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Otosclerosis
- 1. OTOSCLEROSIS Dr. Rajesh Maharjan ENT1st year Resident GMC
- 2. INTRODUCTION • Localized hereditarydisorder of bone metabolism of otic capsule enchondral bone that is characterized by disordered resorption and deposition of bone • Bone resorption by osteoclasts and new bone formation by osteoblasts, accompanied by vascular proliferation and tissue stroma • New vascular spongy bone formation causes ankylosis
- 3. Antonio Valsalva Italian Anatomist Firstdescribed ankylosis of stapes in 1741
- 4. Joseph Toynbee • Firstdescribed the condition causing a hearing loss by fixation of the stapes in 1860
- 5. Anton von Tröltsch •1869 • Final inactive sclerotic stage of the disease, "sclerosis”
- 6. Adam Politzer 1893 Coined theterm Otosclerosis in
- 7. Friedrich Siebenmann Swiss otolaryngologist Coinedthe term Otospongiosis in 1912
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- 9. EPIDEMIOLOGY • Exact etiologyis unknown (? Viral) • Paget’s disease • Measles virus protein, low level of anti measles IgG • Autoimmune • Humoral mediated • Type II and IX collagen
- 10. EPIDEMIOLOGY • Autosomal dominant:variable penetrance • Race: common in white races & Indians • Female : Male = 2 : 1 • Age: Common in 20 - 30 years • Hormonal influence: es in pregnancy, menopause, stress (trauma, surgery)
- 11. TYPES I. Clinical otosclerosis •Stapes • Stapediovestibular joint or round window membrane II. Cochlear otosclerosis • Cochlear endosteum without affecting the stapes or the stapediovestibular joint III. Histological otosclerosis • Histopathological lesions • Do not affect the stapes, stapediovestibular joint or cochlear endosteum • Asymptomatic
- 12. HISOTPATHOLOGY • Two cytokines, •Osteoprotegerin (OPG) • Potent inhibitor of osteoclast activation and maturation • RANKL (receptor activator nuclear-kb ligand) • Osteoclast activator • Breakage in signaling pathway between two cytokines
- 13. LIGHT AND ELECTRONMICROSCOPY • Earliest indication • Resorption of enchondral bone around blood vessels • Enlargement of perivascular spaces followed by deposition of immature (woven) bone • Active otosclerotic foci • Increased vascularity and increased bone turnover • Inactive (sclerotic) foci • Dense mineralized bone • The connective tissue stroma in the foci consists • Fibroblasts and osteocytes • Complete absence of acute inflammatory cells
- 14. ORIGIN AND SITES •Wall anterior to the oval window > round window niche > cochlear apex • Less frequent sites • Posterior to the oval window • The internal auditory canal • Around the cochlear duct and the semicircular canals • Middle ear ossicles • Labyrinthine spaces and the vestibular aqueduct
- 15. CAVITATING OTOSCLEROSIS • Largecavities may form within otosclerotic foci • If contact with endosteum of scala tympani • 3rd window • Persistence of air bone gap following stapedectomy • Communicate with the CSF space in the internal auditory canal • Result in gushers during cochlear implantation
- 16. TYPES OF STAPEDIALOTOSCLEROSIS 1. Anterior focus (commonest): 2 mm anterior to oval Window 2. Posterior focus: 2 mm behind oval window 3. Circumferential: involves footplate margin only
- 17. Types of StapedialOtosclerosis 4. Biscuit type: footplate involved, margin is free 5. Obliterative: obliterates oval window completely
- 18. Symptoms of Otosclerosis •Hearing loss: Bilateral, slowly progressive • Conductive: stapedial otosclerosis • Sensori-neural: cochlear otosclerosis hyalinization and atrophy of spiral ligament • Mixed: stapedial + cochlear otosclerosis • Vestibular symptoms • Vestibular ganglion degeneration • Asso. Meniere’s disease • Vestibular aqueduct filled with active otosclerotic foci
- 19. Symptoms of Otosclerosis 3.Paracusis Willis: Better hearing in noisy surroundings (people increase their voice intensity & pt’s speech discrimination becomes better).
- 21. DIAGNOSIS • Otoscopy • Normal,mobile tympanic membrane • A red blush of the tympanic membrane over the promontory ‘Flamingo flush’ or Schwartz sign
- 22. TUNING FORK TESTS RinneWeber A.B.C. Stapedial Negative (BC > AC) Lateralizes to diseased ear Normal Cochlear Positive (AC > BC) Lateralizes to Better ear Decreased Mixed Negative (BC > AC) Lateralizes to Better ear Decreased
- 23. GELLE & BINGTESTS Vibrating tuning fork placed over mastoid & then: External auditory canal is blocked in Bing test or E.A.C. pressure ed by Siegalization in Gelle test Bing Gelle’s Otosclerosis No change No change Normal / SNHL Intensity es Intensity es
- 24. PURE TONE AUDIOMETRY •Low frequency conductive deafness • Carhart’s notch in bone conduction at 2 KHz • All CHL, irrespective etiology
- 25. CARHART EFFECT • Highestfor 2 kHz
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- 28. RADIOLOGY • High-resolution CT(HRCT) • Active otosclerosis as hypodense or lucent areas within the otic capsule, typically anterior to the oval window
- 29. SURGICAL DIAGNOSIS • Middleear anatomy normal • Bone around oval window whiter than normal but no clear junction • Footplate thicker
- 30. HISTOLOGICAL DIAGNOSIS • Onlyif stapedectomy performed • Stapedotomy • Stapes superstructure • Non otosclerotic bone
- 31. DIFFERENTIAL DIAGNOSIS • OtitisMedia with Effusion: type B tympanogram • Adhesive Otitis Media: absence of T.M. mobility • Tympanosclerosis: white patch on T.M. • Ossicular discontinuity: type Ad tympanogram • Congenital ossicular chain fixation: tympanotomy • Malleus head fixation: tympanotomy
- 32. MANAGEMENT • Medical • Fluoridationof drinking water • No data till date in support • Oral Fluoride • Given in active focus of otospongiosis (Schwartze sign) • Acts by: a. Decreases bone resorption b. Increases bone formation • 20 mg orally, thrice daily for 3 - 6 months • S/E arthritis, gastroenteritis, anemia, plantar fascitis • Bisphonates • may reduce the deterioration of sensorineural hearing loss over time
- 33. HEARING AIDS • Unfitfor surgery • Disadvantage • Aesthetic concern as patient usually of young age • BAHA (Bone Anchored Hearing Aid) • An only hearing ear with otosclerosis • Difficulty using a conventional aid • Post-fenestration cavity
- 34. FAR-ADVANCED OTOSCLEROSIS • Averageair-conduction of > 85 db • Bone conduction immeasurable due to limits of audiometric equipment • Distinguish from Pure SNHL • Timescale of the hearing impairment • Family history, previous audiograms showing an air–bone gap • No CT evidence of cochlear otosclerosis • Managed in 1 of 3 ways • Stapedectomy and subsequent conventional hearing aids • Cochlear implantation • The new direct acoustic cochlear stimulation (DACS) device
- 35. DIRECT ACOUSTIC COCHLEARSTIMULATION (DACS) • The device consists of an active middle ear implant attached to a stapes prosthesis • Mixed hearing loss where a successful stapedotomy alone would not allow the patient to manage without a hearing aid • Indications • Minimum average bone-conduction of over 30 db and with an additional air–bone gap of over 30 db
- 36. COCHLEAR IMPLANT • Patientswith FAO or failed stapedotomy may be candidates for cochlear implantation • There may be problems in electrode placement due to otosclerotic ossification of the round window or basal turn of the cochlear which may necessitate extra drilling
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- 38. SURGERY • 1878 • Transtympanicmobilization and removal of the stapes • 1899 • 6th International Otologic Congress in London Johannes Kessel
- 39. SURGERY • 1956 • Binocularmicroscope • Stapedotomy • Ossicular reconstruction/teflon piston • Vein graft John Shea
- 40. INDICATIONS • Hearing thresholdfor air conduction 30 dB or more • Average air bone gap at least 15 dB or more at frequencies of 250 Hz to 1 kHz • Negative Rinne at 512 Hz
- 41. INDICATIONS • Bilateral involvement •worse hearing ear/ interval 1 yr • Symmetric loss • Patients preference • Concomitant sensorineural loss is not a contraindication for surgery
- 42. CONTRAINDICATIONS • Only hearingear • Infection of external or middle ear • Pregnancy • Tympanic membrane perforation
- 43. FACTORS TO CONSIDER •Age • Children • CT temporal bone • Congenital Middle/inner ear malformation • No upper age limit • Occupations • Scuba diving, commercial air travellers, parachuting • Barotrauma • Tympanometric pressure of 400 mm of H2O, if no nystagmus • Work in noisy environment • Ménière’s disease • Distended saccule close to oval window
- 44. INFORMED CONSENT • Descriptionof the procedure and discussion of all potential risks • Occupational dependence on taste
- 45. OPERATIVE NOTE • Shapeand mobility of the incus and malleus • Presence of otosclerosis • Fixation of the stapes • Patency of the round window • Location of and the bone covering the facial nerve • Status of the chorda tympani
- 46. ANESTHESIA • Local anesthesia •Time saving • Intraoperative report of vestibular symptoms • Prevention of excessive inner ear irritation • General • Painless • Motionless operative field
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- 48. POSITIONING • Supine headtilted 10-15 down • Head turned to contralateral shoulder • Tympanic membrane almost horizontal plane
- 49. EXPOSURE AND EXPLORATION •Speculum holder attached to the bed or the headrest • Size • Too small • Move against EAC • Restrict vision and instrumentation • Too large • Push soft tissue of EAC medially • Obstruct view • Should wedge into lateral aspect of bony canal
- 50. EXPOSURE AND EXPLORATION •Local anesthesia 1% lidocaine and 1:100,000 epinephrine • Minimizes bleeding • 27 gauge needle 4 quadrants of cartilage EAC • Lateral to junction • Bevelled 30-gauge needle into subperiosteal plane at 6 and 12 o’ clock
- 51. EXPOSURE AND EXPLORATION •Permeatal incision
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- 55. VISUALIZATION MIDDLE EAR •Oval window niche • Pyrimidal eminence • Tympanic segment of Facial nerve • Round window • Operative note • Malleus incus, stapes fixation, chorda tympani, patency round window
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- 63. FENESTRATION • Site • Middleand inferior 3rd • Greatest distance from footplate to membranous labyrinth • Argon vs Carbon dioxide • Visible light range • Laser vs Microdrill • Cut and coagulate • No pressure and movement • Less irritation and trauma perilymph
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- 65. Piston placed inperforation
- 66. ALLIGATOR FORCEPS • Graspedby its loop • Pace to fenestra and incus in one movement • Site • Narrowest area of incus
- 67. CRIMPING • Differential movement •Delayed failure
- 68. POSITION • Perpendicular tofootplate and long axis incus • Fitting fenestra snugly but freely moving
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- 72. STAPEDECTOY • Free floatingfootplate • Communited fracture footplate • Inadvertently removed during superstrucure removal • Revision surgery • Instrument for fenestration not availabe
- 73. STAPEDOTOMY VS STAPEDECTOMY •Hearing difference not significant • Vestibular symptoms • Occurrence, duration, and severity
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- 77. Prosthesis placed overvein graft
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- 79. INTRAOPERATIVE • Tear tympanomeatalflap • Perforation TM • Subluxation Incus • Facial nerve overhanging • Obliterative otosclerosis of the oval window • Otosclerosis involving round window • Malleus ankylosis • Perilymph gusher • Floating and depressed footplate
- 80. POST OPERATIVE • Facialpalsy • Chorda tympani dysfunction • Otitis media • Vertigo • Sensorineuronal hearing loss • Conductive hearing loss • Malfunctioning prosthesis Malleus fixation OM, DSSC, round window obliteration
- 81. REFERENCE • Scott-Brow’s Otorhinolaryngology,Head and Neck Surgery, 8th edition • Glasscock-Shambaugh, Surgery of the ear, 6th edition