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Otosclerosis is a disorder of the middle ear that causes progressive deafness.The condition usually develops in both ears. Otosclerosis occurs when overgrowth of bone immobilizes the stapes (the innermost one of the three tiny bones in the middle ear).
To an affected person, sounds are muffled but can be distinguished more easily if there is background noise. Otosclerosis is a hereditary localized disease of the bone derived from the embryonic otic capsule in which lamellar bone is replaced by woven bone of greater thickness and vascularity.
Stapedectomy is a surgical procedure designed to bypass the fixation of the stapes footplate caused by otosclerosis. A small hole (stapedotomy) is then made in the fixed footplate of the stapes using a microdrill, reamer or laser to expose the fluids of the inner ear. Otosclerosis (also known as otospongiosis) is a localized disease of the bone, derived from the otic capsule and characterized by alternating phases of bone resorption and formation. The term “otosclerosis” was introduced by Politzer in 1894 and refers to the final stage of a disease where the bone has become sclerotic and hardened. Otosclerosis was described for the first time by Antonio Maria Valsalva in 1735 as a disease of the human temporal bone.
Most of those previous attempts at stapes mobilization or stapedectomy were unsuccessful, and at the end of the nineteenth century, Siebenmann, together with Moure, “closed the door” on further stapes surgery. Holmgren from Stockholm, Sweden, created a fistula in the horizontal semicircular canal, covering it with mucoperiosteum in 1923, reporting his results in 1937. Histopathologically, the otosclerotic process is characterized by an abnormal bone remodeling, involving the replacement of the otic capsule bone with a hypercellular woven bone, which may undergo further remodeling to finally reach a mosaic sclerotic appearance. Type 1: The appearance of the stapes and annular ligament are strictly normal as seen through a microscope. Type 2: The stapes is fixed anteriorly by an otosclerotic focus in the footplate, involving no more than the anterior quarter of the oval window.
Type 3: The stapes is fixed because of the anterior focus that extends posteriorly, comprising half of the footplate and the oval window niche.
Type 4: The entire footplate is affected but the interface with the oval window niche is visible, even though the niche itself is caught up in the otosclerotic process. Weber and Rinne tuning fork tests are performed at 512 and 1,024 Hz and compared to the audiogram.
Advancing stapedial fixation affects bilateral acoustic reflexes, even in unilateral involvement. A tympanogram in early- and mid-stage otosclerosis shows the presence of a normal, type A tympanogram. Acoustic immittance studies provide no frequencyspecific information about the hearing status, and they do not specify the severity of the impairment. Another typical finding in otosclerosis is the Carhart’s notch, which means a depression in bone conduction at 2,000 Hz. With the development of cochlear otosclerosis, air conduction thresholds continue to worsen and the loss becomes mixed or sensorineural in cases of pure cochlear otosclerosis. Patients with a conductive hearing loss may describe improved speech understanding in noisy situations, unlike those with a sensorineural hearing loss, who understand worse in noisy environments. High-frequency sensitivity critically influences word identification scores; in a mixed or primarily sensorineural hearing loss resulting from cochlear otosclerosis, reductions in word identification are related to the degree of sensorineural involvement. As otoacoustic emissions are generated in the cochlea and must propagate out through the middle ear to be recorded by a probe, it is predictable that they would be reduced in cases of abnormal middle ear function. Symptoms of dizziness and imbalance are reported less frequently by patients with otosclerosis. In addition to audiologic tests, a CT evaluation can be performed to assess middle and inner ear structures. A wide cochlear aqueduct was believed to play a role in the incidence of the perilymphatic (stapes) gusher, but this concept has fallen into disrepute in recent years.
Otosclerotic fenestral foci are usually best diagnosed on axial CT sections owing to the anteroposterior orientation of the ovalwindowand stapes crura. The correct diagnosis of otosclerosis is based upon the histologic analysis of the ankylotic stapes footplates removed during stapedectomy. It is considered an inflammatory disease of the human otic capsule caused by persistent measles virus infection. Speculations in the literature point to stresses and strains in the temporal bone, consequences of an upright posture, as the cause of otosclerosis. The first explanation of a sensorineural hearing loss in otosclerosis came from Politzer, who proposed that the sensorineural hearing loss would derive from a bony invasion of the scala tympani of the cochlea. Surgery is considered for patients with a conductive hearing loss of at least 15 dB in frequencies of 250– 1,000 Hz or higher. An operation should not be performed in an inflamed ear, whether in the middle ear or the external ear canal. A stapes operation in an only hearing ear is indicated just for those patients with a profound mixed hearing loss who cannot be helped by a conventional hearing aid.
Before surgery, patients should be told about the risks of the operation, which include failure to improve hearing with residual conductive hearing loss, the possibility of a sensorineural hearing loss, deafness, vestibular dysfunction, perforation of the tympanic membrane, and disturbance in taste. A posterior tympanomeatal flap is raised and the bone is curetted from the posterosuperior portion of the tympanic annulus until the pyramidal process and tympanic segment of the facial nerve are easily visualized. After opening the footplate, a small air-liquid interface is created, and the concavity of the perilymph offers a barrier to penetration by foreign particles, such as pieces of footplate, mucoperiosteum, or bone dust. The risk of elevation of the endosteum of the vestibule during removal of the entire footplate is eliminated in stapedotomy. In large fenestra stapedectomy, there is an unavoidable risk of damage to the utricle when superior fibrous adhesions are present in the vestibule.
In large fenestra stapedectomy, there is a risk of migration of the wire prosthesis toward the border of the oval window, in contrast to stapedotomy where this cannot occur. In large fenestra stapedectomy, lateral displacement of the oval windowmembrane may occur because of scala retraction, increased perilymphatic pressure, or by strong movements of the incus.
The defect in the epithelial lining in stapedotomy is closed by an ingrowth from its edges underneath the tip of the piston.
The advantages of performing a stapedotomy with a laser (argon, KTP, CO2) (Pictures 1, 2, 3 and 4) are: the hemostatic properties of the laser, “no touch” surgery that reduces the chance of a floating footplate, the ability to create a precise fenestra without entering the inner ear, thus minimizing the risk of acoustic trauma, and the possibility to fenestrate a floating footplate without the risk of depressing it into the vestibule. Picture 3: A prosthesis is inserted into the stapedotomy and fixed on the long process of the incus.
Before stapedotomy, the length of the prosthesis is determined and the piston is trimmed to the right dimension. Some authors have also described a technique to preserve portions of the stapes and the stapes tendon during stapedotomy surgery. Complications and failures in stapedectomy or stapedotomy can happen during an operation, immediately after the operation, in the intermediate period, and in the late period. During the operation, an eardrum perforation may occur while the tympanic membrane is elevated, especially in atrophic eardrums. The chorda tympani nerve should be preserved whenever possible, but sometimes the chordal eminence is very prominent and hampers visibility of the footplate.
Incus bone luxation and subluxation can occur while removing the bony annulus with a curette, in cases of partial or complete ossification of the incudostapedial joint, after removal of the stapedial crura and when fixing the prosthesis to the long process of the incus.
A floating footplate is a serious complication and energetic attempts to remove it may result in a sensorineural hearing loss.
An immediate postoperative sensorineural hearing loss can occur from a hydraulic effect following the removal of the mobilized footplate, rapid loss of perilymph, acoustic trauma from instrumentation, and the presence of blood in the inner ear vestibule with a consequent ionic disturbance. A perilymphatic fistula means the leakage of perilymph from the vestibule into the middle ear.
Conductive hearing loss after stapedectomy is caused by displacement of the prosthesis, an excessively short or long prosthesis, resorption of the incus, and adhesions around the prosthesis (Tos 2000c).
Some 50 years ago, it was suggested that sodium fluoride might have a beneficial effect on the progression of otosclerosis.
Only one double-blind, placebo-controlled trial of sodium fluoride use in otosclerotic patients has been made, and this showed a reduced incidence of deterioration in hearing after 2 years in the treatment group.
No trial has set out to determine how long treatment with sodium fluoride should continue (Cruise et al.
Arnold A, Blaser B, H€ausler R (2007) Audiological long-term results following stapedotomy with stapedial tendon preservation, vol 65, AdvOtorhinolaryngol. Bast F, Schrom T (2009) Das soft-clip Piston Titanium als alloplastische Prothese bei der operativen Versorgung der Otosklerose: erste Ergebnisse. Filipo R, Attanasio G, Barbaro M, Viccaro M, Musacchio A, Cappelli G, De Seta E (2007) Distortion product otoacoustic emissions in otosclerosis: intraoperative findings.
Sziklai I, Batta TJ, Karosi T (2009) Otosclerosis: an organspecific inflammatory disease with sensorineural hearing loss.

Hearing loss progresses slowly over 10 to 15 years and is often accompanied by tinnitus (ringing in the ears) and, more rarely, vertigo (a spinning sensation). It can be cured by stapedectomy, a surgical procedure in which the stapes is replaced by a tiny piston, which moves through a hole created in the inner ear.
The position of the focus of new bone formation determines its effect on the function of the ear. The tympanic membrane is temporarily elevated for access to the middle ear and, under microscopic control, the incudostapedial joint is disarticulated using microinstruments.
The term “otospongiosis” is more accurate from a pathological point of view and pertains to the active and vascular stages of the disease. In 10% of those patients, the focus is localized near the oval window niche leading to a fixation of the stapes with a consecutive conductive or mixed hearing loss. Nylen, his associate, developed a binocular microscope and was the first to use it for that operation.
The results were immediate and an air-bone gap could be closed completely, which never occurred in fenestration surgery. The site of predilection is the fissula ante fenestram, which lies anterior to the stapes footplate. In Type 2-A, the entire annular ligament is also stiff and the stapes is fixed around the entire circumference. The focus around the oval window has completely obliterated the niche, which is only represented by a slight depression between the superior and inferior foci. Some 40% of patients with normal ears may also show a brief compliance increase at the onset of the response. The ipsilateral reflex with the probe and activator sound in the same ear is the first to show reductions in amplitude, followed by elevated thresholds. A type As tympanogram, in the presence of a normal appearing tympanic membrane, should raise suspicion of a fixation at the malleus rather than a stapes. This is thought to be an audiologic artifact, possibly from the effect of stapes fixation upon middle ear resonance. Poor preoperative word identification scores contribute to a poor prognosis for hearing improvement following surgery (Hannley 1993). DPOAEs are not measurable preoperatively, and even postoperatively they register no significant increase. Congenital ossicular deformity can be excluded and a retrofenestral disease should not be overlooked. Deformity of the internal auditory canal fundus is much more commonly associated with this aberration. These foci are of variable size and density and are most often seen in the anterior oval window region. Otosclerosis is an active bone process that occurs within the endochondral layer of the temporal bone that, under normal conditions of development, undergoes virtually no remodeling.
Estrogens are also well-known stimulators of osteocytic activity and may play a role during the ossification of otosclerotic foci.
The second theory suggested circulatory changes in the cochlea as the outcome of abnormal bony foci. Researchers have found viral-like particles within osteoblasts in active otosclerotic lesions, measles antigens and measles virus products within active lesions. Surgery is also contraindicated for patients with tympanic membrane perforation, as well as for those with Meniere’s disease, and it is questionable for patients with a contralateral ear problem which may decrease hearing over time.
If the operation is not successful, the patient is a candidate for a cochlear implantation.
As it occurs in most cases, it should be explained before surgery that there will be some disturbance in taste because of manipulations of the chorda tympani.
In stapedectomy, the concavity is larger and the small pieces will lie deeper toward the utricle.
In stapedotomy, the piston can be pulled out from the stapedotomy opening without damage to the inner ear. In cases of obliterative otosclerosis, where the oval window is filled with massive otosclerotic foci, a stapedotomy should be performed with a drill (Gherini et al.
Although some findings indicate better hearing in noise and less intolerance to noise, there was no statistical significance between the groups with and without stapedius tendon preservation (Arnold et al. It reduces enzyme levels in the perilymph of patients with otosclerosis and inhibits trypsin. The wide diversity in treatment doses offers no evidence regarding the optimum duration of treatment. Larger doses of sodium fluoride (25–60 mg) may slow resorption and occasionally cause gastrointestinal upset, sore tongue, hypersalivation, vomiting, diarrhea, and abdominal pain. In: Arnold W, Hausler R (eds) Otosclerosis and stapes surgery, vol 65, Adv Otorhinolaryngol. Am J Otol 4(2):112–117 Gherini S, Horn KL, Causse JB, McArthur GR (1993) Fiberoptic argon laser stapedotomy: is it safe? Otosclerosis frequently runs in families, and symptoms usually start to appear in early adulthood. A degree of sensorineural deafness may develop, making high tones difficult to hear and causing the sufferer to speak loudly. When new bone develops around the footplate of the stapes it may fix the footplate to the margin of the fenestra vestibuli and prevent it from moving. The limbs of the stapes and stapedius are then both divided, usually with microscissors or a laser, and the superstructure of the stapes removed.
A small graft of connective tissue derived from local fascia or perichondrium is used to seal the hole with a flexible membrane. The disease is bilateral in 70–80% and usually symmetrical in extension and distribution of the otosclerotic foci. In the middle of the nineteenth century, Kessel treated a patient with otosclerosis who had fallen off a wagon and was then able to hear. After removing the stapes, he covered the oval window with a vein and attached an artificial nylon stapes prosthesis from the incus to the oval window vein graft. Otosclerotic foci may also appear in other sites, even in the region of the round window and cochlea (Linthicum 1993).
In the early stages of the disease, acoustic reflex abnormalities may be present only when the probe is placed in the affected ear. As the degree of hearing loss increases, contralateral reflexes become abnormal, yielding the “inverted–L pattern” in which only the ipsilateral reflex in the unaffected ear (in the case of unilateral disease) remains observable.
As the disease progresses and the otosclerotic focus involves more extensive areas of the stapes footplate, the height of the tympanic peak decreases. Early in the development of otosclerosis, patients may have a low-frequency conductive loss that narrows in high frequencies.
Faced with speaking in a noisy environment, most speakers raise the level of their voices to be heard above the din by themselves and others. It remains unclear why DPOAEs are not detected, despite a subjective hearing improvement and a sufficiently closed air-bone gap, at least in middle and low frequencies.
Obliterative otosclerosis can be diagnosed, and also obliterative round window involvement can be distinguished on the CT scan.
The majority of epidemiological studies of families with stapes fixation has shown an autosomal dominant mode of inheritance with incomplete penetrance of approximately 40–45%. The hypothesis that otosclerosis is a generalized disease brought about by abnormal parathyroid function could not be proved.
The third theory held toxic metabolites released from the otosclerotic focus responsible for cochlear damage (Menger and Tange 2003). Otosclerosis appears only in humans and the measles virus also attacks only humans and closely related primates. It should also be explained to the patient that the possibility exists for hearing aid amplification, but that the quality of sound is usually not comparable to natural hearing.
The results do not differ whether the patient was operated on under local or general anesthesia. They are made from stainless steel, fluoroplastics, a combination of wire and fluoroplastic and titanium. The defect should be closed immediately by the adaptation of the perforation edges or by placing a fascia graft. The best solution is to make a fenestra with the laser; the footplate can be removed by drilling a groove at the inferior border of the vestibule and placing a hook under the floating footplate. In an audiogram, the patient will have a sensorineural hearing loss, especially at low frequencies.
It could also increase mineralization and stabilization of otospongiotic lesions and halt the otosclerotic process.

This impedes the passage of vibrations of the tympanic membrane passing through the ossicular chain to the inner ear, producing a conductive hearing loss.
A small piston, usually made of Teflon or titanium incorporating a wire made of stainless steel, platinum or titanium, is crimped onto the long process of the incus and placed in the perforation in the stapes footplate.
Clinical otosclerosis should be distinguished from histological otosclerosis, where changes are present without clinical signs. During the autopsy after the patient’s death, Kessel discovered a fracture through the horizontal semicircular canal. The first stage was mastoidectomy, the second was the creation of an external ear canal skin flap, and the third the creation of a fistula in the horizontal canal, covered with the canal skin. Shea originally recommended covering the oval window with a vein graft; others have used fascia, perichondrium, and Gelfoam.
Also, a new piston design was developed which enables easier crimping of the piston on the long process of the incus.
If the otosclerotic process begins to develop anterior to the footplate, the footplate becomes displaced posteriorly, resulting in a low frequency conductive hearing loss.
The probability of observing a contralateral reflex is reduced up to 50% with an air-bone gap averaging 27 dB between 500 and 4,000 Hz; when reflexes are measured ipsilaterally, acoustic reflex abnormalities may be present when the air-bone gap is only 10 dB. This is due to the posterior displacement of the footplate as the result of an anterior otosclerotic lesion.
It may interfere with their ability to respond reliably to pure tone testing in frequency ranges that correspond to those of the tinnitus.
The listener’s conductive hearing loss attenuates the background noise, leaving the speaker’s voice more audible through an apparent improvement in the signal-to-noise ratio.
The results of our study show that DPOAEs cannot replace behavioral threshold tests; they may only be included in a battery of tests for a complete clinical follow-up to efficiently monitor postsurgical stapes (Filipo et al. In the inner ear membranous labyrinthine, ossification in the basilar cochlear turn may occur, which is an important finding if a cochlear implantation is contemplated. The inner ear may play a direct role in the inhibition of otic capsule remodeling by producing substances that diffuse into the surrounding bone and prevent remodeling. Genetic linkage studies have demonstrated the presence of eight loci (OTSC1, OTSC2, OTSC3, OTSC4, OTSC5, OTSC6, OTSC7, OTSC8) which are located on chromosomes 15q, 7q, 6p, 16q, 3q, 6q, and 9q. Histologic characteristics describe multinucleated osteoclasts, osteocyte-like cells, and proliferating endothelial cells in the pseudovascular spaces of an active otosclerosis. Vaccination against measles should contribute to a decline in the incidence of otosclerosis as well. This technique showed better results when compared with mobilization techniques and soon began to be widely used. It should be explained to the patient that a hearing aid may still be needed after surgery, but ear performance with amplification would be improved.
The external ear canal is also injected in four quadrants with 1% xylocaine with 1: 100,000 epinephrine (Mc Kenna and de Venecia 2006). Removal of the submerged footplate could also be attempted by introducing a blood coagulum and aspirating the blood clot together with the submerged footplate. Symptoms of reparative granuloma include hearing loss, distortion of sounds, tinnitus, and vertigo a few days to 2 weeks after the operation. This is much less common in stapedotomy than in stapedectomy, even without the sealing of the fenestra. Some authors suggested that sodium fluoride treatment would be more effective if the initial sensorineural hearing loss was less than 50 dB. Complete deafness does not result, because vibrations can still pass directly to the cochlea via the bones of the skull, albeit in a markedly less efficient manner.
After having visited Sourdille and Holmgren, Julius Lempert cultivated his own one-stage fenestration operation and was able to achieve an improvement of hearing in most patients. The polyethylene strut introduced by Shea was abandoned after slippage into the vestibule or erosion of the incus occurred, and it was replaced in 1960 by a wire prosthesis introduced by Schuknecht and Oleksiuk.
If the whole footplate is fixated, a conductive hearing loss takes place in all frequencies.
The jammed footplate first maintains the capacity for transmission in the higher frequencies. The use of pulsed or warbled tones may help the patient to separate tinnitus from test stimuli in these cases. The facial nerve should be evaluated for dehiscence or protrusion in the tympanic segment as this may disturb surgery in the oval window area. Patients with tympanosclerosis almost invariably have an obvious middle ear disease that includes an underpneumatized mastoid, making the differentiation from otosclerosis relatively easy (Swartz and Mukerji 2008). The spiral ligament produces a compound called osteoprotegerin (OPG), which is secreted to the perilymph and diffuses into the surrounding otic capsule bone. In the peripheral zone of active foci, CD8 positive, cytotoxic T lymphocytes and activated osteoclasts are found. Later, a stapedotomy technique was developed where a small fenestra was created in the footplate. If both sides are affected by otosclerosis, the poorer hearing ear should be operated on first. Soft-clip pistons enable easier fixation of the piston on the long process of the incus (Bast and Schrom 2009). The granuloma should be removed from the oval window, a different graft material applied, and also a different prosthesis from that of the initial operation. A fistula test with the pneumatic otoscope is positive in two thirds of the cases, and a fistula test with electronystagmography and an impedance bridge are the procedures of choice. The connection between the tympanic membrane and the inner ear is thus reconstituted and hearing restored. He performed mastoidectomy through an endaural incision, created a fenestra over the ampullated end of the horizontal canal and covered it with the previously prepared skin flap from the posterior portion of the ear canal. Rarely, the otosclerotic process may overgrow the footplate and cause obliterative otosclerosis; this occurs in about 2% of the cases.
Patients may be unaware at first of the low-frequency conductive hearing loss until it exceeds 25 dB, but may complain that speech sounds “hollow.” The loss may be limited to frequencies below 1,000 Hz.
Also a meticulous masking technique is necessary to avoid errors in overestimating and underestimating cochlear reserve during surgical planning. Diseases like tympanosclerosis that can give rise to ossicular chain fixation should be ruled out. OPG inhibits the recruitment, formation, and activity of osteoclasts, which resorb bone (Stankovic and Mc Kenna 2006).
Now broadly employed, the small fenestra technique provides a good closure of the air-bone gap with minimal risk of perilymphatic fistula or inner ear trauma and the resultant dizziness and hearing loss.
After a successful operation on the first ear, an operation on the other may follow after a period of about 1 year.
Tympanotomy, with sealing of the fistula, should be done if a perilymphatic fistula is suspected. Sodium fluoride was also administered to otosclerotic patients with vestibular symptoms, effecting complete or partial relief from those symptoms.
Otosclerotic lesions may also involve the cochlear capsule and penetrate the cochlear endosteum with progressive sensorineural hearing loss (SNHL). This characteristic rising audiogram configuration has been referred to as a “stiffness tilt.” When the footplate becomes completely fixed, a mass effect is added to the audiogram. These molecules could flow into the perilymph and interfere with the electromotility of outer hair cells, resulting in a sensorineural hearing loss (Sziklai et al. Both techniques had very good hearing results; stapedotomy should have better results at 4,000 Hz (Fisch 1982). In 1942 Meniere first reported stapes mobilization to improve hearing, and toward the end of the nineteenth century, stapes surgery in Europe was performed by Kessel, Boucheron, Miot, Moure, Passow, and Faraci. It was found that the spiral ligament is hyalinized and the circulation of potassium ions within the cochlea is disturbed. This results in a stabilization of low-frequency thresholds with a progression of loss in high frequencies and a gradual widening of the air-bone gap. Otosclerosis is the most predominant cause of hearing loss in Europe and the USA, whereas it appears to be uncommon in developing countries and among the Japanese population. In rare cases, otosclerosis may also involve the endolymphatic sac causing symptoms like those of Meniere’s disease. In the absence of cochlear involvement, the pure conductive hearing loss produced by complete stapes fixation is limited between 60 and 65 dB with a maximum airbone gap across the frequency range.

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