. ac. sin. with myringotomy and restitution Paracentesis Perforation of ear drum Scared thickened ear drum after otitis Scared thickened ear drum with calcification Subtotal perforation of ear drum Basic types of hearing disorder Sensorineural deafness Weber unto better hearing Rinne positive Schwabach shorter Conductive hearing loss Weber unto worse hearing Rinne negative Schwabach longer Mixed hearing loss Schwabach shorter Rinne negative Peripheral until entry the VIII nerve into brainstem Central Central hearing pathways and centers Retrocochlear Cochlear Basic types of hearing disorder P L 4 V 10 O,5 Vs 10 W + R + zkr. Sch norm Hypacusis perceptiva (Sensorineural deafness) Weber unto better hearing ear Rinne posit Schwabach shorter Peripheral = until entry the VIII nerve into brainstemRetrocochlear Central = central hearing pathways and centers Cochlear P L 4 V 10 3 Vs 10 W –– R + prod. Sch norm. Hypacusis conductiva (Conductive hearing loss) Weber unto worse hearing ear Rinne negat Schwabach longer Hypacusis mixta (Mixed hearing loss) Schwabach shorter Rinne negat. Hypacusis Conductive Sensorineural Mixed Speech audiometry Patient repeats words which are reproduced. One correctly repeated word means 10% of understanding from one set. It is evaluated on increasing levels of intensity till 100% of understanding or maximally possible per cent of understanding. BERA –prodloužená latence o 0,2 ms – suspekce na malý neurinom n. VIII Sensorineural hearing loss Dle průběhu audiometrické křivky  Basokochleární  Pankochleární  Apikokochleární  Mediokochleární Dle lokalizace léze  Kochleární  Retrokochleární Cochlear lesion - bothering, but not life threating Retrocochlear lesion - bothering, but also they could life threaten Sensorineural hearing loss cochlear = damage of cochlear structures  Etiology: – Presbyacusis – Heredodegenerativní – Nois damage – Toxic damage – Menier´s disease – Acute sensorineural hearing loss … atd. Sensorineural hearing loss retro- (supra-) cochlear = damage of structures proximal from cochlea Etiology: Demyelinization - atherosklerosa - sclerosis multiplex Inflammation - boreliosa - neuroviry - meningitida - meningoencefalitida Tumors - neurinom akustiku (vestibulární schwannom) - meningeom - jiné nádory MK a koutu MM Trauma - komoce, kontuze - fraktury base lební … atd Differencial diagnosis cochlear/retrocochlear 1. Subjective tests: - time demanding - active cooperation of pt - complicated for understanding - relatively low validity 2. Objective tests : - časově obvykle méně náročné - vyžadují pasivní spolupráci pacienta - náročné na technické vybavení - mají vysokou validitu Subjektivní testy Založeny na principu průkazu: - recruitment fenomenu - maskovacího efektu šumu - míry únavnosti sluchového orgánu Recruitment fenomen = abnormal increase of loudness in above-treshold in damage of OHC and normal function IHC SI-SI Short Increament Sensitivity Index short time 1 dB increase of intensity 20 dB above treshold (20x) „Hum“ audiometry Langebeck test = increase ability to mask tons by hum in supracochlear hearing loss. Tresholds are higher about more than 10 dB as level of ripple. Langebeck test Suprakochlear hearing loss Tympanometry Surgery for otitis media -Sanation surgery Approach – Schwartze - via planum mastoideum into antrum – Stake - via atticus into antrum – Zaufal – via posterior wall into aditus ad antrum and from this antreriorly and posteriorly Sanation surgery – atticotomy – meatoantrotomy – atticoantrotomy – tympanomastoidektomy Status post mastoid- ectomiam Status post atticoantrotomiam l.dx. Relationship of external meatus to trepanation cavity Possibility for improvement of hearing by surgery and prosthetics Middle ear implant Systém for direct bone conduction External bone hearing aid Middle ear surgery Hearing aid for air conduction PONTO OtomagBAHA Improvement of hearing BONEBRIDGE Implantable hearing aids  Cochlear implants  Bone conduction implants Middle ear implants (MEI) Systems for bone conduction Vibration direct to bone Generating behind skin Systém pro kostní vedení s aktivním implantátem (BONEBRIDGE) Transcutaneous conduction Zevní systém ukotvený v kosti (BAHA) Vibration over skin Hold by the pressure from outward Brýle s kostním vibrátorem Kapesní sluchadlo s vibrátorem na čelence Hold by implanted magnet Systém pro kostní vedení s pasivním implantátem (BAHA Attract, Sophono)  Bone conduction headband  Also for preoperative evaluation Solution for first treatment before BB implantation Bone conduction with active and passive implant Passive implantActive implant BONEBRIDGE Bonebridge System Overview DEMOD Transducer Receiver Coil & Attachment Magnet Skin Amadé BB BCI 601 (Implant) Acoustic Input Force Output to Skull OptimizedTransfer BC-FMT = Bone Conduction Floating Mass Transducer Bonebridge Bone conduction thresholds within the shaded area Audiological Indication Conductive and Mixed Hearing Loss Bone conduction thresholds F (Hz) 500 1000 2000 3000 BC limit 45 45 45 45  Profound unilateral SNHL  Normal hearing contralateral ear Audiological Indication Single Sided Sensorineural Deafness F (Hz) 500 1000 2000 3000 Contralateral HL limit 20 20 20 20 - Intolerance to the following materials - Medical grade silicone elastomer (BCI) - Titanium (BCI) - Titanium alloy (screws) - Xylex ® Resin (Amadé BB) - Skin or scalp conditions precluding AP attachment → medical history, medical checkup - Retrocochlear component → speech test - Pathological situation precluding BCI placement → Osteoiditis - Anatomical situation precluding BCI placement → high resolution CT scan! Contraindications Radiological Planning Key-Dimensions of the BCI  Positioning of the coil • Sound quality, comfort  Positioning of the BC FMT: • Avoid the sinus as first priority • Assess where to expect the dura  Positioning of the screws • There should be enough cortical bone to position the screws • Screws should be on the same plane Anatomical Considerations Analysis of the CT Scan Sigmoid Sinus Dura Anatomical Considerations Structures to preserve EAC Thickness of the skull Cortical part of the skull Anatomical Considerations Structures to consider  Standard screws as used in trauma surgery are packaged with the active implant  Screws are self-cutting, but not self-drilling  Total length 6mm, drill depth 4mm  Energy/Sound transfer exclusively via the screws! Cortical Screws – Energy transfer C-Sizer, T-Sizer, Drill: The Complete Kit C-Sizer (Coil-Sizer): To create the skin flap T-Sizer (Transducer-Sizer): To create the X-ducer bed, also serves as a drill guide Drill 1.5mm: To pre-drill for screws, includes a stopper Active implant Cortical screws 2 standard 1 „emergency“ Bonebridge- fast view První implantace BONEBRIDGEv ČR  Pacient s Treacher-Collinsovým syndromem a atresií zvukovodů  Normální kostní vedení a plná kochleární rezerva oboustranně  Operace proběhla na sále Kliniky otorinolaryngologie a chirurgie hlavy a krku při FN u sv. Anny v Brně dne 29.8.2014  Výkon i pooperační hojení bez komplikací 1. Příprava – vyholené vlasy, naznačená incize 3. Vytvoření lůžka, zavedení měrky pro převodník (T-sizer) 6. Fixace „převaděče“ a uzávěr rány Hearing before and after surgery Vibroplasty - soundbridge Surgery treatment of middle ear inflammation – reconstructive surgery (tympanoplasty) According to prof. Wulstein: I. Myringoplasty II. Columelisation of incus III. Columelisation (stapes) IV. Ekranisation (zástin okrouhlého okénka V. Fenestration olf labyrinth Tympanoplasty - typ I. Myringoplasty Tympanoplasty II. Incus columelisation Tympanoplasty type III.a damaged incus and maleus; stapes intact, sound conducted by prosthesis PORP PORP partial ossicular replacement prosthesis PORP Tympanoplastika typ III.b damaged incus, maleus and suprastructure of stapes, sound conducted by prosthesis TORP TORP Total ossicular replacement prosthesis TORP Tympanoplastika typ III.c Kolumelisation damaged incus and maleus; stapes intact, connected direct to myrinx - myringostapedopexe Tympanoplasty type IV. Ekranisation (cover of round window) Tympanoplasty type V. Fenestration (created new window into labyrinth by surgery) Syndroma: Van den Hoeve de Klein Otosclerosis • Fixation of stapes bilaterally • Blue sclera • Osteogenesis imperfecta Stapedoplasty PISTONs