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PubMed1999

Conductive hearing loss in Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann syndrome is a rare genetic overgrowth syndrome presenting with organomegaly, abdominal wall defects, macroglossia, and postnatal hypoglycemia. Head and neck manifestations of this abnormality include flame nevus of the forehead and characteristic sulci of the ear lobe. We present a 7-year-old child with Beckwith-Wiedemann syndrome and a rare finding of conductive hearing loss on both sides due to congenital malleus and stapedial fixation. Small fenestra stapedotomy and mobilization of malleus fixation in the epitympanum improved the child's hearing. The bony fixation of the malleus and stapes is explained as atavism of the processus anterior mallei and peripheral lamina stapedialis in embryological development.

Beckwith-Wiedemann Syndrome↗
PubMed1989

[Fixation of the malleus and incus].

During the past five years at the ENT Clinic in Olomouc 15 incudomallear synostoses of inflammatory aetiology were observed; 10 times tympanotomy was found, performed in patients without an inflammatory history, fixation of the malleus, or fixation of the malleus and incus or fixation of the stapes and malleus. The authors demonstrate three typical cases, incl. preoperative and postoperative audiograms. As during operation the ossicles are mobilized and left in situ et in function or removed by milling and destroyed and replaced by a reconstruction, the authors demonstrate a primarily fixed malleus found accidentally in the skull from medieval excavations, and histological preparations of primary incudomallear synostosis from a macerated skull in the anatomical museum.

Ear Diseases↗
PubMedDate not supplied

Clinical assessment of ossicular mobility by a ceramic vibrator designed for implantable hearing aids.

Ossicular mobility was assessed by direct coupling of a piezoelectric ceramic vibrator to the ossicles during middle ear surgery. The sites excited were body of the incus, head of the stapes, and footplate of the stapes through a hydroxyapatite ceramic strut. The threshold of the vibratory hearing was determined by the patient's response as a minimum audition, and the vibration threshold was obtained by subtracting the preoperative bone conduction threshold from the vibratory hearing threshold. The results were analyzed by the state of hearing after the operation, which revealed that a patient with a good vibration threshold during the operation had a tendency to get good postoperative hearing. This may mean that postoperative hearing can be predicted to some extent during the operation by the measurement of ossicular mobility.

Adolescent↗
PubMed2006

A new tool for testing ossicular mobility during middle ear surgery: preliminary report of four cases.

OBJECTIVE: We developed an ossicular vibration tester for the objective and quantitative assessment of ossicular mobility, which is one of the most critical factors affecting postoperative hearing after tympanoplasty. METHODS: Our device consists of three components: a probe shaft with a curved tip to be attached to the target ossicle, a vibration exciter to activate the probe, and a piezoelectric sensor to detect vibrations of the probe. These components are encased in a stainless steel holder, allowing easy hand manipulation during ear surgery. The probe is activated with an electric signal at around 1,600 Hz. The system is controlled with a laptop computer, and the results are presented as the ratio of the ossicular resistance (ROR) to a reference value as a percentage. One measurement takes 10 ms. The device was applied in four selected patients during ear surgery. RESULTS: Several measurements in two of the cochlear implantees showed a greater difference in the RORs of the stapes (15-20% in Case 1 and 35-45% in Case 2), whereas the RORs of the malleus and incus were within the same range. This was thought to correspond to the partial cochlear calcification noted in Case 2. In Case 3, who underwent surgery because of otosclerosis, the ROR of the stapes was high, ranging from 70 to 80%. When measured for the malleus-incus fixation anomaly (Case 4), the ROR of the malleus and incus was in the range of 60 to 70%. Owing to the limited surgical view, the ROR of the stapes could not be measured. No problems related to the measurements with this device were noted. CONCLUSION: The design, principles, measuring procedures, and preliminary results of our new tool for testing ossicular mobility are reported. Measuring the ossicular mobility during surgery may provide important information for deciding the surgical procedures.

Adolescent↗
PubMed2003

Reconstruction of the entire ossicular conduction mechanism.

OBJECTIVES/HYPOTHESIS: Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. STUDY DESIGN: Retrospective review. METHODS: Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. RESULTS: Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). CONCLUSIONS: Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.

Adolescent↗
PubMed1983

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↗
PubMed2004

[Surgery for tympanosclerotic stapes fixation accompanied by malleus fixation at the anterior malleus: report of 2 cases].

Most tympanosclerotic stapes fixation involves fixation or erosion of the malleus and/or incus. This status of the ossicular chain is one reason that ossiculoplasty for tympanosclerotic stapes fixation is more difficult than that for otosclerosis. In some cases, the malleus is fixed only at the anterior, while the incus is intact. In such cases, anterior spinotomy can recover mobilization of the malleus, then a prosthesis can be used for the long process of the incus during ossiculoplasty. We conducted stapedectomy with anterior spinotomy on 3 ears in 2 patients. Over 15 dB of hearing was regained in all 3 ears 6 months after surgery. No significant sensorineural hearing loss was seen in any ear. To adapt this surgical procedure, it is necessary to evaluate preoperative CT findings and the status of the ossicular chain during surgery.

Adult↗
PubMed1979

[The use of a chondro-metallic prosthesis when the ossicular chain is destroyed (author's transl)].

The author describes a new prosthesis used in functional rehabilitation when the chain is destroyed. It consists of a tripod part in gold, anchored in the attic region with its foot resting on the plate of the stapes. This prosthesis is covered with a piece of autologous cartilage freshly removed from the tragus which forms the junction with the tympanic membrane or covering fascia. 60 cases are presented, some with a follow-up of 4 years. All of the patients were suffering from the sequelae of chronic otorrhoea, with varying degress of destruction of the chain and tympanic membrane, leaving a mobile plate. Functional rehabilitation in one stage was good in 66% of cases, poor in more than 8% of cases and nil in 25% of cases. Tolerance of the prosthesis was comparable to that of other metallic or plastic materials used in the middle ear. The plate of the stapes would appear to tolerate the prosthesis perfectly since no secondary labyrinthisation was seen. The author suggests the use of this chondro-metallic prosthesis was a reliable means of ensuring functional rehabilitation in the presence of a post-otitic empty middle ear.

Cartilage↗
PubMed2002

Causes of conductive hearing loss after stapedectomy or stapedotomy: a prospective study of 279 consecutive surgical revisions.

OBJECTIVE: To analyze the causes for residual or recurrent conductive hearing loss following stapedectomy or stapedotomy and then propose surgical techniques to avoid these complications. STUDY DESIGN: Prospective study of 279 consecutive stapedectomy or stapedotomy revisions performed by the author. SETTING: Tertiary referral center. PATIENTS: The study included 260 patients who presented with a 20 dB or greater average air-bone gap in the speech frequencies 1 month to 35 years following stapedectomy or stapedotomy (19 patients were explored for possible oval window perilymph fistula). INTERVENTION: Stapedectomy (stapedotomy) surgical revision. MAIN OUTCOME MEASURES: Microscopic inspection and palpation assessed the mobility and continuity of the malleus, incus, and prosthesis. Infrared laser vaporization thinned the oval window neomembrane to identify the precise depth and margins of the oval window, the presence of residual stapes footplate, and finally, the relationship of the prosthesis to the fenestra into the vestibule. RESULTS: Prosthesis displacement out of the oval window fenestration with fixation of the prosthesis against the residual stapes footplate or otic capsule margin was demonstrated in 81% (211/260) of the patients. Of these patients, 31% had complete incus erosion, and an additional 60% demonstrated partial incus erosion, usually on the undersurface of the incus. Residual fixed stapes footplate was found in 14%, and malleus fixation in 4%. Incus dislocation was found in 4%, and incus fixation in 2%. CONCLUSIONS: Prosthesis migration and subsequent fixation caused the majority of stapedectomy failures. Collagen contracture of the oval window neomembrane lifts the prosthesis out of the oval window fenestration. Prosthesis displacement then results from adhesions pulling the prosthesis or mechanical forces further tilting the prosthesis. Incus erosion results from vibration against the fixed prosthesis. Six specific stapedotomy recommendations are made to minimize postoperative prosthesis migration.

Adolescent↗
PubMedDate not supplied

Surgical treatment of chronic middle ear disease. 1. Myringoplasty and tympanoplasty.

Results of myringoplasty or tympanoplasty were evaluated in 225 ears followed for at least one year after surgery. Repair of the tympanic membrane with an underlay connective tissue graft (fascia in 90%) was successful in 97% of the ears. One late perforation developed 3 years postoperatively. The average postoperative air-bone gap was 4.8 dB in 88 cases of myringoplasty, the series including three ears with a rigid footplate. Rigid incus and malleus should not be mobilized but subjected to resection and reconstruction. Poor tubal function caused adhesive changes in one ear (1%). In tympanoplasty the average postoperative air-bone gap was 11.3 dB in 100 ears with stapes present and 20.6 dB in 36 ears with only the footplate remaining. Of the 137 tympanoplasty ears, 10 (7%) showed prominent adhesive changes. In 36 ears with cholesteatoma there was one recurrence 3 years later (3%). An air-bone gap of less than 20 dB was postoperatively noted in 94% of the ears undergoing myringoplasty and in 69% of the ears undergoing tympanoplasty.

Ear Diseases↗
PubMed1976

The oto-palato-digital syndrome.

A boy aged ten years with oto-palato-digital syndrome is discussed. Because of severe conductive hearing loss tympanotomy was performed and abnormal poorly mobile ossicles were found. Stapedectomy was performed without improvement of hearing.

Bone Diseases, Developmental↗
PubMed1985

Tympanoplasty with calcium phosphate.

A new approach to the problems of chronic middle ear disease using the hydroxyapatite prosthesis is described. Open surgery is preferred and, independent of the eradication, reconstruction is possible. The posterior canal wall is reconstructed with a canal wall prosthesis of porous hydroxyapatite. The middle ear chain defects are reconstructed with a prosthesis of dense hydroxyapatite. In the case of a cavity with a mobile bare footplate a total alloplastic middle ear of hydroxyapatite is used.

Ceramics↗
PubMed1985

C-wire hand drill for small-hole stapedectomy.

With the four-sided point of the C-wire hand drill there is less risk of footplate mobilization during small fenestra stapedectomy. The small fenestra stapedectomy is thought to cause less trauma to the vestibule and to pose less risk of aspirating vestibular contents than the stapedectomy that totally removes the footplate.

Stapes Surgery↗
PubMed1990

Use of the argon laser in the treatment of malleus fixation.

This case demonstrates the use of the argon laser for ossicular mobilization. A preoperative audiologic evaluation revealed a severe conductive hearing loss, with a maximum air-bone gap. Since normal drilling procedures would result in a sensorineural hearing loss, the argon laser was chosen to remove a bony spur connecting the malleus to the posterior canal wall. When using the argon laser, no disarticulation of the incus and stapes is required. Postoperative audiologic evaluation revealed normal hearing sensitivity bilaterally.

Argon↗