Otosclerotic Deafness Research Articles

Hemorrhagic Diathesis in van der Hoeve's Syndrome

To the Editor. —The main symptoms in van der Hoeve's syndrome, a disease of simple dominant inheritance, are blue scleras, osteogenesis imperfecta, and otosclerotic deafness, 1 but the literature contains almost no reference to the hemorrhagic disposition associated with this condition. We recently had a chance to study the hemostatic system in a patient with hemorrhagic diathesis. Report of a Case. —A 28-year-old woman had 15 occurrences of bone fracture and reported that hemostasis had presented difficulties on each occasion. She was seen because of purpuric episodes. The patient's height was 147 cm and her weight was 38 kg. The specialists all agreed in their diagnosis of blue scleras, osteogenesis imperfecta, and conductive deafness. Hemorrhagic diathesis was observed in the form of a large number of purple spots in the lower limbs, but no internal hemorrhage was detected in the joints or muscles. Although there was no consanguinity, hemorrhagic diathesis

The spring prosthesis in stapedectomy.

IN JANUARY 1965, we evolved a new prosthesis for use in stapes surgery. 1 This took the shape of a spring, made of stainless steel wire and prepared on a Schuknecht die. We advocated its adoption in preference to other well-known prostheses, because, in theory, it could protect the labyrinth and fulfill at least one of the functions of the stapedius muscle, which is usually severed in stapedectomy operations (Fig 1). The excellent results obtained by stapedectomy in otosclerotic deafness tended at first to overshadow some of the side-effects. But with the passing of time, more attention has been given to perfection of technique and to better hearing results, especially in the drop for high tones which frequently occurs after stapedectomy. 2,3 The importance of the stapedius muscle was known to clinicians in the last century. Muller in 1838 described the protective role played by the middle ear muscles whenever

Mobilization of the Perilymph: Assessment of Techniques

THE PURPOSE of this paper is to clarify, simplify, and bring scientific order to the nomenclature used to describe the many different techniques of stapes surgery for otosclerotic deafness. There are now more than 25 techniques, all different in approach and yet all capable of restoring normal or near-normal hearing. The physiology and pathophysiology involved in stapes surgery are most often spoken of in terms limited to a description of a technique without sufficiently explaining and evaluating its effect. Individual surgical methods are evanescent, changing, and, as we know, not final. However, there are relationships of all these variable techniques to a structure which remains constant at all times. This structure is the perilymph. A successful surgical result depends inevitably upon the mobilization of the perilymph. None of the stapes operations can restore hearing until the cochlear fluids are set in motion. Under normal conditions the drum and ossicular chain

Surgery for Otosclerotic Deafness

Surgery for Otosclerotic Deafness

Platinectomy and vein grafting of the oval window in otosclerotic deafness.

Platinectomy and vein grafting of the oval window in otosclerotic deafness.

Fibromatous polyp of the vestibule. A complication of stapedectomy.

Significant strides have recently been made in the development of surgical techniques for the relief of otosclerotic deafness. At present, the otologic surgeon has at his disposal a number of surgical procedures which can be applied to meet the varying technical problems encountered among his otosclerotic surgical patients. In this regard, probably the most important single contribution to otologic microsurgery evolved during the past 5 years is the introduction by Shea of the total stapedectomy with vein graft and polyethylene prosthesis. 1 Since its first published description, a number of variations of Shea's original procedure have been introduced and applied with approximately the same incidence of significant improvement in auditory sensitivity. 2-5 It is important to emphasize that all of these procedures possess a common element—the partial or complete removal of the stapes footplate and the use of some transplanted material to cover the patent oval window. The present

Assessment of the techniques of stapes surgery. Is there a single stapes operation preferable in every case of otosclerosis?

There are many different procedures for surgical operation on the stapes in the treatment of otosclerotic deafness. Each is capable of restoring the patient's hearing completely, but the procedures differ in the degree of risk involved. From experience with more than 5,000 stapes operations, the author concludes that no single technique guarantees complete success, and none is guaranteed against some failures. Total stapedectomy, when necessary, has been very effective, but it carries the greatest risk of irremediable cochlear damage and even total deafness. The author therefore believes that the proper order in choosing techniques for stapes surgery is to begin with mobilization and end with stapedectomy.

Familial transverse nasal groove. 9.

In 1951 Cornbleet described a minor cutaneous defect, a red transverse stripe or groove across the nose.1,2The groove is 1-3 mm. wide and about 1 mm. deep, is continuous, and lies across the nose, just proximal to the alae nasi (Fig. 1). This paper reports 2 families in which 14 examples of the transverse nasal groove were found. The pattern of inheritance suggests that the trait is due to a single dominant gene. Pedigree I shows that whenever verification can be made affected individuals have an affected parent. The nasal groove was associated with hyperelasticity of the joints in 4 cases. Otosclerotic deafness at an early age occurred in I-3; II-2, and III-7. Diabetes was present in II-3. No allergic rhinitis was found. The irregular genetic pattern seen in Pedigree II is best explained by the assumption that, as in Pedigree I, the trait is caused by a

A surgical and anatomical classification using a polyethylene tube prosthesis in stapes surgery.

The success of operative mobilization of the stapes for otosclerotic deafness depends upon restoration of sound conduction via the ossicular chain to the cochlea. The conductive impairment of sound in cases of otosclerosis is due to ankylosis of the stapes. In the past, improved hearing would result through surgery of the stapes only if the footplate were loosened and remained in continuity with the crura. This meant that there could not be a break in the transmission of sound from the tympanic membrane to the cochlea if improved hearing was to be obtained. Unfortunately, since the degree of stapes fixation and its anatomic structure vary, its delicate crura would separate from the footplate before it could be mobilized; thus, even if the footplate were mobilized, the gap in sound transmission prevented all possibility of improved hearing. This, briefly, was the reason for the operative failure in so many patients who

The stapedectomy procedure of Shea. A report of 250 patients with follow-up study.

The past two decades have produced a number of major advances in the concept and techniques of otosclerosis surgery. The almost universal acceptance and successful application of the single-stage lateral canal fenestration and stapes mobilization procedures have been far too well described elsewhere to require further comment here. The stapedectomy technique of Shea, 1 however, is of more recent origin. Because of the high degree of effectiveness of this technique, it seems appropriate for us to report on our experiences with it and to also present an analysis of follow-up audiometric data. Our awareness of the need for the development of additional microsurgical techniques for the relief of otosclerotic deafness dates back several years to observations made during a series of revisions of previously done stapes mobilizations. We found that many of these earlier patients had failed to gain in auditory sensitivity postoperatively because of various surgical complications which involved

Methods of stapes surgery for otosclerosis.

Stapes surgery today requires the utmost resourcefulness and discrimination in the selection of the technique most appropriate in each case. In 1952, I mobilized the rigid stapes in a case of otosclerotic deafness by exerting pressure upon the neck of the stapes. For a period of time this indirect mobilization of the footplate was the sole technique employed in surgery of the stapes to improve hearing. Good results with this technique occurred in approximately one-third of the cases.^1-10Some of the unimproved cases were then reoperated on and resulted in significantly improved hearing, while others became candidates for the fenestra novovalis operation. The necessity for increasing the percentage of favorable results led me to a direct mobilization of the footplate at the footplate itself in those cases where the indirect method did not succeed. A needle, or explorer, was inserted at various points around the periphery of the footplate

Procedure of "interposition" for otosclerotic deafness.

Procedure of "interposition" for otosclerotic deafness.

Principles of stapes surgery.

Perhaps the best way to describe the principles on which stapes surgery rests is the term dynamic. Modern stapes surgery is not a standardized or fixed procedure; it is rather in process, constantly developing, growing, and expanding. Our measure of advance is the degree of success we meet in restoring and maintaining hearing by the best possible methods. In the seven years of its renaissance, it is not an exaggeration to say that stapes surgery has revolutionized our approach to the problems and surgical treatment of otosclerotic deafness. But since stapes surgery is yet in its infancy, experimentation, evaluation, and careful observation of all that is being done remain of paramount importance. Underlying all growth, change, and progress, there is inherent order. A state of fluidity and experimentation does not mean chaos. Stapes surgery, for all its growth, also has its natural order, and we would do well not to

The Surgical Significance of Stapedial and Labyrinthine Anatomy

The authors' purpose in presenting this report is twofold: First, to describe features in the developmental and adult structure of the stapes pertinent to problems that confront the otologist in attempting stapedial mobilization; second, to demonstrate the relation of the footplate to the otic (endolymphatic) labyrinth—a relationship which inescapably places the labyrinthine duct-system in jeopardy in the course of endaural surgery. In fact, in every variant of the fundamental approach there is danger of damage to some part of the stapes or to vital parts of the endolymphatic (otic) labyrinth. The literature on early attempts at stapes mobilization, reviewed by authors herinafter listed, need not be repeated here. Six years have elapsed since mobilization of the stapes became established as a practical procedure for the restoration of hearing in cases of otosclerotic deafness. Since then, this technique (according to Rosen, 1958) has been employed in an estimated 25,000 cases of

The use of a prosthesis in stapes mobilization.

Restoration of hearing in otosclerotic deafness depends on being able to reestablish the ossicular conductive mechanism so that air-borne sounds will reach the cochlea. Inability to relieve the stapes ankylosis, which is responsible for the hearing loss, is the reason that mobilization surgery fails. Whether the stapes can be mobilized completely, with both crura and the footplate intact, depends on the degree of fixation and the anatomical structure of the stapes.¹Occasionally, when the crura fracture, part of or the entire footplate will be simultaneously mobilized, but more often it will remain solidly fixed. In either case, there is usually a separation of the crura from the footplate (Fig. 1). We know that there must be continuity of ossicular transmission to improve hearing but that it is not necessary to free the entire stapes in one piece Fig. 1.—Fracture of crura and their separation from the footplate. to accomplish

Long term results of surgery for otosclerotic deafness.

Long term results of surgery for otosclerotic deafness.

Bone-conduction thresholds in stapes surgery.

The measurement of pure-tone bone-conduction thresholds figures prominently at present in the selection of cases for stapes surgery for otosclerotic deafness and in the prediction of postoperative results. 1 In the period preceding the development of modern stapes surgery, the importance of the mechanical shift in bone-conduction thresholds following successful fenestration surgery of the horizontal canal was accepted generally along the lines suggested by Carhart, Shambaugh, and McConnell. 2,3 The average improvement in these thresholds as postulated by Carhart was as follows: no shift at 250 cps, 5 db. at 500 cps, 10 db. at 1,000 cps, 15 db. at 2,000 cps, and 5 db. at 4,000 cps. A carefully controlled study by McConnell and Carhart yielded somewhat different values of shifts following successful fenestration nov-ovalis, namely: 1.2 db. at 250 cps, 1.4 db. at 500 cps, 6.5 db. at 1,000 cps, 7.4 db. at 1,500 cps, 8.5 db. at

Experimental fractures of the stapes in rabbits; histopathological studies.

Introduction The mechanism of surgical mobilization of the ankylosed stapes for the relief of otosclerotic deafness has in the last few years become one of the most widely discussed problems in otology. Although very worth-while results have so far been achieved with various operative maneuvers, none of them is completely satisfactory, and therefore the attempts at improving the surgical techniques as well as theories concerning the mechanism continue to develop. The opinions of different authors about the best approach to the problem vary greatly. As stated repeatedly before, it is the conviction of the group doing mobilization surgery at the Presbyterian Hospital in New York that mobilization through the otosclerotic focus itself is not likely to be lasting except in rare instances. The best chance to be successful permanently or at least over a prolonged period of time should in our opinion exist if through the intervention the part of

The development of stapes surgery after five years; mobilization--fenestra ovalis.

It is now five years since stapes mobilization has been established as a safe and practical procedure for restoring the hearing in otosclerotic deafness. I described my approach to the stapes through the external auditory canal in 1952.¹Since then, many otologists in America and abroad have used this technique in an estimated 25,000 cases of otosclerosis. <h3>Indirect Mobilization at the Neck</h3> The first technique described^1-10consists in lifting the drum out of its sulcus and folding it upward upon itself, thus bringing into view most of the middle ear cavity (Fig. 1). Working only through the external auditory canal with an ordinary ear speculum, one removes all of the bone of the posterior canal wall hiding the incus and stapes. With this exposure, the entire long process of the incus, the incudostapedial footplate, facial nerve, and round window can all be seen clearly and in great detail

Mobilization of the stapes in otosclerosis by a transtympanic technique.

Mobilization of the stapes as a method of management of otosclerotic deafness has had a checkered history. 1,2 It is just 80 years ago that Kessel failed with it, after he had previously failed to correct stapedial ankylosis caused by suppuration by removal of the drum, malleus, and incus, and as he was to fail later with total stapedectomy. Boucheron, in 1888, reported mobilization of the stapes in 60 cases, and two years later Miot reported 200 cases. Both had some good results, but in America, between 1891 and 1893, Blake, Jack, and Burnett all had such unsatisfactory results that they soon abandoned this operation in favor of total removal of the stapes. In 1900, Siebenmann flatly stated that all operations on the stapes, whether mobilization or excision, were useless. The previous year, Faraci, although he had had only limited success with mobilization, predicted that when the technique was eventually