Overview of history, pathophysiology and characteristics of otosclerosis Discuss the diagnosis, clinical features and treatment of otosclerosis Discuss the surgical management of otosclerosis through stapes surgery Definition History Prevalence and Demographics Etiology and Pathophysiology Diagnosis Management Non-Surgical Surgical Conclusions Metabolic bone disease of the otic capsule and ossicles Involves abnormal resorption/deposition of bone which can lead to fixation of the stapes Present with slowly progressive conductive or mixed hearing loss 1735, Valsalva-described ankylosis of the stapes to the oval window 1860, Toynbee-described fixation of the stapes as a cause of hearing loss 1893, Politzer-first use of the term otosclerosis to describe ankylosis of stapes footplate 1912, Siebenmann-microscopic examination of the otic capsule revealing spongification of bone 1938, Julius LempertEndaural HSSC fenestration operation 1953, Samuel RosenStapes mobilization 1955, Fowler-Anterior crurotomy w/ footplate fracture 1956, John Shea Jr.Stapedectomy with vein interposition graft and teflon prosthesis Cholesteatoma Ossicular discontinuity Congenital stapes fixation Non progressive early hearing loss Malleus head fixation Paget’s disease Older onset, high alk phos, multiple sites Osteogenesis imperfecta Blue sclera, multiple fx, progressive hearing loss Superior semicircular canal dehiscence CHL, Tullio’s phenomenon, CT findings Non-Surgical Hearing Aid Sodium Fluoride Bisphosphonates Surgical (stapedectomy, stapedotomy etc.) Antagonist of bone remodeling Replaces hydroxyl radical in hydroxyapatite Thought to decrease amount of proteolytic enzymes present in perilymph Fenestration of HSCC, Mobilization of footplate, Anterior crurotomy( no longer performed) Stapedectomy Total vs. Partial Stapedotomy( laser assisted or microdrill) Patient Selection ABG of at least 15 dB Worse hearing ear Good word discrimination Relative contraindications Active infection TM perforation Meniere’s disease Pregnancy Dilated cochlear or vestibular aqueduct Absolute contraindication should be if affected ear is the only hearing ear Surgery is performed transcanal through a fixed speculum ( i.e Shea speculum holder) Canal is injected at bony cartilaginous junction in 4 quadrants Middle Ear examination Evaluate ossicular chain mobility Confirm stapes fixation Evaluate for middle ear anatomical variants( i.e dehiscent facial nerve) Prosthesis Selection(Length x Width) Length- measured from the medial portion of the incus, but the measurement is taken from the lateral surface ▪ Subtract 0.25 mm from 4.5 mm= 4.25 mm in length ▪ Husain and Selesnick demonstrated no significant difference in ABG closure among lengths ranging from 3.75 to 4.75 mm. Width- 0.6 mm is standard. Laske et al performed meta analysis showing significantly improved ABG closure with 0.6 vs. 0.4. Prospective study over 14 years Involved 2,525 patients, 3050 stapedectomies, adult and pediatric arms All had vein interposition w/ prosthesis ABG closure to 10 dB in 94.2% of patients SNHL (>15 dB) seen in <0.5% of patients SNHL Taste disturbance Tinnitus Vertigo Facial paralysis Perilymphatic fistula Reparative granuloma Otosclerosis is characterized by gradual progressive CHL or mixed hearing loss that is usually bilateral and can be asymmetrical Hearing aids are effective alternative and and often are offered in lieu of surgery In the literature, >90% of patients experience closure of ABG to within 10 dB with <1% experiencing SNHL Comparisons regarding technique are equivocal and ultimately depend on surgeon skill or comfort level with a given technique( ie laser vs. drill; total vs. stapes fenestration) No definitve guidelines have yet been established for the use of cochlear implant in advanced otosclerosis