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Ectopic stapes: a case report with embryologic correlations.

A case report of unilateral congenital stapes misplacement revealed by computed tomography is presented. In addition to this malformation, the malleus was synostosed to the middle ear roof. This kind of stapes ectopia has not been described previously. We have analyzed the malformative pattern in the light of normal and teratologic development of the stapes. In a teratologic model in which retinoic acid is administered to pregnant mice, we have observed an ectopic stapes primordium independent of the otic capsule. We discuss the possible pathogenesis of this abnormality in terms of the genetic events of middle ear development, which can be perturbed by retinoic acid administration.

Adult↗

Clinical consequences of feedback on ear surgery: the continuous recording of adverse events and complications with regard to reducing the number of surgeons who perform otosclerosis surgery.

Electronically stored data may be used to generate feedback overviews. This paper describes a method for establishing a picture of ear surgery complications. In this prospective study, the working definition of adverse events and complications is "incidents that are not intrinsic to the surgical procedure and that have a potential or actual negative effect on surgical outcome or postoperative morbidity". A simple method is used to categorise otologic adverse events and complications. This scale varies from adverse events (grade A) to death (grade D). All adverse events and complications in ear surgery that met this definition were documented electronically as part of continuous follow-up between 1 July 1992 and 30 June 1999. In the first 3 years, 1,009 ear operations were performed, and in 51 (5%) of them, adverse events or complications were noted during or after surgery. There were 30 (3%) grade A (adverse events), 18 (2%) grade B (minor complications), 3 (0.3%) grade C (major complications) and no grade D complications. Otosclerosis surgery was evaluated additionally, because halfway through a 7-year period, the number of surgeons was changed. Only three experienced, senior members of staff were involved, and inexperienced residents no longer were allowed to perform this kind of surgery. The reduction of surgeon numbers did indeed improve the outcome of the stapes surgery. Our results were comparable to the literature. This monitoring of outcome-results in relation to changes in care can be seen as a study of care quality. A review of this kind links daily clinical practice to the literature and induces an improvement in quality.

Adolescent↗

Otoacoustic emissions: a new tool for monitoring intracranial pressure changes through stapes displacements.

Changes in hydrostatic intracranial pressure (ICP) are thought to be transmitted to cochlear liquids, thereby altering the mechanical load on the stapes footplate. Hence the stiffness of stapes' annular ligament is expected to reflect ICP changes. Corresponding middle-ear transmission changes have been assessed using click-evoked otoacoustic emissions (EOAE) in two experiments. The first one was performed in 22 normal human subjects submitted to posture changes. In the second one, controlled ICP increases were applied to 18 patients in neurosurgery operating theater. EOAEs were monitored in these subjects throughout the experiments and their phases and amplitudes were analyzed as a function of frequency. ICP affected EOAEs in a systematic manner in both experiments, i.e., the main effect of ICP increase was phase lead of EOAE components below 2 kHz without any significant amplitude or frequency shift. Their variations were much more evident than the modifications in middle-ear impedance assessed by standard impedance or admittance measurements. The second experiment led to a quantitative linear relationship between ICP and EOAE phases. It demonstrated that the sensitivity of EOAE phase monitoring technique proves to be quite high, i.e., 55 daPa increases in ICP are readily detected in all subjects. The results are consistent with the predictions of classical middle-ear models as to the transmission alterations induced by ICP-related changes in the stiffness of the annular ligament of the stapes. Non-invasive monitoring of ICP in patients with hydrocephalus treated with ventriculo-peritoneal shunts might be attempted with this technique, although it is restricted to patients with detectable EOAEs (i.e., about 50% in this series).

Acoustic Stimulation↗

Hyaluronate gel stapedotomy.

OBJECTIVE: To evaluate hearing results and postoperative dizziness after stapedotomy with application of sodium hyaluronate gel (HG) to the oval window niche during fenestration of the footplate. STUDY DESIGN: Case-control study comparing 27 stapedotomies with HG and 32 stapedotomies without HG. OUTCOME MEASURE: Preoperative and postoperative audiometric data, postoperative dizziness (vertigo or dysequilibrium), and nystagmus. RESULTS: Postoperative hearing results were not statistically different for the 2 groups. There were no cases of postoperative sensorineural hearing loss greater than 10 dB. Early postoperative dizziness (chi(2) = 4.08, P = 0.043) and nystagmus (chi(2) = 5.05, P = 0.024) were reduced in the HG group. CONCLUSIONS: HG stapedotomy is safe and effective and results in less early postoperative dizziness and nystagmus when compared with the standard technique of stapedotomy. The application of HG to the oval window niche to prevent blood from entering or perilymph from escaping the vestibule during fenestration of the stapes footplate may contribute to a quicker recovery in outpatient otosclerosis surgery.

Adjuvants, Immunologic↗

KTP laser fine fenestra stapedotomy with vein graft interposition in the surgical management of otosclerosis.

Otosclerosis is an hereditary disease of bone derived from the embryonic otic capsule. The exact aetiopathogenesis remains unclear. It can be associated with significant disability due to hearing impairment. Medical and surgical treatments, with varying degrees of effectiveness and complication rates, are available. The surgical method for the correction of the conductive hearing loss associated with otosclerosis continues to undergo technical refinement with the aim of improving results and minimising complications. The surgical technique of fine fenestra stapedotomy is well established. Here, we describe a new modification of this technique involving the use of a KTP laser. Based on the retrospective review of the results and complication rates of over 200 consecutive cases, we would like to recommend the technique of KTP laser fine fenestra stapedotomy in the surgical management of otosclerosis.

Humans↗

Familial stapes superstructure fixation.

Congenital stapes superstructure fixation was found in a mother in both ears and her daughter in one ear. The head of the stapes was fixed to the pyramid by a bony bar which lay immediately superior to a normal stapedius muscle. Removal of the bony bar resulted in normal hearing, which has been maintained. We believe that these are the first reported cases of this abnormality.

Adult↗

Comparison of argon and CO2 laser stapedotomy in primary otosclerosis surgery.

PURPOSE: The primary use for the laser in otosclerosis surgery is to create a stapes footplate fenestration that obviates the need for mechanical footplate removal. Experimental studies that evaluate the potential safety of visible (argon and potassium-titanyl-phosphate [KTP]) and invisible (CO2) light laser systems in stapes surgery report conflicting results. The purpose of this study is to compare the clinical safety and efficacy of the CO2 and argon laser systems when used for primary laser stapedotomy. MATERIALS AND METHODS: A retrospective case review of 124 primary laser stapedotomies using either the argon (n = 59) or CO2 (n = 65) laser was performed. Data consisted of pre- and postoperative air and bone conduction audiometry, speech discrimination scores (SDS), intraoperative findings, and postoperative complications. Between group differences (argon v CO2) were sought using standard statistical methodology. RESULTS: The argon and CO2 laser groups were comparable with regards to age, sex, preoperative air-bone gap, and laterality. Mean preoperative air and bone conduction pure-tone average (PTA) and SDS were somewhat higher in the CO2 laser group (P < .05). Postoperatively, both groups showed similar results in mean change in air conduction PTA, air-bone gap, and SDS, as well as in the frequency of complications. There were no anacoustic ears in either group. CONCLUSIONS: The results suggest that the argon and CO2 laser systems are comparable with regards to safety and efficacy when used by experienced surgeons for stapedotomy.

Adult↗

[Local antibiotic administration decreases risk of inner ear damage in effodation].

BACKGROUND: Mobilising the stapes via the removal of the tympanosclerotic plaques from the oval window niche (effodation) and stapedectomy or malleovestibulopexy are the different procedures generally available for the surgical therapy of stapes fixation due to tympanosclerosis. These techniques bear a significant risk of sensory hearing loss. Here we analyse our results using the mobilisation technique together with locally applied antibiotics. PATIENTS: Nineteen ears in seventeen patients with tympanosclerosis involving the stapes and its footplate which underwent stapes mobilisation between 1991 and 1999 have been investigated retrospectively. According to the literature this operation has a high risk of cochlear hearing loss. To reduce this risk, azlocillin was instilled locally during removal of tympanosclerotic plaques. RESULTS: Different operation techniques have been used: classic type III with placement of a cartilage disc on the head of the stapes (4), interposition of the incus (3), interposition of the head of malleus (1), interposition of a ceramic-PORP (6) and cartilage columella in cases of significant stapes footplate erosion (3). In two operations the chain was intact and no reconstruction was necessary. Pure-tone-audiometry showed no significant decrease of bone-conduction thresholds. Preoperatively 4 (21.1%) ears had an average air-bone-gap < or = 30 dB, while postoperatively 15 (78.9%) ears had this level of hearing. CONCLUSIONS: Until the exact causes of the loss of hearing after mobilisation or stapedectomy in cases of tympanosclerosis are known, the local administration of antibiotics is certainly recommended, bearing in mind the initial hypothesis that infection may be jointly responsible for cochlear hearing loss on mobilisation or stapedectomy in cases of tympanosclerosis.

Adult↗

Nonotosclerotic ankylosis of the stapedial footplate.

A woman with a long history of chronic otitis media had a stapedectomy after a surgical diagnosis of otosclerosis. In the histologic study of the temporal bones, done after her death four years later, the main portion of the stapes footplate was present but fractured and no otosclerosis was present. New celluluar bone which simulated otosclerosis filled the anterior oval window niche and was continuous with the middle ear surface of the footplate fragments. The new bone extended anteriorly to overlie a tympanosclerotic focus and inferiorly was continuous with the periosteum of the middle ear. At least part of the new bone formation occurred after stapedectomy. This may explain the poor long-term results frequently encountered when a stapedectomy is done on patients with tympanosclerotic fixation of the stapes footplate.

Diagnosis, Differential↗

Teunissen-Cremers syndrome: a clinical, surgical, and genetic report.

OBJECTIVE: To describe clinical and radiologic features, results of ear surgery, and genetic analysis in three families with Teunissen-Cremers syndrome. DESIGN: Case series. SETTING: Tertiary referral center. BACKGROUND: The NOG gene encodes the protein noggin, which has antagonist action in osteogenesis. Malformation of bones and joints may result from defects in noggin. Teunissen-Cremers syndrome is caused by mutations in the NOG gene. Two mutations in this gene were reported previously. The proximal symphalangism-hearing impairment syndrome, also caused by mutations in the NOG gene, is characterized by proximal symphalangism, conductive hearing loss, and occasionally synostoses. METHODS: We examined nine affected members of three Dutch families. Reconstructive middle ear surgery was performed in five patients (nine ears), and we sequenced the NOG gene in these families. RESULTS: Affected members had conductive hearing impairment, hyperopia, and broad thumbs and first toes with brachytelephalangia. Surgery manifested stapes ankylosis with additional incudal fixation frequently in the fossa incudis. Air-bone gaps decreased to less than 10 dB in six ears. Genetic analysis revealed three new mutations in the NOG gene. CONCLUSION: The Teunissen-Cremers syndrome is an entity in its clinical presentation, distinct from other syndromes with proximal symphalangism and hearing impairment. So far, in five families with Teunissen-Cremers syndrome, four truncating mutations and one amino acid substitution were found in the NOG gene. The majority of other mutations found in this gene are missense mutations, which might result in some residual protein activity. Reconstructive middle ear surgery is an option for treatment.

Abnormalities, Multiple↗

Shape-memory stapes prosthesis for otosclerosis surgery.

OBJECTIVES: The aim of this study was to determine the efficacy of a shape-memory alloy, Nitinol, as a component of an improved stapes prosthesis. STUDY DESIGN: Prospective laboratory and clinical study to develop a Nitinol stapes prosthesis. METHODS: Various diameters of Nitinol wire and temperature transition variants were analyzed with regard to ease of deformation, response to heating, and strength. The size and geometry of the closed hook was determined by measurement of 50 incus cadaver bones. Several heat sources for activating the shape memory were evaluated, including electrocautery, lasers, and warm water. Trial surgeries were then performed on human temporal bones in the laboratory. The closure characteristics of the Nitinol loop were studied. Magnetic resonance imaging (MRI) testing at 1.5 Tesla was performed to determine safety during MRI studies. Preliminary human subject trials were then instituted. RESULTS: In all cases, a low heat condition was ample to activate the shape memory characteristics of the hook and return it to a closed position after it had been opened. Laser power was generally set well below the power needed for removing bone. The Nitinol loop closed snugly around the incus with application to the top of the hook with a low temperature laser setting. Almost any heat source was effective. MRI testing at 1.5 Tesla showed no movement of the prosthesis. Preliminary results in human subjects showed excellent air-bone closure. The Nitinol loop holds uniform contact around the incus. CONCLUSIONS: The Nitinol piston greatly simplifies the stapedectomy procedure by taking the need for a hand operated instrument out of the surgeon's hands. Because of the nature of the Nitinol wire, it can never over-crimp. All these characteristics make the prosthesis advantageous for otosclerosis surgery.

Alloys↗