Congenital Stapes Fixation Research Articles

Stapedectomy in congenital stapes fixation: Are hearing outcomes poorer?

Stapedectomy in congenital stapes fixation: Are hearing outcomes poorer?

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.

Stapedectomy in congenital stapes fixation: Are hearing outcomes poorer?

To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. Retrospective chart review. The charts of 463 stapedectomies performed by 1 surgeon from 1996 to 2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate postoperative bone or air conduction data were excluded. Inclusion criteria were met for 25 patients. Operative findings and hearing outcomes were evaluated. The study group was comprised of 25 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 48% of cases, and 80% had closure within 20 dB. A gap of more than 30 dB remained in 3 cases. There were 2 cases of sensorineural hearing loss with worsening of the bone conduction thresholds by 15 dB and 30 dB. There were no instances of perilymph gusher. In addition to an excluded case that was not reconstructed because of facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. There were no facial nerve injuries, and fixation of the malleus or incus was not found in any of the included patients. When hearing outcomes were compared with our results for otosclerosis over the same time period, rates of closure to within 10 dB and 20 dB were significantly worse in the congenital group. Closure of the air-bone gap in this population differs from our results in stapedectomy done for otosclerosis. This difference likely reflects subtle anatomic variations in the congenital group that affect the effectiveness of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective method to achieve significant hearing improvement in the majority of patients. B-3b.

Abnormal facial nerve course associated with stapes fixation or oval window absence: report of two cases

Congenital minor ear malformations are very uncommon. Their etiology can be explained by the embryological development of the middle ear structures. Their classification, diagnosis and treatment pose certain problems. We report on one case of congenital stapes fixation and one case of oval window absence, both associated with an abnormal facial nerve course. Good long-term results show that performing a platinotomy or vestibulotomy in cases of congenital stapes fixation or oval window absence can be a relatively safe procedure, even in presence of deviated facial nerve course. The literature and present classifications of minor ear malformations are discussed.

Stapes fixation in children

The audiometric results after stapes surgery in children with isolated stapes footplate fixation were reason enough to analyze intraoperative findings and surgical procedures. A total of 438 stapes surgeries were performed in the years 1985-2000, and a retrospective analysis was made of the anamnestic data, intraoperative findings and audiometric data from 12 children with isolated fixation of the stapes footplate. The average age of the children when operated was 10.2 years (the youngest was 7 and the eldest 13). A loss of hearing had set in 5 years earlier. Taking the anamnestic data and the intraoperative into account, otosclerosis was found in 5 children. One child had a narrow oval window niche, in a further six children a congenital fixation of the stapes footplate was evident. Only 58% (n=7) of the 12 children operated showed acceptable postoperative hearing results. The best hearing results were found in children with juvenile otosclerosis. In three of six children with congenital stapes fixation, a worsening of both bone and air conduction was evident. The average conductive hearing loss was 30 dB at 1, 2 and 4 kHz. When explorative tympanotomy is indicated, a thin-layer CT scan of the temporal bone should be made to assess the risk of a liquor pressure labyrinth developing.

Stapedectomy in congenital stapes fixation: Are hearing outcomes poorer?

Objectives: To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. Methods: The charts of 463 stapedectomies performed by one surgeon from 1996–2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate bone conduction threshold data were excluded. Inclusion criteria were met for 22 patients. Operative findings and hearing outcomes were analyzed. Results: The study group comprised 22 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 54.5% of the cases and 77.1% had closure within 20 dB. A gap of more than 30 dB remained in 13.6% of the cases. The rate of sensorineural hearing loss was 13.6%. In addition to an excluded case that was not reconstructed due to facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. Abnormalities of the malleus or incus were not found in any of the included patients. Conclusions: We recently reviewed our stapedectomy results in otosclerosis patients and found closure to within 10 dB to be 86%. This difference in results likely reflects subtle anatomic variations in the congenital group that affect the mobility of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective technique to achieve significant hearing improvement in the majority of patients.

A novel laser-assisted stapedotomy technique for congenital stapes fixation

Objective: To investigate whether a novel laser-assisted stapedotomy technique is effective in treating congenital stapes fixation. Methods: With this surgical method, the suprastructure of the stapes is vaporized continuously from the superior half of the anterior crus to the inferior part of the posterior crus using a potassium-titanyl-phosphate (KTP) laser. The midmost point of the vaporization is the head of the stapes. This method was used to treat three patients with congenital stapes fixation (total of four ears). Results: The average hearing gain using this method was 32.2 dB, which lasted for at least 1 year. No complications were observed. Conclusions: This novel laser-assisted stapedotomy technique is an excellent method for the treatment of congenital stapes fixation.

Apert syndrome and hearing loss with ear anomalies: a case report and literature review

Acrocephalosyndactyly Type I, or Apert syndrome is a congenital disorder characterized by craniosynostosis and syndactyly. When hearing loss occurs, it is usually bilateral and conductive, often attributable to congenital stapes fixation. In determining treatment, the risk of gusher with stapedectomy becomes an important consideration. We present an adult with Apert syndrome and hearing loss, with particular emphasis on a progressive conductive component in one ear. Surgical exploration failed to reveal any definite middle ear abnormality and no stapedectomy was performed. A theory on the pathogenesis of the conductive loss in our patient follows a brief review of the literature on gusher in patients with ear malformations.

Predictive factors in pediatric stapedectomy.

The objective of the study was to investigate predictive factors in the postoperative hearing outcomes in pediatric stapedectomy. Retrospective case series. The study was performed in a tertiary academic otological practice. The outcome of 66 stapedectomies in children 17 years of age and younger were analyzed according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium guidelines. Factors evaluated included patient age, underlying diagnosis (tympanosclerosis, otosclerosis, congenital fixation), type of footplate graft and type of prosthesis used, associated ossicular abnormalities, and revision surgery. The mean postoperative air-bone gap following stapedectomy in children with tympanosclerotic footplate fixation (24.9 dB [+/-11 dB]) was significantly worse than in patients with an underlying diagnosis of congenital stapes fixation (15.7 dB [+/-9 dB]) or otosclerosis (13.1 dB [+/-3 dB]) (P =.024). Revision stapedectomy was also associated with a poorer outcome, but patient age and prosthesis and graft type did not contribute to the outcome in a statistically significant manner. Pediatric patients with stapes fixation resulting from tympanosclerosis showed poorer outcomes from stapedectomy than patients with congenital or otosclerotic fixation. Outcomes for congenital or otosclerosis fixation more nearly matched the outcomes in the literature for adult series.

A duplicated tympanic facial nerve and congenital stapes fixation: An intraoperative and radiographic correlation

A duplicated tympanic facial nerve and congenital stapes fixation: An intraoperative and radiographic correlation

カラー図説 指骨癒合症を伴う先天性アブミ骨固着症

カラー図説 指骨癒合症を伴う先天性アブミ骨固着症

合趾症および指節癒合症をともなったアブミ骨固着症の一家系

合趾症および指節癒合症をともなったアブミ骨固着症の一家系

Ｓｙｍｐｈａｌａｎｇｉｓｍを伴った先天性アブミ骨固着症の２家系５症例

Five patients in two different families were treated surgically for congenital stapes fixation. The footplate of the stapes was found to be completely fixed to the oval window in each case. Ankylosis was present in the proximal interphalangeal joints of both hands. This type of symphalangism was probably due to a maldevelopment of ossification and a lack of formation of joint apertures through the differentiating regions of cartilage. These processes were presumed to occur at about the eighth week of gestation. In such patients, stapes surgery is indicated and at the same time, all the joints in the body should be examined.

Congenital middle-ear deafness: CT study.

Computed tomography (CT) was used to study 25 patients with congenital conductive hearing loss and normal external auditory canals. Deformities were subdivided according to ossicular, fenestral, and cholesteatomatous origin. Isolated ossicular deformities were found in 14 patients (five bilateral), cholesteatoma in eight, oval-window nondevelopment (with ossicular deformity) in one, and normal studies in two (congenital stapes fixation at the level of the annular ligament). Ossicular deformities may be subdivided into incudostapedial disconnections into incudostapedial disconnections (most common), malleoincudal fixations, and stapes fixations. Most are due to developmental anomaly of the first or second branchial arch. The stapes has a dual origin (second arch and otic capsule). A cholesteatoma is defined as congenital only if there is no history of otitis and the tympanic membrane is intact. In this series, six were in the middle ear proper, and two were within the attic beyond otoscopic view. Their CT appearance, with one exception, was essentially identical to that of acquired lesions.

Otologic Manifestations of Acrocephalosyndactyly

Four patients with Apert's acrocephalosyndactyly were found to have congenital maximum conductive hearing losses. A congenitally fixed stapes footplate and perilymph gusher, suggesting abnormally patent cochlear aqueduct, was found in the ear of one patient, whose mother also had the syndrome. In another young adult Negro patient with stable, long-term hearing loss, middle ear impedance studies were consistent with ossicular fixation. In the fourth patient, a young child, the hearing loss was believed to be present since early life, and the eardrums were normal. The classic overt physical findings of this disorder and occult findings that may be associated are reviewed. Congenital stapes fixation is believed to be a frequent finding in this disorder and should be sought in all patients bearing the stigmata of acrocephalosyndactyly.

A case of the bilateral congenital malformation of the middle ear, especially of the stapes

The cases of congenital malformation of the middle ear have often been reported, but the condition limited to the stapes alone as presented in this paper, seems to be rare in literature.The patient was a girl of 13 years old whose chief complaint was hard of hearing. No abnormal finding was seen on otoscopic examination, but functional examination revealed the impairment of hearing which might be attributable to congenital stapes fixation or otosclerosis. On exploring the middle ear cavity, it was found that the stapes on each side were malformed, the left malleo-incudal joint was dislocated and a part of the right Fallopian Canal was impaired as to expose the facial nerve.Fenestration operation was performed on the left ear with remarkable improvement of hearing.The same procedure will be tried on the right ear in the near future.Review of the literature and some considerations on the development of the malformation of the case were made from the aspect of embryology.