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    O To Sclerosis

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    Otosclerosis Brandon Isaa Otosclerosis (OS) is a fibrous osteodystrophy of the human otic capsule. Its clinical manifestations are primar. ily conductive hearing loss (CHL), although sensorineural hearing loss (SNHL) and mixed hearing loss (MHL) can also occur. The disease process causes abnormal resorption and deposition of bone. OS is noted clinically in 1% of the Caucasian population; itis transmitted in an autoso- mal dominant fashion but with incomplete penetrance Females appear to be affected twice as often as males (1) In 1873, Schwarize described a reddish hue medial 10 an intact tympanic membrane (TM), which was second ary to the increased vascularity of the cochlear promontory in active OS lesions (the phase known as otospongiosis) This finding is named afier him and is known as sign, It is seen in 10% of patients with OS, In 1881, von Troltsch noted abnormalities of the middle ear mucosa in this disease and was the first (o use the term OS, In 1893, Politzer described OS as a primary disease of the otic cap sule, rather than a condition related to previous episodes of inflammatory ear disease, as originally thought (2), The clinical entity of OS was further described by Bezold in 1908, when he discussed its historical, physi cal, and audiometric findings. In 1912, Siebenmann dis- cussed the possibility of OS causing SNIIL, Since that time, numerous etiologies of OS have been suggested, including hereditary, endocrine, biochemical, metabolic, infectious (eg, measles), traumatic, vascular, and even autoimmune faciors (3). In fact, Lopez-Gonzalez and Delgado (4) sug- gested that oral vaccination with type II collagen may miti: gate the autoimmune reaction in those susceptible to OS through hyposensitization. I is also possible that interplay of these different factors exist and vary {rom individual to individual, while causing the same pathologic and dini- cal findings, In other words, OS may be the common, final pathway of a clinically and genetically heterogeneous sroup of disorders (5) hwartze Mm Crea The maturation of the bony labyrinth plays a role in the pathogenesis of OS, The otic capsule arises from mesen chyme surrounding the olic vesicle at 4 weeks of embryo: logic development, At8 weeks, the cartilaginous framework is initiated. At 16 weeks, endochondral osseous replace ‘ment of this framework begins in 14 identifiable centers In some people, complete bony replacement does not occur and leaves cartilage in certain locations, One of these regions, the fissula ante fenestram, is anterior to the oval window (OW) and is usually the last area of endochon. dral bone formation in the labyrinth. According to tempo: ral bone studies, this region i allecied in 80% (0 90% of patients with OS (8), In 1985, Schuknecht and Barber (7) reporied other areas of predilection for otosclerotic lesions, such as the border of the round window (RW), the api cal medial wall of the cochlea, the area posterior to the cochlear aqueduct, the region adjacent to the semicircu lar canals, and the stapes footplate itself (Which is derived from otic capsule, as opposed to the superstructure, which is a branchial arch derivative), There are three forms of otosclerotic lesions: otospongiosis (early phase), transitional phase, and OS (late phase). The early active phase lesions consist of histiocytes, osteoblast, and the most active cell group, the osteocytes, The osteo: cytes resorb bone around preexisting blood vessels, which causes widening of the vascular channels and dilation of the microcirculation, Otoscopic oF mi reveal the reddish hue caused by these lesions (Schwartze sign if sen on clinical examination). As osteocytes become ‘more involved, these areas grow rich in amorphous ground substance and deficient in mature collagen, resulting in copic exam can 2487 2488 Section IK: Otology formation of new spongy bone. With hematoxylin-eosin (H&E) staining, this new spongy bone appears densely blue. This was described in 1914 by Manasse and is known, as the blue mantles of Manasse. Interestingly, mantles are found in up to 20% of normal temporal bones. On elec. ton microscopy, the foci of perivascular bony invasion coalesce as the lesions enlarge within the otic capsule (7). ‘The predominant finding in the late phase of OS is the formation of sclerotic, dense bone in areas of previ- fous osseous resorption. The vascular spaces that were once dilated are narrowed due to bony deposition. Within each temporal bone containing OS, lesions can be found in early, transitional, and late phases, although the overall histologic status of the developing lesions is fairly uniform. Although OS begins in endochondral bone, as the spon- siosis and sclerosis continue, the endosteal and periosteal layers also become involved (8). BASIC SCIENCE A number of mechanisms including autoimmune, genetic, and infectious have all been described as potential caus- ative factors for the development of OS, Definitive evidence for an autoimmune etiology for OS is currently lacking Conflicting evidence exists for increased levels of type ILand 1X collagen antibodies in patients with OS. Animal models of type I collagen autoimmunity were found to have lesions similar to OS in contrast (o another nearly identical study where autoimmune mice were found to have no lesions (5). (OS has a significant genetic component with seven dis- tinct loci reported to date, Autosomal dominant transmis sion with incomplete penetrance is the predominant mode of inheritance. The OS loci identified include genes that regulate growth regulation, intercellular communication; cartilage, bone, and collagen homeostasis and metabolism, Additional work is needed to identify potential candidate ‘genes involved in the pathogenesis of OS (9). ‘A number of findings point toward a viral etiology for 8, Measles antigens and RNA, a5 well as nucleocapsic structures identical to measles virus have all been identi- fied in otosclerotic lesions. Increased levels of measles-spe- cific IgG have also been detected in the perilymph of OS patients undergoing stapedectomy. It is not yet certain that the measles virus is involved in the development of OS, and the pathogenesis has yet to be elucidated (9). ‘A more in-depth understanding of the molecular biol- ogy of bone remodeling has shed additional light on the pathogenesis of OS, The otic capsule is unique compared to the rest of the skeleton in that afier the age of one, no further osseous remodeling occurs. Bone remodeling is rigorously regulated via a balance between the cytokines and receptor: osteoprotegerin (OPG), receptor activator of nuclear factor kB (RANK), and RANK ligand (RANKL), RANKL is present on the surface of osteoblasts and binds to RANK receptors on the surface of osteoclast precursor cells, which results in osteoclast differentiation, activation, and subsequent bone remodeling, Soluble OPG competes with RANKL in binding to the RANK receptor on the sur face of osteoclast precursor cells and results in decreased, bone remodeling. A fine balance exists between RANKL and OPG, which regulates skeletal remodeling including the otic capsule. High levels of OPG (reduced predilection for bone turnover) have been detected in the inner ear and are secreted into perilymph by type I fibrocytes located within the spiral ligament. It has been postulated that genetic, infectious, and autoimmune mechanisms likely alter this pathway, which eventually results in OS (9), PATHOPHYSIOLOGY ‘The areas of OS involvement dictate the clinical presen tation. The most common type involves the stapes and accounis for those cases in which CHL is the presenting symptom, The CHLis due to fixation of the stapes footplate ‘usually beginning a the issula ance fenestram (Fig. 154.1. Progressive involvement of the footplate can create a thick focus of OS that fills the OW niche (obliterative OS) (5). 1f OS involves only the footplate and spares the annu lar ligament, minimal fixation may occur. Such a thickened footplace is called a biscuit footplate. Because of minimal fixation, biscuit footplates can become mobilized inadver- tently during a stapes procedure. placing the patient at a hhigher risk of postoperative SNHL. The RW is involved in 30% of all clinical cases of OS; complete closure of this niche is uncommon (10) SNHL a8 a result of OS is an ongoing controversial subject. Some patients with OS have a greater amount of SNHL than expected considering their age and history of noise exposure. The mechanism for the SNHL is possibly the liberation of toxic metabolites into dhe inner ear with resultant injury to neuroepithelium, vascular compromise, fr direct extension of lesions into the cochlea, causing Figure 154.1. Photomicrograph of temporal bone section in 8 79-year-old Caucasian male demonstrating a mature otosce- Fotic focus () invaving the promontory and anterior stapes foot plate (sf) st, scala tympan' v, vestibule, fr, facial nerve. (x20) (Courtesy of MIM. Paparells,drsctor, and 5. Lamey, coordinator, (Ocopathology Laboratory University of Minnesota). disruption of electrolytes and changes in basilar mem- brane mechanics, SNHL is usually associated with signiti cant stapedial OS, although some otologists contend that isolated pure SNHL can be seen without associated CHL, ‘he latter presentation is also known as cochlear OS (11). Shambaugh (11) has suggested seven criteria to identify patients suffering from SNHL due to OS 1, Schwarze sign in either ear Family history of OS 3. Unilateral CHL consistent with OS and bilateral, sym- metric SNHL Audiogram with a flat or “cookie-bite" curve with excel lent discrimination Progressive pure cochlear loss beginning at the usual age of onset for OS 6, Computed tomography (CI) scan showing demineral ization of the cachlea typical for OS 7. Stapedial reflex demonstrating the biphasic “on-off effect” seen before stapedial fixation Vestibular symptoms occur in up to 40% of patients with OS, OS lesions have been described in the lateral semicircular canal during fenestration procedures (which. have been replaced by the stapedectomy/stapedotomy), ‘The vestibular symptoms are usually not severe, but objective evidence can be obiained with electronystag. mography testing, Non-Ménitre-lype vertigo or disequ librium associated with OS has been termed OS inner ear syndrome, I isimportant to differentiate this disorder from Ménitre disease or superior semicircular canal dehiscence (SSCD). Méniére disease is an absolute contraindication for stapedectomy/stapedotomy. When the endolymphatic space is dilated (endolymphatic hydrops), the saccule may be enlarged to the point that it adheres to the undersur face of the stapes footplate. A stapes procedure can injure the saccule and result in profound SNHL. The distinction between OS inner ear syndrome and other causes of dizzi- ness is based on differences in clinical presentation, Rarely does the inner ear syndrome of OS cause well-defined epi: sodes of severe rotational vertigo, nausea, vomiting, and fluctuating SNHL. Dizziness in OS inner ear syndrome is. milder but more persistent; low frequency SNHL is gener ally not present (12). In 2004, Mikulec et al. (13) reported on eight patients with presumed OS/unilateral CHL that did not improve after a stapes procedure, These patients were ultimately found to have SSCD, In contrast to typical SSCD patients, these patients had only CHL and no vestibular symptoms. ‘One should keep this entity in ming, especially in a patient with CHL and pressure or sound-induced vertiginous symptoms. Intact acoustic reflexes in the setting of CHL should prompt further imaging to evaluate for an inner car third window. SSCD, posterior and lateral semicircular canal dehiscence, enlarged vestibular aqueduct, and modi- olar deficiencies (ie, X-linked stapes gusher) can all pres. ent with a CHL or MHL with intact acoustic reflexes (13) Chapter 154: Otosclerosis 2489 EPIDEMIOLOGY (8 is ransmitted in an autosomal dominant fashion with incomplete penetrance (25% to 40%), The degree of pen: ‘trance is related to the distribution of lesions in the otic capsule, Some lesions are located where they cannot cause clinical symptoms, About 10% of Caucasians have histo- logic findings of OS, However, of those with histologic ‘changes, only 12% have clinical symptoms; thus, overall, this represents about 1% of the Caucasian population, In the Japanese and South American populations, the inci dence is 50% of that in Caucasians. The African American population has fewer cases of OS; only 1% demonstrate histologic findings ofthe disease. In all aces, when one ear is affected, the contralateral ear shows histologic involve- ‘ment 80% of the time. Generally, the lesions occur in simi lar anatomic locations and at similar histologic phases The age at which symptoms become apparent is variable due to the insidious progression of hearing loss, but hear ing loss often begins between the ages of 15 and 45 years. ‘The average age at presentation is 33 years (14) About 60% of patients with dlinical OS report a family history of this condition. The remaining 40%, as suggested by Morrison and Bundey (14), make up a collection of cases that fall into one of the following categories: 1. Autosomal dominant inherited cases with failure of penetrance in other family members 2. Phenocopies (an individual expressing a trait that is environmentally as opposed to genetically induced) New mutations 4. ‘Those rare cases transmitted by altemate modes of inheritance (i.e, autosomal recessive) (0S has been reported to advance more rapidly in females than males, although no difference has been noted in age at onset. A recent study by Clayton et al. (15) examined the relationship in elderly women between osteoporosis and OS; both diseases show some similarities, including an ILIA gene. The study showed that a much higher percentage of women with OS also had osteo: porosis as compared with a similar aged group with only presbycusis (P< 0.007), Juvenile OS may progress more rap- idly than the adult form, Hormonal factors may play a role some females with OS appear to have theircondition worsen uring pregnancy: Estrogen receptors have been noted in the 08 plagues. However, more recent data minimize the asso ciation between pregnancy and worsening of OS (9) association with the HISTORY AND PHYSICAL EXAMINATION Patients with OS usually present with a slowly progressive hearing loss over a period of years. Patients may describe hearing speech more easily in noisy situations, The CHL improves the signal to noise ratio by subduing back ground noise (paracusis of Willis). Tinnitus is present in 75% of patients. A complete head and neck examination 2490 Section IX: Otology is performed to nule out concurrent otolaryngologic abnor- malities, Otomicroscopic examination with pneumatic insufflations is done of the external auditory canal (EAC) and TM to asses for the presence of a middle ear effusion or mass, cholesteatoma, or TM retraction. The physical appearance of the TM is normal in most patients with OS, ‘A Schwarize sign (a red to pink appearance of the cochlear promontory occasionally seen in active OS through the “TM) may be present ‘The primary purpose of performing a tuning fork exam is to confirm the findings of the audiogram, The Rinne test should demonstrate bone conduction to be better than air conduction (Rinne negative) in patients contemplat ing a stapes procedure, In the initial phases of the disease, CHL may be limited to the 256-Hz tuning fork. As foot plate fixation progresses, the 512- and 1,024-Hz tuning forks will “reverse” as well. The amount of air-bone gap required to reverse the tuning forks are about 10 to 15 dB for the 256-Ilz tuning fork and 20 to 25 dB for the 512-1lz tuning fork. The Weber test should lateralize to the ear with the greater degree of CHL, although this testis also affected by concurrent SNHL. If the tuning fork exam does not correlate with the audiogram, repeat testing is recommended since inadequate masking may falsely lead the clinician to believe a conductive loss is present in the setting of anacusis (16), AUDIOLOGIC TESTING The main objective measurement in OS is the audiogram, (Fig. 154.2). On the audiogram, OS is seen as a widening air-bone gap that usually begins in the low frequencies. Variable degrees of SNIIL may also be present, Bone con- duction may show a 20-dB loss at 2,000 Hz and a 5-dB loss at 500 and 4,000 Hz. Such an apparent depression of bone conduction at 2,000 Hz is known as Carhart notch, which is most commonly seen in OS but can be seen in other types of CHL. This notch is an artifact of the audiogram and disappears after a stapedectomy. It is secondary to sta pes fixation and a resultant change in the resonance of the otic capsule (17), ‘Word recognition scores are usually excellent in patients with OS even in the later stages of the disease process, Impedance can show reduced TM compliance (type A fr As), Slapedial reflexes are characteristically absent in the setting of CHL, Intact stapedial reflexes can occasion- ally be observed in the earliest stages of OS depending on the degree of fixation. With early stapes fixation, a char acteristic abnormal decrease in impedance may be noted at the onset and offset of the eliciting signal. This is the on-olf effect of OS. The presence of stapedial reflexes with a significant CHL warrants evaluation for an inner ear third window (ie, SCD). Vestibular testing should be included when dizziness is present. Although there ate not charac teristic findings for OS inner ear syndrome, findings or a clinical history suggestive of SSCD or Ménitre disease will alter treatment planning (17), High-resolution CT scans can help identify or confirm, Patients with OS, Radiolucent areas in and around the cochlea are noted early in the course of the disease, creating the “halo sign.” Diffuse sclerosis is found in mature cases (Cig. 154.3). Negative results on the CT scan are not diag- nostic because some patients have disease below the capa bilities of scanning protocols, The CT can rule out middle cear masses, vascular anomalies, or facial nerve abnormali- ties but is not an essential part of the workup. These scans can also assess the ossicular chain in addition to the osse ous labyrinth (cochlea, semicircular canals) (18), DIFFERENTIAL DIAGNOSIS ‘The differential diagnosis should include other causes of CHL or MHL, A history of progressive CHL of MHL in the absence of history of trauma or infection but with the pres- ence of a normal TM limits the possibilities. However, a definitive diagnosis can only be made during exploratory tympanotomy. ‘The most common conditions that mimic 8 are those that result in ossicular discontinuity or exert a mass effect on the TM or ossicles. A history of recurrent chronic otitis media suggests an ossicular discontinuity due to incus necrosis. The TM may be normal or thickened or atrophic in cases of chronic infection. The'TM in these ears is sometimes abnormally compliant, which can be manilested. as atype Ad tympanogram. Fibrous union of the incudosta- pedial (IS) joint can produce an air-bone gap wider in the high frequencies than in the lower frequencies (16). Fractures or displacement of the ossicular chain is rot uncommon in the setting of temporal bone trauma, Hemotympanum of otorthea is frequently encountered in the immediate period following the injury. A follow-up examination with audiometry is typically recommended in patients with temporal bone fractures (o allow for resolu tion of hemotympanum and spontaneous healing of TM perforation. In cases of traumatic ossicular chain displace- ment, a fibrous union often forms with resultant resolu tion of CHL. Distorted TM surface landmaris occasionally provide evidence of prior temporal bone trauma that has resulted in CHIL or MIIL, A fracture of the stapes super- structure or incus long process may have a similar audi metric configuration a5 OS Congenital stapedial footplate fixation presents at an earlier age than does juvenile OS, De la Cruz noted in his series that congenital footplate fixation was detectable at age 3, whereas juvenile OS was not detectable until about age 10 (19). In the setting of lateral ossicular chain fixa tion, the malleus and/or incus become fixed in the epi tympanum (usually at the superior malleolar ligament), resulting in immobility of all the ossicles; this can occur congenitally or may be acquired through tympanoscle- rosis. The entire ossicular chain must be examined with Chapter 154: Otosclerosis 2491 PURE TONE AUDIOGRAM Frequency in Hz 12s 250500 —(to0 20000008000 10,000 Aucogram Code = re AIR BONE al 0 [e huata| Master) Uh, ]uasiad|ooo] 4 <| to R[CO|AA] < | 0 |rm I dk e [x xfoo] >| a fo 2s] Pl ty = o—>-—0 JN 2» FA * aye ” © | o-b— » ry © 2 tg 70. Adon! Toots ~~. ee | \ 80K etd AE UAB CUNE 0 4 20 _ AUSKING \L LG 100| 100 110] 110 120 120 ‘SPEECH HEARING TESTS. Test Rf fe] ‘ssitest ale Sp:Reception Threshold SAT) | Weal Oa] wo] ao ta »|_* ‘sp. Oiserm. Scores | FO wri} 0Onl—“s| | son x| | 9) | wl —“a| Ol «| «I Sa Sh aaa on «| s{ s[ Most Comfortable Loudness (MCL) | | o | Loudness Dicomion teva.) | @| a] | a Figure 154.2 An maximum CHL the remains at 100% in both ears, every exploratory tympanotomy to avoid overlooking this lesion, Tympanosclerosis can mimic OS, but a history of recurrent otitis media or tympanostomy tubes is usually present. In addition, the TM is often thickened with associ- ated myringosclerosis, Persistent middle ear effusion, neo- plasms of the middle ear and EAC (such as glomus tumors or facial nerve tumors), and chronic suppurative otitis ,gram of a 27-year-old Caucasian woman with OS demonstrating a near ‘and a Carhare notch at 2,000 Hz in both eas, Notice that discrmination media with and without cholesteatoma can also cause CHL. Audiometry and physical examination should help make the diagnosis apparent (16), Paget disease (osteitis deformans) is a disease with dif. fuse bony involvement that is histologically similar to OS. In contrast to OS, Paget disease begins in the periosteal layer and involves the endochondral bone last. Temporal 2492 Section IX: Otology Figuro 154.3 High-resolution axal temporal bone CT scan dern- “onstrates extonsive OS involving the right cochlea. classic “halo Sign (arrow) surrounds the cochlea. bone involvement can produce SNHL, but stapes involve- ‘ment or fixation rarely occurs (20), Osteogenesis imperfect (van der Hoeve de-Kleyn syn- rome) is an autosomal dominant defect of osteoblast, activity resulting in mulkiple fractures. Stapes fixation and unique blue sclera are also found in 40% to 50% of affected patients. Stapes surgery can be performed in these patients, usually with results similar to those in patients with OS (Table 154.1) (21) History Progressive hearing loss Famiy history af O8 Trnitur Possible vestibular symptoms (rule our Méniére or SSCD) Otkis media/otorhes (absent) Head trauma fabsent) Physical examination “Tympanic membrane (normal) 31 256 and 512 He) Weber test lateraize to side wth greater CHL) Ancilary studies ‘Audiogram (assess for CHL, mixed HL, SNHL, Cathart notch) ‘Tympanomeny (type A or As; absent or on-off staped al reflex) Imaging (CT scan showing radiolucent areas around bony labyrinth), (CHL, conductive hearing loss: CT, computed tomagraphy: HL hearng loss SNH, sensormeual hearing loss SSCD,supevior semiccular canal dehtcorce MANAGEMENT Ninety percent of patients with histologic evidence of OS are asymptomatic; active lesions usually mature without stapedial fixation or cochlear loss. In the symptomatic patient, slowly progressive CHL and SNHL usually begins between the ages of 30 and 50 years with a peak incidence in the 40s (22).The disease may advance more rapidly at times, possibly depending on environmental factors, Periods of progress may be followed by periods of quies cence, The CHL stabilizes at a maximum of 50 to 60 dB. AMPLIFICATION Patients with hearing loss secondary to OS should be offered the option of amplification with typical hearing aids as an alternative to observation or surgery. Unilateral or bilateral hearing aids may provide effective treatment. Some patients may not be suitable candidates for sur- ‘ery, making amplification the only reasonable option, Another option is to use bone conduction hearing. aid Abone anchored hearing aid (BAHIA) is another option for patients with CHL or MHL secondary (0 OS who cannot ‘wear a hearing aid, Two BAIA systems are currently avail- able in the United States (BAHA, Cochlear Corporation; PONTO, Oticon), A BAHA bypasses the ossicular chain and amplifies sound that stimulates the cochlea directly through bone conduction. Mclamon et al. (23) reported that satisfaction levels for three groups of patients receiv ing a BAHA was highest in patients with congenital aural atresia, followed by patients with OS, and was lowest in patients with single-sided deafness (eg, acoustic neu- roma patients). Although amplification avoids potential risk of pro- found hearing loss that could occur from surgery, itis not capable of providing many of the benefits or patient satisfaction of successful stapes surgery. Hearing aids are usually not used at night. The canal occlusion effect. dif- ficulties with feedback, and the physical sensation of the ‘device within the EAC are disagreeable and have a negative impact on patient satisfaction, In addition, in the United Staies most insurance companies cover the costs of surgery but few cover the cost of hearing aids, which include bat- teries and a finite lifespan of 3 to 5 years For those with severe to profound SNHL bilaterally due to OS, cochlear implantation is an option. However, Rotteveel et al. (24) reported that partial electrode inser tions, misplacement ofthe electrode, and inadvertent facial nerve stimulation are more likely than in patients without (08 and normal cochlear anatomy. MEDICAL MANAGEMENT. ‘Medical therapy can be considered for all patients with OS, whether they are managed by observation, amplification, cor surgery. In 1923, Escot was the frst to suggest the use of Intraoperative Bleeding (high jugular bub, tympanomeata flap, persistent stapedialartry, mucoperiosteum) Facial nerve injury (<1%), Periymph qusher Fractura/dilocation of incur Floating foorplate Postoperative ‘Acute oct media TM perforation CCHL middle ear effusion, displaced prosthessincus erosion) SSNHL, vertigo, tinnitus (due to intraoperative trauma, labyrinth, reparative granuloma, perilymph ful Facial nerve paey (local anesthetic, intraoperative trauma, layed Bel palsy CHL, conductive hearng lose; OS, otorcloron; NHL, conaorineural bearing los TM, tympanic membrane calcium fluoride for the weatment of OS (25). Shambaugh (11) predicted stabilization of OS lesions with the use of sodium fluoride. Fuoride ions replace the usual hydeoxyl radical, forminga more stable fluorapatite complex instead of hydroxyapatite crystal. The fluorapatite complex resists osteoclastic degradation that has been confirmed with his tology. The effect of fluoride on OS remains to be eluc dated, but many otologists prescribe it to stabilize active (0S in an attempt to prevent progression of CII, SNIIL, and dizziness (25) ‘The recommended dosage of sodium fluoride is 20 to 120 mgd. Evaluation of efficacy may be based on the di appearance of Schwartze sign (if it was present), stabiliza- tion of hearing, and improvement in the CT appearance of the otic capsule. Side effects of this therapy are usually ‘minor. Gastrointestinal side eflects (nausea) may affect patient compliance, but these effects can be minimized by lowering the dose or using enteri-coated tables. Bone, muscle, of joint pain occasionally occurs with Buoride therapy, which usually resolves with temporary disco tinuation of therapy, Rarely, uid retention, cutaneous eruptions, and eye problems occur. Using this treatment regimen, patients may improve or show no progression of their symptoms (Table 154.2) (25). SURGICAL MANAGEMENT. Although Rosen (8) introduced the stapes mobilization procedure in 1953, most otologists replace the stapes superstructure with a prosthesis (due to reduced risk of recurrent fixation). The stapedectomy was popularized by John Shea in the late 1950s (26). He removed the entire footplate and placed a vein graft to close off the vestibule ‘The stapes superstructure was reconstructed with a poly. ethylene prosthesis (26). This procedure has since been Chapter 154: Otosclerosis 2493 modified by using a wire prosthesis with an attached piece of fa, connective tissue, or gelatin material, The use of gelatin material in replacement of the footplate is no lon- ser advocated secondary to the increased risk of reparative granuloma formation. Paral stapedectomy and stapedot ‘omy have also been performed (instead of a total stape sdeciomy), Further modifications of these procedures have been described, but the essential principles have remained the same (27) PATIENT SELECTION AND CONTRAINDICATIONS The clinical circumstances favoring successful stapes surgery include unacceptable CHL, reversal of the Rinne 512-Hz tuning fork exam (bone conduction greater than air con- duction), and good word recognition. When maximal CHL. due to OS coexists with significant SNIIL, detection of the bone line and conductive component can be difficult due to the limits of the audiometer. The situation is worse with, bilateral MHL. Airconduction thresholds may be present al very low levels (90 10 100 dB) of entirely absent. A his- tory of progressive hearing loss should raise suspicion that (OS may be involved. Suspicion should be heightened if dis. timination scores or the ability to function appears much. better than one would expect with such a significant degree ‘of hearing loss. The presence of As tympanograms, abnor. malities of stapedial reflex, ora family history of OS should raise suspicion even further, Use of the tuning forks may also separate advanced OS from SNHL of other causes (28) ACT scan will sometimes permita diagnosis of advanced OS when audiometry is inconclusive. If advanced OS is suspected, exploratory tympanotomy should be consid. ‘ered, Stapedectomy in such a setting can produce meaning. ful results, Air-conduction thresholds and discrimination scores can be improved enough to allow more effective amplification (29). ‘Age is an important consideration when contemplating stapes surgery. A temporal bone C'scanis recommended in. any patient presenting with a normal otoscopic exam and. conductive or MHL present since childhood, Inner ear mal formations are occasionally identified in the pediatric age group and can present with mixed or CHL, Amplification is typically recommended when an inner ear malformation is identified in patients with conductive or MHL (30). The very young patient has a higher incidence of OW reclosure after a successful initial procedure. Although revision can. be performed, a secondary procedure in any patient has a reduced success rate and a greater risk for postoperative SNHL, De la Cruz noted that in addition to being mani fest at an earlier age, congenital footplate fixation is less likely to have a positive family history (19). Half of the children with juvenile OS have a positive family history, but only 10% of children with congenital footplate fixa- tion have other family members with CHL, Patients with Xlinked CHL have a high incidence of poststapedectomy. 2494 Section IX: Otology profound SNHL due (0 a perilymph (cerebrospinal uid [CSF] leak) gusher. The incidence of congenital anomalies of the malleus and incus is substantially higher in children ‘with congenital footplate fixation (2596) than in children with juvenile OS (3%). This difference in abnormalities, of the remainder of the ossicular chain probably accounts for the poorer results. Eighty-two percent of the children with juvenile OS had closure ofthe air-bone gap to within 10 dB. This is in contrast to children who had congenital footplate fixation: only 44% had closure within 10 4B, Very young children with OS are also at greater risk posiopera- tively due to their increased incidence of otitis media and eustachian tube dysfunction (27). ‘Age is an important variable for surgical outcome; poorer results in the high-frequency range have been seen in older patients who have undergone stapes surgery However, Meyer and Lambert (30) reviewed the recent literature and reported that primary and revision slape- dectomy is still a reasonable option in elderly patients Lifestyle and occupation are important factors in selecting patients for tapecdectomy, Persons whose activites include repeated exposure to barometric pressure changes (e8, scuba diver) may be at greater risk for postoperative fistula and prosthesis dislocation. Patients whose work or hob. bies dictate excellent balance should be considered ques- tionable candidates for surgery. Amplification instead of surgery is recommended in individuals whom taste is of the utmost importance (eg, chefs, vintners), secondary to the risk of stretching or cutting the chorda tympani nerve with resultant dysgeusia (31). Patients with otologic complaints not attributed to their 0 must be carefully evaluated. For example, patients with Méniére disease and OS have a greater risk of cochlear hearing loss after stapedectomy. Patients with TM per forations and OS should have their perforations success- fully repaired before attempted stapedeciomy (Le, staging of the eat), The incidence of severe to profound SNHL is much greater if stapedectomy is performed in an ear with a perforation, Patients with a history of severe eusta chian tube éysfunction or a history of cholesteatoma are not good candidates for stapedectomy. Those with canal exostoses that are obstructing surgical access should have them removed before the stapedeciomy. Relative contra. indications for stapes surgery include the presence of an extemal or middle ear infection or effusion, suspected endolymphatic hydrops, active OS (positive Schwarize sign, pregnancy; andTIM atelectasis), Patients with external ear infections or otitis media can undergo stapes surgery once the process has resolved, Patients presenting with OS and endolymphatic hydrops can undergo stapes surgery if they have been symptom free from their Méniére disease for atleast 6 months. An absolute contraindication for sta pes surgery is OS in an only hearing ear (27) Stapes surgery should intially commence with the poorer hearing ear in patents with bilateral OS. The likeli, hhood of achieving serviceable hearing in a unilateral MHL is an important consideration, Improved communication, in such a patient may not be achieved after elimination, of the CHL due to the continued SNHL. Some surgeons believe that significant high-frequency SNHL after stapedec tomy in the first ear contraindicates attempting the second, A minimum of approximately 6 months should elapse before attempting surgery on the second ear due to the small, but present risk of delayed postoperative hearing loss (32), PATIENT COUNSELING Observation, fluoride use, and a trial of hearing aid use are discussed with every patient, whether they have CHL or (MIL.The patient must understand the elective nature ofthe procedure The patient must be candidly informed of the risk of stapedeciomy (postoperative deafness of less than 2%). Sueiching or contusion of the chorda tympani nerve is quite common and can produce alteration of taste (or rarely, dryness of the mouth), When these symptoms occu, they are usually sel-Himited and disappear in a few weeks or months. A severely stretched or contused chorda «ym- pani nerve may produce more symptoms than one that is divided, Dehiscence ofthe fallopian canal over the OW can permit exposure or prolapse of the facial nerve. Irthe nerve is waumatized or injured during the stapes procedure, facial nerve palsy can result. Fortunately iatrogenic facial nerve paralysis is quite rae in the setting of stapes surgery (less than 1:1,000) (33). Facial nerve paralysis occurs less than 0.5% of the time and most commonly presents 7 10 10 days after surgery secondary to reactivation of the her- pes simplex virus. Delayed facial paralysis is managed with oral prednisone, antivirals, and appropriate eye care (34) ‘A postoperative TM perforation may occur 2% of the time as a consequence of trauma or vascular injury to the TM flap. Acute balance disturbance is common alter sta pedectomy. It usually resolves in 3 t0 7 days. Long-term balance disturbances or vertigo rarely occur. Sparano et a. (35) reported that 85% of patients with tinnitus improved after stapedectomy (with 52.5% reporting complete res lution, 12.5% reporting no change, and 2.5% reporting, ‘worsening of thei tinnitus) SURGICAL TECHNIQUE A well-performed stapes procedure is gratifying for both surgeon and patient; however, stapes surgery is one of the ‘most technically challenging procedures performed by an otologist. One concern about current resident training is the paucity of stapes surgery being performed. Prior to graduation, the average number of cases a resident per forms as the operating surgeon is three. Vrabec and Coker (36) proposed that the number of surgical OS cases per surgeon could be decreasing due to the use of measles vaccination and because the number of surgeons able 10 perform the surgery has increased significantly over the past 30 years, The pros and cons of local versus general anesthesia must be presented (o the patient, Although general anesthesia is often easier for the patient and sur- geon, local anesthesia may be safer to the patient (sys- temically) and to hearing, preservation. Local anesthesia allows the patient to provide feedback about whether any dizziness is occurring during the procedure, allow. ing the surgeon to terminate the procedure momentarily or permanently. The patient can also let the surgeon know about the status of hearing improvement or loss intraoperatively ‘Two keys to stapes surgeny are adequate exposure and, hemosiasis, Preoperativey, the ear canal andTM are care- fully inspected for evidence of inflammation or infec tion that would dictate postponement of the procedure, ‘The patient can also be consented for use of endaural or postauricular approaches ifthe ear canal is too small for a ‘ranscanal approach, Far canal vibrissae may be trimmed to improved operative exposure. Trauma to the drum or the ear canal from the preparation can occut, making the procedure more difficult, The ear canal is injected with Tocal (196 lidocaine with 1:100,000 epinephrine) for both, anesthetic and vasoconstrctive effect with care being taken to avoid blebbing of the osseous canal skin. The earis then prepped and draped in the usual sterile fashion. The larg est speculum that can be placed is used: in adults, a 7-mm speculum can usually be placed. Some surgeons prefer to usea black speculum as opposed to a silver speculum; the black color absorbs light and is les likely to rellect light from the microscope back to the surgeon. It is often pos- sible to dilate the ear canal during the procedure with progressively larger speculums. A speculum holder is used by some surgeons to reduce the technical demands of stapes surgery. A tympanomeatal flap is elevated from the 6 o’dlock to 12 o'dlock position superior to the level of the lateral process of the malleus. The superior expo: sure is important to allow inspection of the epitympanum and lateral ossicular chain if necessary, The flap is approxi- ‘mately 6 mm wide, as measured from the fibrous annulus, ‘This flap must be sufficient to cover any osseous defect cre- ated by curetting the scutum (Fig. 154.4). ‘The TM is elevated with the fibrous annulus (Fig. 154.5), ‘The chorda tympani nerve is identified and preserved if, possible. Enough scutum (the medial most posterior superior EAC wall) is removed to visualize the OW, pyra ‘midal process, and the tympanic facial nerve (Fig, 154.6), ‘The scutum may be removed either with a sharp cureite or drill. Care is taken to assess whether the facial nerve has an aberrant course or if the nerve is dehiscent or herniat ing too much to allow the procedure to continue. Prior to assessing stapes movement, the lateral ossicular chain is assessed for normal motion; one needs to rule out other ‘causes of preoperative CHIL. The stapes superstructure and fooiplate are palpated. A RW reflex can also be assessed. A stapes mobilization instead of a stapedectomy or stapedotomy can be performed in selected cases. Stapes mobilization may be performed in a select group of Chapter 154: Otosclerosis 2495 Figure 154.4 Typical design fora tympancmeatal lap. patients, in whom a small point of fixation from OS can be seen and where improved stapes mobility can be clearly demonstrated. This technique can also be used in cases of tympanosclerosis, Poe (37) described a minimally invasive ‘mobilization using an endoscope and the argon laser 10 potentially avoid the placement of a prosthesis; he called the procedure a stapedioplasty. Long-term follow-up of patients undergoing stapes mobilization frequently dem. ‘onstrate refixation (38). Anterior crurotomy with partial stapedectomy, as described by Hough in 1960, requires removal of only the anterior footplate and crus (39). This procedure is helpful in a patient with isolated anterior fixation at the fissula ante fenestram, The footplate is fractured in its midportion, and only the anterior half is removed. A Figure 154.5 Technique for tympanomeatal flap elevation. The skin is elevated to the tympanic suleus, and then the annulus is clovated from the suleus 2496 Section IX: Otology Figure 154.6 The tympanomestal flap is naw elevated. The ‘dotted line indicates the area of bone removal for exporure £0 the OW. connective tissue graft is then placed over the exposed area, The IS joint is not disturbed, The stapedial tendon is not divided, which makes this technique of potential benefit to patients working in a noisy environment (39). This technique has been further modified with the intro- duction of the laser that facilitates removal of the anterior crus and footplate (38). Introduction of the laser has significantly reduced the echnical demands of stapes surgery especially in revi- sion cases, Perkins (40) was the first (o use the laser in the treatment of OS in 1980. Since his original description, several types of lasers have been used: argon, potassium— titanyl-phosphate (KTP), erbium-YAG, and carbon diox ide. A laser provides the surgeon with a “no touch” means of transecting the stapes crura and fenestrating the sta- es fooiplate. A CO, laser allows for rapid vaporization. of bone and soft tissue and is the least traumatic to the membranous labyrinth medial to the stapes fooiplate KIP and argon have a more favorable hemostasis pro- file as compared to a CO, laser and up until recently had the advantage of using a handheld probe as opposed (0 a micromanipulator, No difference has been shown between diferent types of lasers in terms of safety and elficacy oF bbe«ween lasers and the use ofa drill or microinstruments Surgeons using the laser should become very familiar with individual laser properties and settings (power, pulse dura tion, pulse interval) so a8 to reduce the risk of thermal inju- fies to the facial nerve and inner ea, ligation, short pulse durations, and clustering laser shots all serve to reduce hheat i the operative site, thus reducing the risk of Unermal injuries A retrospective study of patients undergoing argon laser or carbon dioxide laser stapedotomy (-60 patients in each group) demonstrated no difference in the incidence of postoperative complications, hearing improvement, or speech discrimination (41). The surgeon's operative exper ‘ence and skill are considered the mos: important factors in determining the success and incidence of complications. Surgeon preference often dictates the whether one should perform a total stapedectomy, paral stapedec- tomy, of stapedotomy with the current practice trending toward partial preservation of the footplate. In patients undergoing stapedectomy or stapedotomy, a measuring tool is used to assess the distance between the fooiplate and the distal incus long process. Placing a control hole in the stapes footplate is sometimes performed to reduce the risk of injuring the membranous labyrinth in the event that the fooiplate subluxes from the OW while manip lating the stapes (Fig, 154.7). Dividing the IS joint prior to transecting the stapedial tendon provides additional stability to the stapes thus reducing the chance of floating, or fracturing the footplate A laser or otologic scissors are used to divide the stapedius tendon allowing visualization of the posterior crus of the stapes. Transecting the posterior ‘rus of the stapes with a laser reduces the chance of a float- ing or fractured stapes footplate. The anterior crus of the stapes, in some cases, can also be transected with a laser The stapes superstructure is then down fractured toward A 3 Figure 154.7 A: The tympancmeatal flap is now elevated, and bone has been removed. The stoped footplat tendon is died by a pair of microscssors Centro! holes ate placed in the stapedial the promontory and is extracted (Fig. 154.8). The entire footplate is then removed in a piecemeal fashion using small right angle hooks of varying sizes for a stapedectomy (Fig, 154.9). Care is taken to avoid injuring the inner ear now that the vestibule has been exposed. Perilymph may be suctioned in an area away from the vestibule. However, fone must avoid direct suctioning in the vestibule and a resultant dry vestibule, which has a tisk of postoperative deafness. The OW is sealed with a graft, and the prosthesis is placed (Fig. 154.10). The most common tissue types used for grafting are the dorsal hand vein, tragal perichondrium, orfascia, Schmerber etal (42) recently reported that use of a vein graft (as opposed (o perichondrium) showed better postoperative air-bone gap closure and a lower incidence of postoperative SNHI. Figure 184.9 A: The pos Chapter 154: Otosclerosis 2497 Some cases of OS have formation of exuberant sclerotic bone that fills and obliterates the OW niche. Obliterative 8 is encountered less commonly now than previously. When present in one ear, obliterative OS is present in the contralateral ear in 50% of cases. Obliterative OS requires thinning of the thickened footplate before creation of a fenestration and prosthesis placement. Thinning is usually accomplished with a small electrical or hand drill, which can also be used (o perform the fenestration, The fenestra is usually 0.4 to 0,6 mm in diameter. A prosthesis is then placed through the fenestra into the vestibule. The vesti- bule is sealed by placing soft tissue around the prosthe- sis, However, some surgeons use just a blood seal with the stapedotomy approach. Ayache et al. (43) demonstrated 8 62% success rate (with closure of the air-bone gap to 10 dB or less) in the drll-out procedure, As experience grew with the drill-out procedure for obliterative OS, some otologisis began using the stapedotomy technique in all their patients with OS, The surgical technique involves creating a fenestra in the midportion of the stapes using. a drill, picks, laser, or a combination thereof. The steps of a stapedotomy are identical to stapedectomy up until footplate work is initiated. A rosette pattern is created in the midportion of the footplate with a laser (Fig. 154.11) ‘A Micropick or Skeeter drill is then used to complete the sta- pedotomy (Fig, 154,12), Altera stapedotomy is performed, 4 prosthesis is then positioned over a tissue grafi or the graft is positioned around the stapedotomy (Fig. 154.13). An. autologous blood patch or hyaluronic acid gel can also be used to seal the OW in place ofa tissue graft (43,44). Longstanding results of the stapedotomy procedure hhave been comparable to a total stapedectomy. Some reports suggest a decrease in postoperative cochlear deaf ness and improvement in the air-bone gap closure above 2,000 Iz with stapedotomy. Stapedotomy and partial stapedectomy may show better postoperative hearing at higher frequencies (4,000 Hz) than total stapedectomy; Extraction of posterior ous rior half of the stapedialfootplat 's removed with 2 sharp pick. B:The anterior half of the stapecial footplate i removed by 3 joint knife or Hough hos, 2498 Section IX: Otology Figure 154.10 Prosthossin place. however, (otal stapedectomy may result in improved gains at lower frequencies (250 and 500 Hz) (44). Numerous stapes prostheses have been developed since John Shea popularized the stapedectomy procedure. Early prostheses with a sharp or beveled end were found to cause 4 prohibitive number of postoperative fistulae. The designs that have proved most effective include a connective tis- sue OW graft and piston prosthesis. Tissue can be obtained from tragal perichondrium, dorsal hand vein graft, or temporalis fascia. The graft is combined with a variety of prosthetic designs, such as a wire Teflon piston or a Robinson-type bucket-handle prosthesis, which has a wire loop that is placed over the lenticular process of the incus. If the stapedotomy procedure is performed, the prosthesis is positioned, and connective tissue is placed around the prosthesis base to seal the vestibule or autologous blood is. tused to fill the middle ear space (Fig. 154.9). A recent addi tion to the variety of available stapes prostheses is a heat- activated self-crimping prosthesis, thus eliminating the crimping step necessitated by prior piston type implants, The heat-activated nitinol stapes prostheses also allow for a more uniform crimp around the incus thus reducing the risk of a loose prosthesis (45). rode Angeles \. pia ter on \\ stat Figure 154.11 A rosette patter is created with a lazer in the ‘migportion ofthe footplate. Ths step ean be completed prior t0

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