An autosomal dominant inherited syndrome with congenital stapes ankylosis

A newly recognized autosomal dominant inherited syndrome associated with congenital conductive deafness, hyperopia, broad thumbs, broad first toes, short distal phalanges, and syndactyly is reported. The conductive loss was the result of congenital stapes ankylosis and, in two cases, was associated with ankylosis of the short process of the incus in the fossa incudis. Stapedectomy improved hearing in these patients. Fused cervical vertebrate are also an associated feature.     

Isolated congenital stapes ankylosis: an embryologic survey and literature review

OBJECTIVE: Isolated congenital stapes ankylosis is rare but is a definite entity. Both small series and case reports have been published in various languages. The aim of this study was to review the world literature regarding isolated congenital stapes ankylosis and to critically evaluate the embryonic development of the stapes to explain the possible pathologic development of this ankylosis. DATA SOURCES: All the publications in the English, German, and French literature regarding congenital stapes anomalies were reviewed, and original research articles on the embryonic development of the stapes and related structures were extensively and critically reviewed. STUDY SELECTION: Of the many varieties of congenital stapes anomalies described in the literature, only the isolated congenital stapes fixation due to footplate or suprastructure fixations were selected in this study. DATA SYNTHESIS: After extensive and critical review of the embryonic development of the stapes, the complex and confusing embryonic development is explained in a simplified way with schematic illustrations for easy understanding. The possible theories of congenital stapes ankylosis are explained on an embryologic basis and supplemented with schematic illustrations. CONCLUSION: Based on the development of the stapes an attempt has been made to explain the possible theories for the basis of suprastructure fixation. Theories of congenital fixation of footplate also discussed.     

Allograft stapes surgery for conductive hearing loss in patients with ossicular chain anomalies

An allograft stapes was used during surgical intervention for conductive hearing loss due to ossicular malformations in 11 ears of seven patients. The external auditory canal and tympanic membrane were normal in all ears. The surgical findings for the ossicles were stapes fixation (7 ears), stapes fixation with discontinuity of the incudostapedial joint (in both ears of one patient), a deformed incus and stapes crura compressed by the facial nerve (one ear in which the stapes was not fixed) and an anomaly of the incus and stapes combined with a dermoid cyst (in one ear in which the stapes was also not fixed). In all ears, the following procedures were performed: stapedectomy followed by sealing the oval window with a vein graft and placing an allograft stapes between the oval window and the lenticular process of the incus or the handle of the malleus. The allograft stapes was placed with its capitulum on the oval window in all cases, and fibrin glue was used for stabilizing the seal and the allograft stapes. The indications for stapedectomy for conductive hearing loss due to ossicular chain anomalies and the utility of allograft stapes are discussed.     

Repair of the ossicular chain with an ionomer cement by an inadequate incus after prior stapes surgery for otosclerosis

Use of an ionomer bone cement is described for repairing the ossicular middle ear system in revision stapes surgery. The cement was used to enlarge an eroded inadequate incus to allow placement of a new stapes prosthesis. Reconstruction of the incus with this new bone cement was readily performed and hearing results after 2 years of follow-up have been promising.     

Persistent stapedial artery with stapes ankylosis

The persistent stapedial artery (PSA) is a very rare, congenital, vascular anomaly. It presents as a pulsatile middle ear mass and sometimes causes conductive hearing loss. The diagnosis of the presence of a PSA is always accidental, because it is so rare and difficult to predict. CT findings include the absence of the foramen spinosum and a soft-tissue prominence in the region of the tympanic segment of the facial nerve. The risks of surgery include facial palsy, hemiplegia caused by coagulation of the PSA, and bleeding due to injury of the carotid artery during surgery in cases of aberrant internal carotid. In this article we report a case of PSA with stapes ankylosis for which we performed malleus-stapedotomy using a Teflon wire piston. We did not coagulate the PSA. Nevertheless the PSA attached to the prosthesis, the patient presented significant improvement in hearing level and had no complaint of pulsating tinnitus. Thus, we have shown that attachment of the prosthesis to the PSA does not necessarily disturb improvement of hearing level after malleus-stapedotomy for otosclerosis with PSA. Based on our experience, many cases can be treated by stapedotomy using a prosthesis and without coagulating the PSA.     

Surgery for congenital anomalies of the middle ear with mobile stapes

Summary In a series of 104 patients with congenital middle ear anomalies operated on from 1964 to 1986, 27 cases were found in which the stapes footplate was mobile and the conductive deafness was due to an anomaly in the remaining part of the ossicular chain. In 8 cases the middle ear anomaly was caused by discontinuity of the ossicular chain owing to congenital malformation. In the other 19 ears, epitympanic fixation of the ossicular chain was observed, whether or not in combination with malformation of the stapes, incus or malleus. Ossicular chain reconstruction produced an improvement of at least 15 dB in 6 of the first 8 cases with discontinuity of the ossicular chain. The mean gain was 31 dB. Epitympanotomy with exposure of the fixed ossicle and if necessary ossicular chain reconstruction led to an improvement of at least 15 dB in 12 of the 19 ears, with a mean gain of 28 dB. Correspondence to: E. Teunissen     

Bilateral congenital ossicular chain disruption mimicking otosclerosis

A rare case of bilateral congenital ossicular chain disruption whose history and findings mimicked those of otosclerosis is reported. A 54-year-old male consulted us for slowly progressing hearing loss. Audiogram showed bilateral intermediate mixed hearing loss. The stapedial reflex was negative and the tympanogram was normal A-type. Based on the diagnosis of otosclerosis, stapes surgery was planned for the left side. Surgical findings revealed normal mobility of the stapes and a small disruption at the incudostapedial joint with connection of intact mucosa. The disruption was repaired with auto-bone columella. The patient's hearing improved after surgery. The surgery for the other side was performed successively, and similar ossicular disruption was observed. The mechanisms of findings that misled the preoperative diagnosis are discussed.     

Analysis of the best site on the stapes footplate for ossicular chain reconstruction.

Experiments were performed in 22 fresh human temporal bones to compare the relative acoustic function of three stapes footplate sites for an incus stapes superstructure replacement prosthesis (I-SRP). The three sites evaluated were the anterior, centre and posterior footplates. A new round window (RW) measurement method was used to make the comparisons. A small glass microsphere was placed in the centre of the RW as a target. A Polytec laser Doppler vibrometer was used to measure round window displacement in response to 50 pure tones between 200 and 10,000 Hz presented at 80 dB SPL at the tympanic membrane (TM). After a baseline measurement of RW displacement in the intact temporal bone, the incus was removed and a cement I-SRP (CIRP) formed between the mid-malleus handle and each of the three test footplate sites, in random order. RW displacement was again measured after placement of the CIRP at each of three sites. We found the centre site to be 3.0-7.0 dB better than the anterior site above 2,000 Hz. There were no differences between the anterior and centre sites below 2,000 Hz. The posterior site was the worst at all frequencies.     

Tympanosclerosis involving the ossicular chain: mobility of the stapes in association with hearing results

Conclusion. The preoperative bone conduction level provides not only prognostic information but also information on the mobility of the stapes in tympanosclerosis. The surgical results depend upon the stapes mobility.

Objectives. We aimed to evaluate operative findings and hearing results of tympanosclerosis involving the ossicular chain, in order to understand the pathophysiology and to establish better surgical treatment of tympanosclerosis.

Patients and methods. Between January 1998 and March 2004, 29 patients (29 ears) with tympanosclerosis involving the ossicular chain underwent tympanoplasty at our hospital. Patients with myringosclerosis only, or with an associated cholesteatoma, were excluded from this study. The clinical and operational records and pre- and postoperative pure tone audiograms were reviewed retrospectively.

Results. Intact canal wall tympanoplasty was applied to all 29 patients. A non-staged operation was performed on 21 patients, and a staged operation was performed on the remaining 8 patients. In 25 patients (86.2%), the sclerotic lesion of the ossicles was located in the epitympanum. In the remaining four, the sclerotic lamella coated only the ossicular chain. On average, the preoperative air conduction hearing level of 57.9 dB was significantly improved to 46.3 dB after tympanoplasty. The success rate of middle ear surgery was 65.5% (19 of 29 patients), according to the criteria of the Otological Society of Japan. In 16 patients (55.2%), the mobility of the stapes was preserved (group A), while in the remaining 13 patients (44.8%), the stapes was fixed (group B). The mean preoperative bone conduction of 25.5 dB in group A was significantly better than that of 37.2 dB in group B. The hearing result significantly improved in group A but not in group B. The success rates were 75% (12 of 16 patients) in group A and 53.8% (7 of 13 patients) in group B.     

Tympanosclerosis involving the ossicular chain: mobility of the stapes in association with hearing results

CONCLUSION: The preoperative bone conduction level provides not only prognostic information but also information on the mobility of the stapes in tympanosclerosis. The surgical results depend upon the stapes mobility. OBJECTIVES: We aimed to evaluate operative findings and hearing results of tympanosclerosis involving the ossicular chain, in order to understand the pathophysiology and to establish better surgical treatment of tympanosclerosis. PATIENTS AND METHODS: Between January 1998 and March 2004, 29 patients (29 ears) with tympanosclerosis involving the ossicular chain underwent tympanoplasty at our hospital. Patients with myringosclerosis only, or with an associated cholesteatoma, were excluded from this study. The clinical and operational records and pre- and postoperative pure tone audiograms were reviewed retrospectively. RESULTS: Intact canal wall tympanoplasty was applied to all 29 patients. A non-staged operation was performed on 21 patients, and a staged operation was performed on the remaining 8 patients. In 25 patients (86.2%), the sclerotic lesion of the ossicles was located in the epitympanum. In the remaining four, the sclerotic lamella coated only the ossicular chain. On average, the preoperative air conduction hearing level of 57.9 dB was significantly improved to 46.3 dB after tympanoplasty. The success rate of middle ear surgery was 65.5% (19 of 29 patients), according to the criteria of the Otological Society of Japan. In 16 patients (55.2%), the mobility of the stapes was preserved (group A), while in the remaining 13 patients (44.8%), the stapes was fixed (group B). The mean preoperative bone conduction of 25.5 dB in group A was significantly better than that of 37.2 dB in group B. The hearing result significantly improved in group A but not in group B. The success rates were 75% (12 of 16 patients) in group A and 53.8% (7 of 13 patients) in group B.     

在听骨链三维重建中对镫骨显影的体会

颞骨解剖结构复杂,中耳发生畸形及病理改变的情况较为多见,了解术前中耳的解剖结构十分重要。为更形象地显示中耳结构,常采用后处理技术对中耳结构主要是听骨链进行三维重建,锤、砧骨显影较容易,但提高镫骨的显影则需特殊处理,以便为临床医生提供重要信息并作出术前评估。     

Bilateral congenital cholesteatomas associated with ossicular anomalies: a case report

A case of bilateral congenital cholesteatomas, which occurred in a 15-year-old male was reported. His family had been aware of his hearing impairment since he was 3 years old. He had experienced neither otalgia nor otorrhea. Tympanograms were A type bilaterally, and an audiogram showed conductive hearing loss of about 60 dB on both sides. High resolution CT of a horizontal section revealed soft tissue density occupying the space medial to the body of the incus in the right ear, while the middle ear space appeared to be completely clear on the left side. The membranous cholesteatomas on both sides were accompanied by ossicular anomalies with hypoplasia of the long process of incus and the superstructure of the stapes. This case constitutes the seventh case report of bilateral congenital cholesteatomas in the literature. The literature is reviewed and the pathogenesis of congenital cholesteatomas associated with ossicular anomalies is discussed.     

Middle ear imaging using virtual endoscopy and its application in patients with ossicular chain anomaly

Virtual endoscopy (VE) is a recently developed technique to provide a realistic surface rendering of various organs, which can be applied to the use of three-dimensional (3D) studies of several lesions. However, its advantages in otological disease have not been well investigated. In this study, we evaluated the application of VE in patients with ossicular chain anomalies. Virtual middle ear endoscopy was a time-saving method, however, we needed the appropriate technical procedures of algorithm and reconstruction spacing to generate accurate 3D images of ossicles. We obtained virtual surgical views of middle ear structures and related anomalies, and confirmed by intraoperative findings that these images were mostly compatible with the actual lesions of ossicles. VE allowed an identification of the anatomy of the ossicles and adjacent structures simultaneously. The elements of the stapedial crura were clearly visualized with VE images in 93.3% of normal ears. Pathological ossicular chain findings such as malleus or incus fixation, dislocation and disruption, except footplate fixation were investigated successfully. One possible procedure, using alterable CT value in the obtained VE images on the monitor, is proposed for further detection of fine lesions of the ossicles. These observations suggest that virtual middle ear simulations accurately represent major intraoperative findings. This technique may have an important role in preoperative planning, surgical training, and/or postoperative evaluation in otology.