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Bilateral congenital absence of stapes and oval window in 2 members of a family: etiology and management.

OBJECTIVE: To date, bilateral congenital absence of stapes and oval window in 2 members (brother and sister) of a family has not been reported in the literature. Carhart's notch as one of the indications for the diagnosis of this disease is proposed. The etiology and management of this condition are discussed. MATERIALS AND METHODS: The older brother and younger sister of a family had severe bilateral hearing loss and concomitant speech disability since early childhood. Their parents are first cousins and have 2 sons and 1 daughter, but the younger son is in good health with normal hearing. No similar disease was found among other relatives. Physical examination showed that the older brother and the younger sister had normal external ears and tympanic membranes. Congenital bilateral absence of stapes and oval window were confirmed by history, audiologic examination, high-resolution computed tomography scanning, and/or surgery. The modified Lempert's fenestration operation on the horizontal semicircular canal was performed on 1 ear of each patient. RESULTS: Postoperative hearing threshold was 25 dB for speech frequencies with a gain of 47 dB for the older brother and 28 dB for speech frequencies with a gain of 52 dB for the younger sister. Follow up at 5 to 13 years individually, their hearing gains were steady. Both sister and brother now enjoy a normal life. CONCLUSIONS: Bilateral congenital absence of stapes and oval window in the 2 family members may be related to inheritance. This report points out Carhart's notch as noted in otosclerosis as an important indication for diagnosis of this disease. The modified Lempert's fenestration operation of the horizontal semicircular canal is a safe and good choice for these patients and a better choice than a hearing aid throughout life.

Adolescent↗

Reparative granuloma seen in cases of gold piston implantation after stapes surgery for otosclerosis.

OBJECTIVE: to determine the occurrence of the unusual side effect of a reparative granuloma after the implantation of a pure gold piston in cases of otosclerosis. STUDY DESIGN: a retrospective case review study of 475 stapes operations with a pure gold piston. SETTING: Department of Otorhinolaryngology of the University of Amsterdam, The Netherlands and the HNO clinic in Luenen (Brambauer) in Germany. PATIENTS: four hundred and seventy five patients (328 women, 147 men, average age: 45.2 years), who clinically and per-operatively had otosclerosis, underwent a stapedotomy using a pure gold piston prosthesis. Therapeutic intervention: in cases of suspicion of a reparative granulomas or those cases that did not have improvement of the hearing after the stapedotomy, a re-operation by transcanal approach was performed. RESULTS: in seven cases a reparative granuloma was diagnosed by this revision surgery. The postoperative incidence of these granulomas following stapedotomy using the pure gold piston turned out to be 1.5%. CONCLUSION: reparative granulomas can occur after stapedotomy with a pure gold stapes piston although the incidence is low. The role of grafting material to seal the oval window niche and the treatment of these reparative granulomas following stapes surgery are discussed.

Ear Diseases↗

X-linked deafness with stapes gusher in females.

A 22-year-old woman presented with severe mixed hearing loss and a flow of cerebrospinal fluid in the middle ear during stapes surgery (stapes gusher). HRCT of the temporal bones showed characteristic abnormalities of the inner ear (bulbous dilatation of the lateral portion of the internal acoustic meatus with incomplete separation from the cochlea, and widening of the first part of the facial nerve canal) described in X-linked progressive mixed deafness with stapes gusher. The evaluation of the patient's family revealed a sister with the same clinical history and identical HRCT findings, and 11 normal male relatives. This is the first report with typical findings of this entity that affects only female members of a family, suggesting another type of inheritance.

Adult↗

Stapes-replacing prosthesis (S.R.P.).

Any chronic inflammatory condition of the middle ear may bring about bone destruction. Today, in the antibiotic era, this destruction concerns mostly the ossicular chain. Any one of the ossicles or all of them together may be partially damaged or completely destroyed (Sadé et al, 1981). Hearing is usually impaired if the stapes or the incus or both of them are damaged. To overcome the resulting hearing deficit, various surgical techniques, using various materials, have been introduced, with various degrees of success. The most problematic situation encountered surgically is the one in which the stapes is missing, and it is with this situation that the present study deals. The surgical solution so far advocated has been in the form of a surgical bridge or columella between the mobile footplate and the drum. The columella (Fig. 1) is made out of bone or some non-organic biocompatible material. Often, however, this solution is not very successful, for two reasons: A. Instability of the columella (prosthesis) is often unavoidable because of its slim attachment to the footplate; B. Biologically, an incompatibility between the drum and the prosthesis is all too often present, when the latter is made of plastic materials. These columellas have been found, sooner of later, to extrude. It is the purpose of this communication to report a new technique and a new concept, which tries to overcome the above shortcomings, using a different type of stapes-replacing prosthesis.

Hearing Loss↗

Thirty years of stapes surgery.

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. A 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.

Aged↗

Rosen mobilization of the stapes: does it have a place in modern otology?

We describe two patients with stapes fixation, in both of whom Rosen mobilization seemed to be the most prudent surgical choice. In both cases, a useful hearing gain was achieved initially. In the patient with otosclerosis, the conductive hearing loss recurred and a stapedectomy was subsequently carried out. In the patient with congenital stapes fixation, the hearing gain was maintained for two years eight months. We believe that this technique still has a place in stapes surgery in rare instances.

Adolescent↗

Robot-assisted stapedotomy: micropick fenestration of the stapes footplate.

OBJECTIVE: Micropick fenestration of the stapes footplate, a difficult step in stapedotomy, was selected for trials evaluating the potential for robotic assistance (RA) to improve clinical measures of surgical performance. STUDY DESIGN: In a surgical model of stapedotomy, we measured accuracy of fenestration to a desired point location and force applied to the stapes footplate. Performance variables were measured for 3 experienced and 3 less-experienced surgeons. RESULTS: RA significantly reduced the maximum force applied to the stapes footplate. For fenestration targeting, RA significantly improved accuracy for less-experienced surgeons and significantly worsened targeting for more-experienced surgeons. CONCLUSIONS: RA significantly improves performance for micropick fenestration in a surgical model of stapedotomy. For certain tasks, RA differentially affects performance for users of different experience levels. CLINICAL SIGNIFICANCE: These are the first results showing quantitative improvements in performance during simulated ear surgery using RA and differential effects of RA on performance for users of different experience levels.

Clinical Competence↗

Autosomal dominant stapes ankylosis with broad thumbs and toes, hyperopia, and skeletal anomalies is caused by heterozygous nonsense and frameshift mutations in NOG, the gene encoding noggin.

Although fixation of the stapes is usually progressive and secondary to otosclerosis, it may present congenitally, with other skeletal manifestations, as an autosomal dominant syndrome-such as proximal symphalangism (SYM1) or multiple-synostoses syndrome (SYNS1), both of which are caused by mutations in NOG, the gene encoding noggin. We describe a family that was ascertained to have nonsyndromic otosclerosis but was subsequently found to have a congenital stapes ankylosis syndrome that included hyperopia, a hemicylindrical nose, broad thumbs and great toes, and other minor skeletal anomalies but lacked symphalangism. A heterozygous nonsense NOG mutation-c.328C-->T (Q110X), predicted to truncate the latter half of the protein-was identified, and a heterozygous insertion in NOG-c.252-253insC, in which the frameshift is predicted to result in 96 novel amino acids before premature truncation-was identified in a previously described second family with a similar phenotype. In contrast to most NOG mutations that have been reported in kindreds with SYM1 and SYNS1, the mutations observed in these families with stapes ankylosis without symphalangism are predicted to disrupt the cysteine-rich C-terminal domain. These clinical and molecular findings suggest that (1) a broader range of conductive hearing-loss phenotypes are associated with NOG mutations than had previously been recognized, (2) patients with sporadic or familial nonsyndromic otosclerosis should be evaluated for mild features of this syndrome, and (3) NOG alterations should be considered in conductive hearing loss with subtle clinical and skeletal features, even in the absence of symphalangism.

Abnormalities, Multiple↗

Incus and stapes footplate simulator.

Prosthesis placement in stapes surgery is difficult to master. Although temporal bone dissection is an important adjunct to operative experience and anatomic knowledge when training residents to perform this procedure, the high cost and scarcity of temporal bones available for teaching purposes limit their convenience as teaching tools. Therefore, to augment training of residents, the authors developed an inexpensive, easily constructed middle ear simulator made from a disposable drinking cup, toothpicks, and a tongue depressor. The device is used with a microscope and ear instruments to manipulate, place, and crimp stapes prostheses. Improved surgical skills can lead to better surgical results, and this middle ear simulator can help to improve otologic surgical skills by giving residents the opportunity to practice techniques necessary to master stapes surgery.

Ear Ossicles↗

Ossiculoplasty with an intact stapes: superstructure versus footplate prosthesis placement.

OBJECTIVES/HYPOTHESIS: Many techniques and prostheses are used for ossiculoplasty in the various situations of ossicular chain status that the surgeon may encounter in tympanoplasty procedures. The current study seeks to determine whether any particular technique or prosthesis used by the author demonstrates superior hearing results with fewer failures. STUDY DESIGN: Retrospective review. METHODS: This study retrospectively reviews a consecutive series of cases with intact stapes superstructures, with an intact malleus handle (Austin-Kartush group A) and without (Austin-Kartush group C). The ossiculoplasties were performed with either partial prosthesis techniques from the stapes superstructure to the drum, malleus, or malleus/drum (as is traditionally reported by most authors) or with less frequently advocated total prosthesis techniques from the stapes footplate to the drum, malleus, or malleus/drum. RESULTS/CONCLUSIONS: Of cases for which 1-year postoperative air and bone hearing results were available, group A cases had significantly smaller air-bone gaps than group C cases. There was no difference in mean air-bone gaps between partial and total reconstruction within either group A or C. A few partial and total prosthesis types had low failure rates, good mean air-bone gap hearing results, and tight spread of results to recommend them in group A cases. Total reconstruction had a lower prosthesis failure rate than partial reconstruction in group C cases, although mean air-bone gap hearing results were the same.

Bone Conduction↗

Stapes surgery in osteogenesis imperfecta: results of a new series.

OBJECTIVE: To evaluate the results of stapes surgery in osteogenesis imperfecta. STUDY DESIGN: Audiological and clinical analysis of the results of stapes surgery in a consecutive series of 15 ears in 13 patients with osteogenesis imperfecta. SETTING: Tertiary referral center. RESULTS: Stapedotomy was successful in 11 of 12 ears in 11 patients with osteogenesis imperfecta type I. Air-bone gaps were within 10 dB in four ears and within 20 dB in the seven remaining ears. In one ear, revision surgery with malleovestibulopexy was needed to achieve this result. In another ear, the initial good result within 20-dB air-bone gap decreased to 28 dB; therefore, it is not considered as a success. In one other patient, revision surgery resulted in only marginal improvement in the hearing level. In one case of, probably, osteogenesis imperfecta type III, surgery resulted in a dead ear. CONCLUSION: Stapedotomy successfully improved the hearing of patients with osteogenesis type I. These results are in accordance with those reported in a few previous series. It is questionable whether stapes surgery will be as successful in osteogenesis imperfecta types other than type I.

Adult↗

Malformation of stapes in VACTERLS association: a histopathologic study and three-dimensional reconstruction.

OBJECTIVE: To investigate the temporal bone with a malformed stapes in VACTERLS association. STUDY DESIGN: The study design was a case report, histopathology of the temporal bone, and three-dimensional reconstruction of the ossicles and cochlea. SETTING: The study was carried out in the Temporal Bone Bank at Charles University, Hradec Králové, on a case from Temporal Bone Foundation, Boston. PATIENT: A 1540-g, 41-cm female neonate with VACTERLS association, histopathology of the temporal bone, and three-dimensional reconstruction of the middle ear ossicles and cochlea. RESULTS: The posterior part of the footplate was missing, and the posterior crura of the stapes was not connected to the footplate but found freely suspended in the sinus tympani. CONCLUSION: Computer-assisted three-dimensional reconstruction was performed from histologic sections of the temporal bone removed from a case of VACTERLS association. The reconstruction included structures such as the bony labyrinth and the middle ear ossicles. The abnormality observed in the reconstruction included the mislocation of the stapes and a shortened footplate.

Abnormalities, Multiple↗

Gold and titanium in the oval window: a comparison of two metal stapes prostheses.

OBJECTIVE: The objective of this study was to evaluate the hearing results of stapes surgery with 2 different full metal stapes prostheses. STUDY DESIGN: Retrospective analyses were carried out on the pre- and postoperative hearing results obtained after primary stapedotomy with 2 different prostheses. PATIENTS: We studied 106 patients with otosclerosis treated by stapes surgery. INTERVENTION: Fifty-three patients had a stapedotomy with a gold piston and 53 patients received a titanium piston after stapedotomy. MAIN OUTCOME MEASURES: The results were compared according to mean audiometric parameters. Furthermore, the individual audiologic results were demonstrated with the Amsterdam Hearing Evaluations Plot (AHEP). This method is a visual presentation of the hearing result for each operated ear. RESULTS: These AHEPs showed that the heavier gold piston (shaft Ø 0.4 mm and weight 10192.0 microg) gives more overclosure gain (28.3%) than the lightweight (shaft Ø 0.4 mm and weight 2541.0 microg) titanium piston (9.4% cases of overclosure). CONCLUSIONS: Because of the different properties of the 2 pistons (gold very malleable, titanium rather stiff), a choice can be made for different anatomic or pathologic situations in the operated middle ear. The "overclosure effect" of the gold piston is higher compared with the titanium piston.

Adult↗

Measles virus prevalence in otosclerotic stapes footplate samples.

HYPOTHESIS: The cause of otosclerosis is still unknown. Persistent measles virus infection of the otic capsule is supposed to be one of the etiologic factors in otosclerosis. Chronic viral antigen expression on the surface of infected cells can induce a secondary autoimmune reaction against the otic capsule. BACKGROUND: In the past 15 years, some reports proposed the possible etiologic role of measles virus in otosclerosis. The presence of measles virus was shown in otosclerotic patients by reverse-transcriptase polymerase chain reaction amplification of the viral RNA, detecting the viral proteins by immunohistochemistry and detecting antimeasles immunoglobulin G in the perilymph samples. Many concerns were elicited by these results. METHODS: Nucleic acid was extracted from pulverized, frozen stapes footplate samples of otosclerotic patients. Measles virus RNA was amplified by reverse-transcriptase polymerase chain reaction: reverse transcription and the first round polymerase chain reaction amplification was performed by heat stable recombinant Thermus thermophilus polymerase, whereas in the nested round, polymerase chain reaction Taq-polymerase was used. Measles virus nucleoprotein RNA-specific oligonucleotide primers were used in these reactions. An Edmonston-type measles virus served as a positive control and cortical bone fragments or stapes superstructures served as negative controls. RESULTS: Among 34 otosclerotic patients, 20 stapes footplate samples contained measles virus RNA. Measles virus RNA was not detected in other bone specimens of the patients. CONCLUSION: The etiologic role of measles virus in the pathogenesis of otosclerosis should be considered. The 14 negative samples may be genetically determined otosclerotic cases.

Adult↗

Comparison of titanium and Robinson stainless steel stapes piston prostheses.

OBJECTIVE: Although stainless steel stapes prostheses have generally been considered magnetic resonance imaging safe, there is concern that this may change with the development of more powerful imaging systems. The objective of the study was to determine whether a titanium piston stapes prosthesis would be audiometrically and surgically equivalent to a Robinson stainless steel piston for stapedectomy. STUDY DESIGN: Retrospective chart review. SETTING: Private otology practice. PATIENTS: In all, 50 patients underwent stapedectomy with a Gyrus titanium piston prosthesis. These patients were matched on the basis of age and preoperative bone-conduction scores with patients who underwent stapedectomy with a Robinson stainless steel piston prosthesis. MAIN OUTCOME MEASURES: Audiometric results are analyzed, and surgical complications noted. RESULTS: There was no significant difference between groups in hearing improvement or postoperative air-bone gap. The mean four-frequency hearing improvement was 27.7 dB for the stainless steel group and 27.8 dB for the titanium group. The mean postoperative air-bone gap was 2.65 dB for the stainless steel group and 2.60 for the titanium group. Neither group had a surgical complication. CONCLUSION: The titanium stapes prosthesis is a good alternative to a stainless steel prosthesis.

Adult↗

Stapes surgery: the diameter of the long process of the incus.

HYPOTHESIS: The aim of this study was to examine the long process of the incus in respect of its shape and its dimensions at the site of the attachment of a stapes prosthesis. BACKGROUND: One of the complications in stapes surgery is the erosion of the long process of the incus at the site of the attachment of the prosthesis, resulting in a fluctuating conductive hearing loss. Knowing the dimensions of the attachment site of the prosthesis at the long process of the incus will make it possible to optimize the size of the prosthesis loop. METHODS: The incus was obtained from 11 patients who had undergone middle ear surgery for hearing improvement or cholesteatoma removal. The ossicles were kept in 4% paraformaldehyde and were processed for histological examination after decalcification in ethylenediamine tetra-acetic acid. The 5-microm slices were stained with toluidine blue and examined in the light microscope. Using digitized video images of the histological slices, the diameters, circumference, and surface of the specimens were determined. RESULTS: The diameter of the long process of the incus at 1.4+/-0.28 mm from the tip, which is the average site of prosthesis attachment, showed an oval shape with a minimum diameter of 0.66+/-0.05 mm, a maximum diameter of 0.81+/-0.1 mm, and a circumference of 2.46+/-0.23 mm. CONCLUSION: The loop of a stapes prosthesis should have the following dimensions: diameter 0.9 mm, loop length of 2.2 mm, and opening of loop 0.7 mm. However, the material of the prosthesis and its malleability are also important factors.

Adolescent↗

The learning curve in stapes surgery and its implication to training.

OBJECTIVE: To identify the stapedotomy learning curve of two U.K. otolaryngologists. STUDY DESIGN: A retrospective review of the outcome of first 100 stapedotomy operations by each surgeon. Included in the study was a postal survey of the incidence of stapes surgery by U.K. otolaryngologists. SETTING: Two tertiary referral centers. PATIENTS: All ears in which primary stapedotomy was performed for otosclerosis. Nonotosclerotic cases and malleus stapedotomy cases were excluded. INTERVENTION: One surgeon used the technique of small fenestra stapedotomy with either a Teflon-wire or titanium piston but without vein graft interposition, whereas the second used the technique of stapedotomy with vein graft interposition and a Teflon piston. MAIN OUTCOME MEASURES: A moving average with a window of 15 dB was used to plot learning curves for the postoperative air-bone gaps. Using a postoperative air-bone gap of 20 dB or better as a definition of 'success,' the success rates with the increase in surgical experience of both surgeons were plotted on graphs, the learning curves. The end point of the learning curve was defined as the point where the curve reached its peak, and the results were sustainable. RESULTS: There was no clear-cut end point in both learning curves, although it appears that there is a landmark point at 60 to 80 cases for both surgeons. Both surgeons also had one "dead ear" in their first 15 cases. The postal survey showed that some trainers only performed small numbers of stapes surgery, whereas some otolaryngologists who performed stapedotomies regularly were not trainers. CONCLUSIONS: The study supports a learning curve in stapes surgery. To maximize the training opportunity of trainee surgeons, it may be advisable for learning centers to form network to provide target training for the trainee who has demonstrated the necessary dexterity and temperament of an otologist.

Adult↗

Homograft stapes in middle ear surgery.

Descriptions are given of various uses of stapes homografts in tympanoplasty on ears with missing stapedial arch. The use of homograft stapes-incus assembly, placing the head of the homograft stapes on the patient's own footplate and on top of that an incus, is a method which at long sight has proved to afford better results than the use of an incus columella.

Ear Ossicles↗