Α Β Γ Δ Ε Ζ Η Θ Ι Κ Λ Μ Ν Ξ Ο Π Ρ Σ Τ Υ Φ Χ Ψ Ω  

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

ιστορία της ωτοσκλήρυνσης

     istori΄a tis otoskli΄rinsis    
history of otosclerosis

     χίστορι οφ οτοσκλερόσις    

Ερμηνεία:

Τhe pioneering  minds in the History of Otosclerosis

Βy Dr Dimitrios N. Gelis, MD, ORL, DDS, PhD, Corinthian Institute for Otosclerosis research, Corinth, Greece, Dr George P. Kotsis, MD, ORL, PhD,  Stavros G. Georgopoulos, MD, ORL, Research group for Otosclerosis of ‘‘ELPIS’’ General Hospital and Oncological ''Agios Savvas" Hospital, Athens, Greece

 Introduction

Dr Dimitrios N. Gelis
Dr George P. Kotsis
Dr Stavros Georgopoulos

Otosclerosis is a bone remodelling disorder of the osseous otic capsule of unknown etiology inducing progressive conductive and/or sensorineural hearing loss (HL) which affects exclusively the human temporal bone. Otosclerosis may induce stapes ankylosis. Generally, stapes ankylosis is a heterogenous disease causing conductive hearing loss with different causes. Nonotosclerotic stapes fixations may belong to degenerative disorders with variable histopathology. Various complex etiopathogenetic factors may contribute to the genesis of otosclerosis which have been based on several proposed etiopathogenetic hypotheses.

The major hypotheses considered are genetic factors, immunologic factors and viral infection. Since the familial incidence of otosclerosis is known a recent genetic analysis has given evidence of three otosclerosis genes (OTSC1-3). Mutations in the collagen gene COL1A1 have been found in one large family with several cases of otosclerosis. Concerning an immunologic etiopathogenetic process, the presence of serum antibodies against collagen II and IX in patients with otosclerosis confirms the hypothesis of a collagen autoimmune mechanism.

Niedermeyer and Arnold, (2002) characterized otosclerosis as a chronic inflammatory disease and after morphologic and biochemical investigations have revealed a measles virus association. Persistent measles virus (MeV) infection and chronic viral antigen expression on the surface of infected cells can induce a secondary autoimmune reaction against the otic capsule (Karosi et al, 2004, 2005).

This pathological process is supposed to be one of the etiologic factors in otosclerosis according to morphologic and biochemical investigations. The presence of measles virus was shown in otosclerotic patients by reverse-transcriptase polymerase chain reaction amplification of the viral RNA and detecting the viral proteins by immunohistochemistry. According to Karosi et al, (2005) the persisting measles virus infection of the otic capsule and the presence of CD51/61 positive osteoclasts in otosclerotic bone containing viral sequences provides the basis for an inflammatory bone remodelling disorder. The persistent measles virus infection and reactivation of resting embryonic osteoclasts in the otic capsule induces otosclerosis, (Karosi et al, 2006).

Combination of decreased antimeasles IgG serum level and conductive HL has a great specificity and sensitivity as a diagnostic method in the preoperative evaluation of ossicular chain fixations otosclerosis. Low antimeasles IgG level indicates otosclerosis, whereas high level suggests non-otosclerotic ossicular chain fixations. Preoperative elucidation of the cause of a conductive hearing loss (HL) may suggest optional medical treatment in preference to surgical methods (Karosi et al, 2006).

Gantumur et al (2006) reported the first determination of MeV sequences in total RNA isolated from primary cells cultured from otosclerotic tissue. Persisting MeV in primary footplate cells could not be recovered by coculturing with B95a cells.Persistent measles virus infection of the temporal bone with increased tumor necrosis factor (TNF)-alpha and decreased osteoprotegerin mRNA expression is supposed to be the main etiologic factor in otosclerosis.

Detection of TNF-alpha mRNA demonstrates activated osteoclast functions and inflammatory pathways in otosclerotic stapes footplates associated with measles virus presence. Increased expression of TNF-alpha and its action on RANK production inhibits the protective functions of osteoprotegerin on normal bone turnover in the otic capsule, (Karosi T, Jokay I,et al, 2006).

Historical events connected with research on otosclerosis

The history of otosclerosis started with the description of stapes ankylosis by Antonio Valsalva at 1741. The terminology that was used in order to describe the clinical results of stapes ankylosis was not the proper one and that was a reason of misunderstanding among the otologists of the past two centuries.

In 1968 as ‘‘otosclerosis” was characterized a decalcifying disease of the osseous labyrinthine capsule or a localized osteoporosis of the osseous labyrinthine capsule (Shambaugh and Petrovic, 1968). In 1980 otosclerosis was described as a primary focal spongifying disease of the osseous labyrinthine capsule (Shambaugh and Glasscock, 1980). In 2003 as otosclerosis has been characterized a localized bone dystrophy with increased bone turnover (Grayeli A, et al, 2003).

Very soon it was understood that otosclerosis is a disease of hereditary liability. Otosclerosis has been considered as a genetically-mediated via autosomal dominant transmission with incomplete penetrance (approximately 40%) and variable expressivity.   The genetic penetrance is determined by multifactorial influences (Yi Z, et al, 2000).

Histologically otosclerosis has been described as a primary disease of the ossicles and otic capsule, in which endochondral bone is replaced with periosteal bone over a period of several years. This process results in a more porous and less dense bone, which has led many to prefer the term "otospongiosis".

The most common site involved, or "site of predilection" is just anterior to the oval window niche. The focus may gradually invade the annular ligament and stapes causing conductive hearing loss due to stapes ankylosis or fixation. A sensorineural component of hearing loss is not uncommon, and is thought to be due to ototoxic enzymes released into the cochlea.

The disease is usually subclinical, with only 10% of patients with microscopic evidence of the disease ever developing symptoms. There is a significant sensorineural component in patients with otosclerosis planned for stapedotomy, which is worse than age-related hearing loss by itself.

Deterioration rates of AC and BC thresholds have been reported, which can be helpful in clinical practice and might also guide the characterization of allegedly different phenotypes for familial and sporadic otosclerosis, (Topsakal et al, 2006).

Otosclerosis occurs only in the osseous labyrinth capsule that surrounds the inner ear and, as far as is known, only in man. The otic capsule is the only bone to remain unchanged after birth but in otosclerosis, patches consisting of many vascular channels appear. Histologically, this appears as an otospongiosis and in radiographs the otic capsule becomes demineralised.

After many years the combination of the demineralization and fluid spaces of the inner ear give a double-barrelled appearance. There may be new bone accumulation within the osseous cochlea spiral but this is not necessary for sensory hearing loss. The SNHL is the result of “enzymes”, circulatory changes, or toxins. If SNHL is apparent by early adolescence there is greater chance the threshold shifts will deteriorate to profound deafness.

Fixation of the footplate can occur from accumulation of the new bone and at its worst the oval window depression can be obliterated with new bone. New bone may have to be removed to provide access for a cochlear implant. As seen at autopsy by sectioning the temporal bones, the lesion consists of one or several foci of new bone occurring in and replacing the endochondral bone of the labyrinthine capsule.

It is unique in that it remains unchanged from the fifth fetal month throughout life asa primitive type of bone derived from cartilage, ivory hard, nearly avascular, and almost devoid of the remodeling activitythat characterizes the rest of the skeleton. The so-called otosclerotic lesion begins in the inert solid endochondral bone as a focus of new, vascular bone, poor in calcium and rich in osteoblasts and osteoclasts, indicating its vigorous remodeling activity.

Later on some of these foci recalcify and become less vascular with slowed remodeling activity. These are regarded as mature inactive lesions. There is great variation in the extent of the otosclerotic involvement of the labyrinthine capsule, as well as in the degree of remodeling activity, with the majority of foci found at autopsy quite small. While active and inactive foci are found at all ages, there is a definite tendency for very vascular actively enlarging foci to be more frequent in young individuals, while mature inactive foci are somewhat more frequent in older individuals.

Τhe disease process called otosclerosis in its actively enlarging state is the exact opposite: it is in reality a localized osteoporosis of the labyrinthine capsule. Only when it has matured does its density approach that of the normal labyrinthine capsule. The name otosclerosis obviously inappropriate and misleading was applied to ankylosis of the stapes at a time when the fixation was believed to be secondary to sclerotic changes in the mucous membrane of the tympanic cavity. The first description of ankylosis of the stapes is attributed to Antonio Valsalva, who, in 1741, carried out a post mortem examination on the body of a patient who was believed to be deaf, (Nager GT. 1969), 

 Fig. 1. Antonio Mario Valsalva (1666-1723). He wrote De Aure Humana describing anatomy as seen in over 1000 temporal-bone dissections.

He distinguished areas of ear as outer, middle, and inner and named scala vestibule and scala tympani.

He described muscles of pinna, auditory nerve, and ankylosis of stapes.

He first rationalized treatment in otology and put manoeuvre bearing his name on rational basis, first advocating it for expelling pus in otitis, later for replenishing air in the middle ear when Eustachian tube was obstructed.

He noted relationship of preauricular lymph node to surrounding tissues and its significance. He clearly described incisura of external ear.

 

 

 

Other pathologists of the eighteenth century also described ankylosing of the stapes noticed at autopsy on deaf subjects, the most noteworthy report being that of Toynbee in 1841 (Toynbee 1841), who concluded, on the basis of 1659 ear dissections, that  ‘‘osseous ankylosis of the stapes to the fenestra ovalis was one of the common causes of deafness’’. He noted that stapes ankylosis occurred in 39 dissections.

 In 1873- Schwartze- described the reddish discoloration of the tympanic membrane, later found to be increased vascularity of the cochlear promontary during the active stage of otosclerosis.

The term ‘‘sclerosis’’ was first applied to stapes ankylosis or fixation by von Tröltsch in 1881 (Anton Friedrich Freiherr von Troeltsch, 1829–1890,credited with devising first modern otoscope). This name was given, in order to differentiate bony stapes ankylosis from ‘‘common middle ear catarrh’’, as there was the belief that sclerosing changes in the tympanic mucosa were the cause of the stapes fixation. The general interpretation of sclerosis as a disease of the middle ear mucosa leading to progressive stiffening of the windows persisted until the report by Politzer in 1894.

In 1890- Katz- first found microscopic evidence of otosclerosis resulting in stapes fixation.

Soon after Adam Politzer at 1893 demonstrated, by sectioning the temporal bones of sixteen patients

 

 Fig. 2. Joseph Toynbee of London (1815-1866). Toynbee was the first who systematically dissected temporal bones (2000 bones) and correlated pathology with clinical history. He disproved belief that stricture of Eustachian tube was common affliction, noting that tube was normally closed and opened only during yawning and swallowing. He described ten osteomas, numerous cholosteatomas, which he called molluscous tumors, and fistula of lateral canal. He is the first who described 160 cases of otosclerosis.  He invented successful artificial drumhead made of disc of gutta-percha mounted on silver wire. He died after the inhalation of chloroform and performance of Valsalva manoeuvre experimenting while trying to treat experimentally his own tinnitus.

 with stapes fixation that he had examined during life, that the fixation was due to a primary bone disease of the labyrinthine capsule (Politzer 1893). The general interpretation of sclerosis as a disease of the middle ear mucosa leading to progressive stiffening of the windows persisted until the report by Politzer in 1894. Politzer conducted his preliminary research from 1862 to 1893.

From the concept of a dry catarrh of the middle ear, the fixation of the stapes became progressively associated with a specific ossification in and around the footplate. Politzer presented his first results in 1893. He completed his research by concluding in 1901 that otosclerosis had become an independent disease and should have the right to its own chapter in otologic books. He selected the word otosclerosis to describe this new pathologic entity, (Mudry, 2006). After that the much more suitable name of   ‘‘otospongiosus progressiva’’ was suggested.

Due to the tenacity of tradition the misnomer otosclerosis has persisted and continues to be in general use for this localized osteoporosis of the labyrinthine capsule (Shambaugh 1967).

Bezold had observed the immobility of the stapes macroscopically and manometrically in patient in whom, during life, the characteristic tuning fork findings had been a negative Rinne test, elevation of the lower tone limit, and prolonged bone conduction, along with normal eardrum and tube. These findings were afterward known as the ‘‘Bezold triad’’ (Bezold and Siebenmann, 1894). Ιn 1908- Bezold - described the typical history and physical findings, as well as audiometric findings of otosclerosis.  

Fig.3. Friedrich Bezold.  German otologist, born February 9, 1842, Rothenburg, Mittelfranken; died October 5, 1908.

He clarified the diferentiation by tuning fork tests of conductive and sensorineural hearing losses and the clinical diagnosis of otospongiosis (otosclerosis).

His clear and concise Textbook of Otology served as a model for this Surgery of the ear.

  In 1911 Friedrich Siebenmann (Swiss otorhinolaryngologist, 1852-1928), proposed that the correct term of otospongiosis should replace the misnomer otosclerosis, since the diseased bone is more porous and less dense than the normal capsule that it replaces,

 Τhe term otosclerosis it is continuously used  until now for the description  of the fixation of the stapes footplate by bony deposits leading to progressive ankylosis of the footplate in the oval window niche and a progressive conductive deafness. Otosclerosis means the final inactive stage of the process, whilst the essential pathological lesion is in fact a replacement of lamellar bone by bone of greater thickness, cellularity and vascularity.

The term otospongiosis more accurate and refers to the active vascular stage of the process. It indicates that an active lesion may be present and there is something more than a simple fixation of the stapes by inactive sclerotic bone.  The false belief that the process is inactive or soon becomes inactive delayed the research of the sensorineural hearing loss which is so often an integral part of the disease.

Forty years after the introduction and acceptance of the medical treatment with sodium fluoride plus calcium carbonate (Florigkel), which promotes the recalcification and inactivation of an otospngiotic focus it is obligatory to be used the term otospongiosis. Otospongiosis is classified pathologicaly as mature and immature.

Mature otospongiosis means that there is inactive otospongiotic focus as seen through the operation microscope. This focus contrasts with the slightly yellowish normal capsule by its whiter, chalk-like color, as well as by the distortion of the normal anatomy of the oval window niche.

Immature otospongiosis means the active otospongiotic focus which is coverd by the thickened vascular mucosa, giving a pinkish hue to the promontory (Schwartze’s sign) when viewed through a normally translucent tympanic membrane.

Εικ 4. Ηermann Hugo Rudolf Schwartze. German physician, born September 7, 1837, Neuhof bei Penlum (Pommern); died August 20, 1910.

He established indications and method of simple mastoidectomy. He put paracentesis on a rational basis.

In 1873- Schwartze- described the reddish discoloration of the tympanic membrane, later found to be increased vascularity of the cochlear promontary during the active stage of otosclerosis.

This later became known as Schwartze's sign, and occurs in 10% of otosclerosis ears.

The simultaneous occurrence of otosclerosis and otospongiosis in the same lesion is well documented. The logical sequence of events would seem to be softening and destruction of the bone by the otospongiotic lesion, which is subsequently converted to otosclerosis. Computed tomography demonstrated that cochlear otosclerosis can exist without the oval window lesion. Schwartze sign can be used as a sign of the otosclerotic invasion to the promontory. The reddening of the Schwartze sign is likely due to increased blood flow (Nakashima et al, 2006).

The frequency of the otosclerotic bone lesion has been demonstrated by routine sectioning of the temporal bones at autopsy. Thus in 1944 Guild, in 1,161 routine autopsies found a focus of otosclerosis under the age of 5 in less than
0.6% but an increasing incidence with age, to between the ages of 30 and 50, when 9.7% of white males and 18.5% of white females exhibited the disease. In Negroes, otosclerotic lesions of the labyrinthine capsule were comparatively rare, occurring in around 1%. Ο Guild observed that in the great majority of ears the otosclerotic pathologic process remains quite small, without producing any sort of clinical symptoms.

 

 

Fig. 5. Adam Politzer (1835-1920). Austrian otologist, born October 1, 1835, Alberti, Hungary; died August 10, 1920, Vienna. He has been recognized as a prolific author and teacher. He established in Vienna from 1861 to 1913 the first successful university aural clinic of his time.

In 1877 he introduced one of first mechanical audiometers. In 1893 he first correctly described otosclerosis as primary disease of labyrinthine capsule, rather than as being secondary to chronic interstitial middle ear catarrah, as was thought. He published an atlas of the first photos of tympanic membrane by illumination. His classical textbook Lehrbuch der Ohrenheilkunde was the Standard for many years.

He wrote ‘‘The history of otology’’ in two volumes (Geschichte der Ohrenheilkunde. In 1900 he and Siebenmann condemned stapes mobilization and threw the operation into disrepute for decades.

Today, his name remains associated with Politzer bag, cone of light, speculum, and test for unilateral deafness (Saunders/Paparella/Miglets, 1980).

 

These are termed   ‘‘histologic otosclerosis’’. In only 12% of ears with otosclerosis, in Guild’s study, did the focus reach the oval window and footplate of the stapes, producing a conductive type of hearing loss. These are termed ‘‘stapedial otosclerosis’’ and constitute the most frequent cause for progressive conductive type of hearing loss in adult life. In the majority of cases of otosclerotic fixation of the stapes there is also involvement of the wall of the cochlea with a sensory-neural type of hearing loss.

A pure sensory-neural or nerve type of hearing impairment can be due to involvement of the wall of the cochlea without involving the oval window.. These are termed ‘‘cochlear otosclerosis’’. In contrast to stapedial otosclerosis where hearing can be restored by stapes mobilization or by stapes extraction replacing the stapes with a stainless steel or tantalum prosthesis, the sensory-neural loss of cochlear otosclerosis cannot be restored.

It was with the hope of finding a way to promote recalcification and inactivation of an a ctively growing otosclerotic focus so as to retard or arrest the progressive sensory-neural loss of cochlear otosclerosis that the following experiments were undertaken.

 Historical events of the surgical treatment of otosclerosis

The earliest attempts to improve surgically hearing in stapes fixation were directed to the stapes itself. In 1878 Kessell incised the posterior part of the tympanic membrane, separated the incus from the stapes, removed part of the bony meatal wall when necessary for proper exposure of the stapes and then tried to mobilize the stapes by applying pressure to its head in various directions. (Kessel employed first the endaural approach to temporal bone and described first radical mastoidectomy, using endaural approach in 1885).

In 1878 Kessell performed stapes extraction, but because of infection, switched to stapes mobilization. He described sound projection of round window in 1879, using remnants of drum membrane. Later, in 1885, he reported hearing improvement by adhesion of drum membrane to head of stapes.

In 1888 Boucheron reported the mobilization of 60 stapes, with the best results in cases of early ankylosis without involvement of the sound-perceiving apparatus.

In 1890 Miot  in a series of five articles reported 200 stapes mobi1izations with techniques and results amazingly similar to Rosen's operation of 62 years later, Miot observed the improvement in bone conduction ("cranial perception") following successful mobilization due, we know nοw, to elimination of Carhart's notch. There were nο deaths or labyrinthine complications in his series. Miot stressed indications, aseptic technique, ossicular chain continuity, and postoperative Valsava maneuvers (Saunders/Paparella/Miglets 1980)

In 1892 Blake of Boston practiced stapes extraction for otosclerosis, in order to improve hearing, and in 1893 Jack reported a series of cases of extraction of the stapes. Ten years later he described one of these as having maintained quite good hearing, noting that: "The drums have healed.  The portion covering the seat of the operation is somewhat sunken forming a moveable membrane οn the oval window. ... Remova1 of the stapes does not destroy the hearing but sometimes improves it. The contrary statements in most text­books ...were incorrect."

In 1897 Passow reported first operation for otosclerosis in which ankylosed stapes was bypassed. He drilled new fenestra at promontory covering it by the tympanic membrane. Such an operation had relieved tinnitus and slightly improved hearing.

In 1899 Faraci  pub1ished his results in 30 cases of stapes mobilization. That was the last recorded stapes operation to improve hearing for more than half a century, for in 1900 at the International Congress Siebenmann was joined by Politzer and other leaders in otology in condemning all surgical attempts to improve hearing in otospongiosis as both useless and dangerous. The reasons for the concerted and effective opposition is not clear, and one can but surmise that there were some unreported serious comp1ications.The first stapes era began in Europe, ahead of its time, and in those halcyon days before the turn of the century, the Belle Epoch, proceeded, uncorrected to its tragic extreme, and then was stopped suddenly, quite rightly, by the establishment (Shea JJ Jr., 1998).

Prior to 1938 otospongiotic stapes fixation was rarely diagnosed in the USA and UK, and when recognized was considered a dreadful, progressive disease for which there was nο treatment, leading inexorably to profound deafness. The earliest and forgotten operations upon the fixed stapes by Kessel in 1878,Miot in 1890, and Blake and Jack in 1892 and 1893 might have begun a fruitful development of operations for stapedial otospongiosis had not Siebenmann's pronouncement of 1900, joined by Politzer and other leaders in otology, that "attempts at mobilization of the stapes are not only useless but often harmful" effectively banned for more than half a century further surgery upon the fixed stapes. 

Fig.6. Gunar Holmgren (1875-1954) He revived interest in surgical correction of hearing loss.

He pioneered the fenestration operation and performed long series of operations for otosclerosis, demonstrating that with modern sterile technique, inner ear could be opened safely.

In 1922 he introduced use of binocular operating microscope in otosclerosis surgery. Founder of the Acta Oto-Laryngologica.

 

The first report, after Siebenmann's pronouncement, of surgery for otospongio­tic stapes fixation came from England in 1913 when Jenkins made a fenestration in the horizontal semicircular canal and covered it by a Thiersch skin graft (a very thin skin graft consisting of epidermis and a thin layer of dermis, often cut in long, broad strips (Thiersch 1874), with a slight improvement in hearing in two patients. 

7.  Robert Bárány was born on April 22, 1876, in Vienna. He died at Uppsala on April 8, 1936. He invented chair for use during vestibular testing; devised pointing test for localization of circumscribed cerebellar lesions; and described syndrome of unilateral deafness, vertigo, and pain in occipital region.

 

In 1914 he received Nobel prize as  clarified the physiology and pathology of human vestibular apparatus. He corrected deduced mechanisms of action of vestibular apparatus from his experiments with caloric testing.

He established both caloric testing as diagnostic tool and new criteria for diagnosis of labyrinthitis.

Bárányand then Holmgren in 1917, quietly dared to go against the prevalent opposition to surgery for stapedial otospongiosis in a long series of  operations to "decompress the labyrinth" by making various fistulas, usually in a semicircular canal. Repeatedly, an immediate improvement in hearing was observed, but always it receded in the ensuing months.

In 1921, the microscope was used for the first time in ear surgery by a Swedish otologist, Carl Olaf Nylén. This monocular microscope was rapidly replaced by a binocular microscope developed in 1922 by Gunnar Holmgren. The microscope followed an evolutionary process that led to the actual binocular microscope, which is found in every institution where otology and ear surgery are performed (Mudry 2000).

The operations reported by Holmgren, who used careful sterile technique, gradually dispelled the dread of opening the labyrinth in a clean field. Using a binocular operating microscope, Holmgren had applied Nylen's operating microscope to otospongiosis surgery 1922. Holmgren demonstrated that osseous closure of the labyrinthine window was the great stumbling block to permanent hearing improve­ments in these cases. He was the first to observe through the operating microscope the otospongiotic (otosclerotic) focus in front of the oval window fixing the stapes.

Holmgren's scientific integrity, backed by the experimental investigations of Engström and the histologic observations of Nager helped to dispel the prejudice against surgery for otospongiosis.

 

 

Fig.8. Maurice Louis Joseph Marie Sourdille (1885-1961).

He achieved first permanent restorations of hearing for the cure of progressive hearing loss in patients with otosclerosis uniting a neotympanic system to a labyrinthine fistula (tympanolabyrnthopexy involved fenestrating horizontal canal and covering it with meatal skin flap.

This complicated, multistage procedure was the basis on which Julius Lempert in 1938 developed one stage fenestration operation.

 

 In 1924 Sourdille visited Holmgren 's clinic and observed operations for constructing a horizontal canal fistula . When he returned to France he devised the method of covering the fistula in the horizontal semicircular canal with a meatal skin flap attached to the tympanic membrane.

For the first time, lasting improvements hearing were achieved by fenestrating the labyrinth.Shamaugh is describing in the textbook SURGERY OF THE EAR (Shamaugh & Glasscock, 1980) two cases of Sourdille: In 1947 I examined two of Sourdille's early operations, one done in 1932, and the other in 1935.

In  both patients, the hea1ed cavity appeared exact1y like one of Lempert's early fenestra­tions with the incus beneath the flap and the wide open fenestra clearly visible through the very thin skin covering it, and both patients had excellent hearing comparable audiometrically to successful Lempert fenestration operations.

 Sourdille reported the results of his technique before the New York Academy of Medicine in 1937. Lempert was influenced attending this lecture and was inspired to use this method in a series of cases of stapes fixation. With a dentist's drill Lempert created a new window on the horizontal canal, in this way the sound wave could stimulate the inner ear, (Pietruski J., 1998).

Lempert modified the technique and performed the operation endaurally and in one stage, rather than in the two or three stages that Sourdille employed. Lempert did precisely what Sourdille done in leaving the incus in place. He was amputating the head of the malleus and covering the horizontal semicircular canal fenestra with a tympanomeat­al skin flap. He named his operation "fenestration operation" instead of Sourdille's "tympanolabyrinthopexy."

Meanwhile, due to several developments it was set the stage for the revival ofoperations upon the fixed stapes: In  1938 Lempert with his one-stage operation for fenestration (Lempert's fenestration) had overcome the prejudice against surgery for otospongiosis; antibacterial drugs afforded added protection against infection; electric illumination and magnification by loupe made endomeata surgery more practical; audiometry to measure and report hearing results verified improvements: and last, but not least, Lempert's endomeatal ap­proach to the tympanic cavity without perforation of the tympanic membrane proved ideal for operations upon the stapes. All the work of Lempert led to the renaissance of reconstructive surgery for conductive deafness.

Through his personal efforts to teach his operation to many otologists, he was largely responsible for changing climate of otologic thought in favour of reconstructive surgery. The fenestration operation, hardly ever employed today for stapedial otospongiosis and only occasionally for congenital malformations of the middle ear. This operation will remain forever as a mightly landmark in the evolution of surgery to restore hearing. The fenestrated ears required annual or oftener cleaning of the fenestrated cavity, but the successfully stapedectomized patients have no need for repeated visits to their surgeons.

Soon after Lempert's first publication in 1938, other otologic sur­geons from America and abroad came to observe his skilful endaural technique and his favourable hearing results, and remained to learn the technique by cadaver dissec­tions. Meanwhile, Lempert found that the incus was not needed as Sourdille had insisted "to carry sound across the mobile incus from the tympanic membrane to the horizontal canal window," and by removing the incus, a larger fenestra could be constructed over the ampulla of the horizontal canal. This Lempert named the "fenestra nov-ovalis," as it resembled in size the oval window that it replaced.

 

Fig 9.. Julius Lempert (1890-1968). Juliusz Lempert was born in 1890 in Poland. A few years later the poor Jewish family emigrated to the United States. Lempert obtained his MD degree at Long Island Medical College, and soon after that established a small hospital of his own in Manhattan.

Later he bought an old five-storey building and converted it into a new otological medical center, which he called Endaural Hospital. His life was rather difficult with the wave of antisemitism in the United States and the adversity which he encountered so many times. However his contribution to the microsurgical treatment of conductive deafness is immense, (Pietruski J., 1999)

 In 1938 he succeded the one-stage fenestration operation. Uniting a neotympanic system to a labyrinthine fistula for the cure of progressive hearing loss in patients with otosclerosis.

 The fenestra operation is now replaced by stapes operations.

‘‘Fenestration does not pretend to cure the lesion of otosclerosis in the region of the oval window but it can and does cure clinical otosclerosis by rendering it symptom free….’’

LEMPERT

Lempert was never a member of any ENT society and worked in his hospital alone. When suddenly his only son was stricken with leukemia and died, Lempert was completely broken, and never returned to this work. The next blows were new operative methods of otosclerosis: stapes mobilisation introduced by Rosen and stapedectomy by Shea. He never accepted these new techniques. It was a painful experience for a surgical genius who had at so many times been hurt during his life. He could not believe that his fenestration was definitely gone. Lempert quickly deteriorated physically and mentally, and died in 1958 (Pietruski J., 1999)

It was Lempert's one-stage endaural open operation that gave the fenestration operation the worldwide acceptance it gained, (Shea JJ Jr., 1999).

 

  Τhe fenestration era proceeded to an extreme, when its technical master Julius Lempert would allow no criticism or improvement in "his" one-stage endaural technique, however good, nor would he accept the new mobilization and stapedectomy operations, and he and it ended badly, (Shea JJ Jr., 1999).

In 1940 Shambaugh further refined Lempert’s fenestration operation. In a series of experimental fenestrations on monkeys done by Shambaugh   and his wife, they developed a microsurgical technique with enchondralization of the fenestra and continuous irrigation under an operating microscope to remove all bone dust particles. With this technique it was reduced to around 5 per cent of fenestrations, the tendency toward osteogenic closure of the fenestra. The one-stage fenestration operation introduced in 1938 by Lempert with the application to it of the operating microscope in 1940 by Shambaugh  marked the turning point from surgery for aural suppuration to the extraordin­ary blossoming of microsurgery in a clean field for the restoration of hearing(Shea JJ Jr, Derlacki E, 1999).

Meanwhile, beginning in 1940 Wullstein and Zöllner were among the first who used the microscope for fenestration operations, and then used it for their pioneer techniques of microsurgical tympanoplasty. Wullstein's lectures at the First Workshop in Chicago in 1959 convincingly established the techniques of tympanoplasty and the advantages of the operating microscope, until then opposed by Lempert who insisted that the microscope "made things larger, but not clearer."

 

 

                                    Fig. 10                                                                 Fig. 11

Zöllner and Wullstein were among the first who used the microscope for fenestration operations, and then used it for their pioneer techniques of microsurgical tympanoplasty

 The Zeiss otomicroscope, demonstrated for the first time in 1951 soon became an essential tool in the new era of otologic microsurgery in a clean field to restore hearing.This model replaced all other models progressively, thanks to its ease of use and the possibility of changing the magnification without modifying its focal distance.

It allowed for the development of tympanoplasties and stapes surgery. The latest developments include the three-dimensional imaging and navigation systems, (Mudry, 2000). 

Fig. 12. George E. Shambauch, Jr. (1903-1999).

He further refined Lempert’s fenestration operation in 1942, adding suction-irrigation apparatus and binocular operating microscope.

In 1964 he proposed the use of moderate doses of sodium fluoride to promote recalcification and inactivation of an actively expanding focus of Otospongiosis.

 

 

Fig 13. Samuel Rosen (1897-1981): The originator of stapes mobilization. He was born in Poland and died in China.

Samuel Rosen did not however limit himself to microsurgery of the ear. He created a group of international scientists who on the basis of investigations carried out by some of them, in the quiet noiseless African bush demonstrated that not only hearing is protected and the ageing process of this sensory organ delayed but also the development of arteriosclerosis is slowed down that which is the root cause of more and more cases of heart disease which among other factors can be attributed to the noisy stress ridden world we live in.

After much success and fame which he achieved throughout the world, the American Medical Association awarded Sam Rosen a gold medal in 1967 (Pietruski J., 1999).

 

In 1952 Samuel Rosen unaware of Miot's earlier work, began to use Lempert's endomeatal approach to the tympanic cavity to palpate the stapes and verify the degree of fixation prior to fenestration. In one such case the stapes suddenly mobilized, with a remarkable restoration of normal hearing, even better than fenestration would have yielded. Rosen followed this chance observation by purposeful attempts to mobi1ize the stapes, reporting hearing improved to the 30 decibel level (A.S.A.) or better for the speech frequencies in 22 per cent of 211 operations.

Samuel Rosen was the originator of the surgical procedure so called “mobilization”, was second only to Julius Lempert as one of the great modern discoverers of new surgical techniques in the treatment of otosclerosis. Rosen having had excellent scientific training and knowledge was well prepared to interpret accidental stapes mobility and so design a new surgical technique. This operation enabled thousands of patients with otosclerosis to regain their hearing. However, he did not receive widespread acclaim in his own country. He received many invitations from abroad, travelled to several countries around the world where he taught stapes mobility testing and demonstrated his surgical procedure. He died in 1981 in China (Pietruski J., 1999).

Soon many fenestration surgeons were attempting preliminary stapes mobilization which, when successful, could surpass the hearing achieved by fenestration with a fraction of the time needed for convalescence and nο need for lifetime fenestration cavity care.

The microscope made possible the development by Heermann and Derlacki of microchisels to loosen the foot plate, of anterior crurotomy mobilization by Basek and Fowler, and finally the revival of stapedectomy by Shea in 1956. Unlike Blake and Jack, who failed to comprehend the need to reconstruct an ossicu1ar chain and who left the oval window open, Shea closed the ova1 window after extraction of the stapes "with a thin slice of connective tissue" and inserted a prosthesis from incus to oval window to restore the norma1 mechanics of the middle ear.

 


Fig. 14. John J. Shea, Jr., M.D. (September 4, 1924 – February 8, 2015) was the son of Ear, Nose and Throat specialist John J. Shea, Sr., M.D. (1889-1952). Dr. Shea, Jr. attended Notre Dame, where he graduated magna cum laude, and Harvard Medical School, where he graduated with honors in 1947.

He did his residency at the Massachusetts Eye and Ear Infirmary in Boston and served in the United States Navy Medical Corps during the Korean War.

Dr. Shea became interested in the treatment of hearing loss and went to Los Angeles to study with Dr. Howard House in 1953 and to Vienna, Austria in 1954 to study at the University of Vienna. He performed the first successful stapedectomy in May, 1956.

The patient was a 54 year-old housewife who could no longer hear even with a hearing aid. Dr. Shea removed the stapes, covered the oval window opening with a vein graft removed from the back of the patient's hand, and inserted a prosthesis to replace the diseased stapes bone.

The patient’s hearing was restored and she heard well for the rest of her life. Dr. Shea has performed nearly 40,000 stapedectomies during his career with recovery of hearing in more than 90%. His technique with very little modification is now used all over the world.

Dr. Shea was a member of more than fifty scientific societies and has received honorary doctorates from Christian Brothers University and Rhodes College in Memphis and honorary fellowships from the Australian and English Royal College of Surgeons.

 

 

 The second stapedectomy era began before the fenestration era ended with the accidental and originally unrecognized mobilization of the stapes by Rosen and the resurrection of stapedectomy by Shea JJ Jr who sealed the oval window with a living elastic membrane and reconstructed the sound-conducting mechanism of the middle ear with biocompatible implant prosthesis to make it successful.

For Shea, in 1955-1956, the "Zeitgeist" was finally right. Shea realized the stapes could be removed and covered the oval window with a vein graft, and Harry Treace made for him biocompatible implant prosthesis out of the newly discovered Teflon. For a new technology to be accepted, it must be much better than what it replaces, and stapedectomy was much better than fenestration. In the new microsurgical era of otology that began, improvements in the stapedectomy operation came from everywhere and were readily accepted.

 Many modifications of Shea's stapedectomy have been designed and employed, all following the principles firmly established by Shea that the ova1 window needs to be sealed, and there must be ossicular continuity by using the posterior crus of the stapes or a prosthesis frorn incus to oval window.

Among these modifications are a prosthesis of fat tied to a wire lοοp; a preformed wire lοοp against compressed Gelfoarn, vein, fascia or perichondrium to seal the oval window; and a piston-type prosthesis of stainless steel, tantalum, platinum or teflon from incus to oval window, Most surgeons currently prefer a tissue graft, rather than Gelfoarn, to seal the oval window', as being less likely to result in a perilymph fistula.

The tendency for a preformed wired  lοοp to be come adherent to the edge of the oval window has prompted the use of a piston-type prosthesis of teflon or stainless steel, in contrast to results obtained when removing the entire stapes footplate or a major part of it, there appears to be less labyrinthine reaction when the prosthesis is inserted into a smaller opening in the footplate, using a very thin graft made from a pressed vein or from  the dried loose areolar tissue superficial to the temporalis fascia.

Stapedectomy has now become so successful, like many treatments in medicine, the problem has now largely disappeared (Shea, 1982). After 30 years of stapes surgery Shea declared: The modern stapedectomy with prosthesis insertion and living oval window seal, like the modern cataract extraction with lens replacement, is now performed, very much the same, throughout the world. I have reviewed the evolution of stapes surgery during these last thirty years and tried to gain some agreement for several important facts about otosclerosis and several basic principals of stapes surgery. While a well-performed stapedectomy can eliminate the conductive component, the sensorineural hearing loss continues and, in about one-third, will progress till the patient, after age 65, must return to a hearing aid.

Piston prosthesis gives the best hearing results: 0.6 mm diameter, when half the footplate is removed and a living oval window seal interposed, and 0.6 mm diameter when a small opening is made in the footplate obliterated by otosclerosis. I prefer a teflon prosthesis to stainless steel because it can be altered by the surgeon at operation, and vein as an oval window seal.

I have presented a rare group of patients who develop facial palsy 5-1/2 days after uncomplicated stapedectomy, of whom all recover quickly and completely. I am confident that progress will continue to be made in the understanding of otosclerosis, and the performance of stapes surgery, but in these last thirty years we have made a good beginning (1988).

Surgical technics for bypassing ankylosis of the stapes in the management of otosclerosis gradually evolved; modifications were prompted by problems which were revealed by the ongoing audits carried out by earlier surgeons. Auditing of performance must remain an integral part of any otologist's work. The natural history of otosclerosis should be taken into account so that a patient may be fully informed of treatment alternatives available. Stapedectomy will remain a most effective operation which can be recommended with confidence, provided the surgeon has acquired the skill to perform it properly. It is now accepted that many ear, nose and throat surgeons are neither physically nor temperamentally capable of this task and should not perform stapedectomy, (Willis, 1991).

 In 1999 Shea wrote ‘‘If the measles virus is the cause of the growth of the otosclerotic focus, as it seems to be, then vaccination against measles eventually will eliminate the hearing loss of otosclerosis completely. What the history of stapedectomy reveals is the truth of the quotation from Ecclesiastes, "There is nothing new under the sun." Progress is only made when the Zeitgeist is right, by someone who puts together the truths of the past with the new discoveries of the present’’, Shea JJ Jr.1999.

Stapedotomy or the small fenestra technique was originally used in patients with obliterated or solid footplates.  It was initially performed in Europe and began to gain acceptance in the United States in the late 1970s and 80s.  Because of the early success of this technique, many began using stapedotomy for non-obliterative cases.  Decision to perform total or partial stapedectomy versus stapedotomy depends on the extent of stapes fixation and other characteristics of the foot plate, and surgeon preference. 

The advantage of stapedotomy include less risk of trauma to the vestibule and less incidence of migration of the prosthesis and fixation by scar tissue as is seen in stapedectomy techniques.  The advantages of stapedotomy over stapedectomy in terms of producing less postoperative hearing loss and dizziness, have been reported in many series, (Fisch, 1982, Causse, 1985, Marquet 1985, Lippy et al, 1999), but according to House et al (2002), in the hands of an experienced surgeon, either technique provides satisfactory and stable long-term results.Initially sharp instrumentation was used to make the fenestration which required a great degree of technical proficiency.

In 1978, the first laser stapedotomy was performed by Perkins and has since become a widely accepted method for fenestrating the footplate (Lippy et al, 1999). The three most commonly used lasers for stapedotomy are the potassium titanyl phosphate (KTP/532), argon, and carbon dioxide lasers.  The KTP/532 and argon lasers have similar biosurgical effects.  They both are visible beams of light and therefore, do not require a separate laser to act as an aiming beam. 

This allows for far greater target accuracy because they act as their own aiming beam.  The CO2 laser is an invisible surgical beam and must be used with a separate aiming beam, usually a red helium-neon beam which produces an ill-defined fuzzy border.  The CO2 beam must be perfectly lined up with the aiming beam or the target will be off center.  Both the KTP/532 and the argon lasers are absorbed by hemoglobin making them better coagulators than the CO2 laser. 

The CO2 laser is less expensive and requires less maintenance. When using the laser, it is important to evaluate all equipment in the pre-operative period to ensure proper function.  The risk of using the any laser in the oval window area is overheating of surrounding tissue including the facial nerve.  Causse recommends allowing 10 to 15 seconds between pulses to allow for cooling.   In addition suction must be used to evacuate the smoke and inadvertent suctioning of perilymph may occur. 

In addition to the laser, a small microdrill can be used to create a fenestration in the footplate.  Most commonly a 0.7mm diamond burr is used.  Some surgeons use the drill after using the laser to complete the fenestration which allows the turning of the drill bit to remove bone dust and fragments (Causse et al., 1993).  The fenestration site is usually aimed at the center of the footplate, however, Causse recommends placing the fenestration more posteriorly.  He argues that because the stapes actually produces a vestibular fluid wave by rocking posteriorly, placing the piston into a more posterior fenestration is more physiologic. 

Laser stapedotomy has been shown to have advantages over traditional small fenestra stapedotomy technique. By vaporising the posterior crus and fashioning the hole in the footplate, the operation is thought to be less likely to result in trauma to the inner ear (Shabana et al, 1999).

 Patients diagnosed with far-advanced otosclerosis have a good prognosis with cochlear implantation comparable to that of other patients in whom postlingual implants are performed.

 Medical therapy of otosclerosis

 As with conductive hearing losses of other etiologies, hearing aids are usually helpful in otosclerosis. In 1923, Escot was the first to suggest that calcium fluoride be used in the treatment of otosclerosis.  It was later popularized by Shambaugh (1965) the administration of sodium fluoride (Florical). 

NaF reduces osteoclasis and increase osteoblastic bone formation. Naf prevents sensorineural deafness by acting on cytotoxic enzyme. The fluoride ion replaces the usual hydroxylion in periosteal bone, forming fluorapatite, instead of the usual hydroxyapatite.  This results in decreased bone resorption and increased calcification of new bone.  Actively expanding foci of otosclerosis are inactivated, as has been documented by computed tomography. 

In addition, tinnitus and imbalance are reduced, and Schwartze’s sign frequently becomes negative.  The most frequent side effects are rash, arthritis, and gastric distress.  Permanent serious side effects are very rare.  It is available over the counter as Florical (sodium fluoride and calcium carbonate), and the usual dose is about 20-120mg of fluoride a day in adults.  This dosage is decreased for children and pregnant women. 

After two years, the efficacy of the treatment can be evaluated.  Schwartze’s sign, and the degree of tinnitus and imbalance are reassessed, and a CT scan is repeated.  If overall stabilization of the disease has occurred, the patient is placed on a life-ling maintenance dose of about 25mg of fluoride a day. 

Sodium fluoride treatment should be considered in patients who are not surgical candidates, those who decide against surgery, and those who have SNHL or vestibular symptoms believed to be due to otosclerosis.  In addition, patients who are candidates for surgery with an active focus detected by a positive Schwartze’s sign may be given fluoride treatments for 6-12 months prior to surgery to induce the focus to mature and potentially prevent the progression of disease after surgery. 

If the focus is determined to be active during surgery, postoperative treatment can be initiated.  Overall, 50% of patients have stabilization of their disease, 30% improve, and the rest continue to progress.  Stopping the treatment in those patients whose disease process stabilized while on therapy may result in a reactivation of the disease in 2-3 years. 

Forquer BD, et al (1986) examined the effectiveness of sodium fluoride in treatment of cochlear otospongiosis in ninety-four patients with cochlear otosclerosis and ninety-eight patients with stapedial otosclerosis and sensorineural hearing loss. The drug halted or slowed the progression of sensorineural hearing impairment in 63% of the patients with cochlear otosclerosis and 46% of the patients with stapedial otosclerosis. The single factor that best predicted which patients would respond most favourably to treatment was rate of progression before treatment. Sodium fluoride therapy was successful for 79% of the patients losing their hearing at a rate of 5 dB or more per year at one or more of the speech frequencies.

The finding that patients with more rapid rates of progression responded most favourably to sodium fluoride therapy suggests that patients with the most active otospongiotic processes will be the most responsive t

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