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Clinical features of female heterozygotes in the X-linked mixed deafness syndrome (with perilymphatic gusher during stapes surgery).

A new pedigree with 9 obligate and 10 probable female carriers of the X-linked mixed deafness syndrome is presented. In the male the syndrome is characterized by a perilymphatic gusher during stapes surgery, a severe progressive mixed hearing loss and lack or strong reduction of vestibular responses. Four of the 9 obligate heterozygote females show similar but much milder audiological abnormalities. The other five obligate female carriers have a normal audiogram. None of the obligate female carriers showed vestibular abnormalities such as observed in the affected males. Ten females, sisters of affected males, have a chance of 50% to be heterozygote carriers. Five of these females have also a hearing loss, the other 5 have normal audiograms. The relevance of these findings for genetic counseling and for identification of affected males is discussed.

Deafness↗

Eliminating the limitations of manual crimping in stapes surgery? A preliminary trial with the shape memory Nitinol stapes piston.

OBJECTIVE: Manual piston malcrimping in stapedotomy may be the major cause of the occurrence of the significant, interindividual variations of postoperative air-bone gap (ABG), air-bone gap closures (ABGC), and postoperative recurrences of conductive hearing loss. To eliminate the effects of manual crimping on stapedotomy outcomes, the self-crimping, shape memory alloy Nitinol stapes piston was investigated and hearing evaluated. STUDY DESIGN: Prospective, preliminary case-control study in a tertiary care referral center. METHODS: Sixteen patients with otosclerosis undergoing reversed stapedotomy using the Nitinol stapes piston were matched to reference patients out of our conventional titanium piston database. The effects of the self-crimping Nitinol piston on the postoperative ABGC, the postoperative air-bone gap (ABG) variations, and the postoperative short-term hearing results were investigated 3, 6 and 9 months postoperatively. These data were statistically compared with the results of the control patients in our titanium stapes piston database. RESULTS: The mean postoperative ABG and the interindividual variations of the postoperative ABG were significantly smaller in the Nitinol group, the extent of ABGC greater in the Nitinol piston group, but not significant. The postoperative short-term stability of ABGC was similar in both groups. No infections or adverse reactions occurred during follow-up. CONCLUSION: Our preliminary results suggest that the self-crimping shape memory alloy Nitinol stapes piston eliminates the limitations of manual malcrimping in stapedotomy, thus optimising the surgical procedure. This allows reliable, safe, and consistent air-bone-gap closure in patients with otosclerosis up to 1 year after surgery.

Adult↗

Outcome of stapes surgery for otosclerosis.

Otosclerosis is an autosomal dominant disease affecting the otic capsule. It is believed to be rare in Asian countries including Malaysia. We analyzed the results of 29 patients (35 ears) who had undergone stapes surgery for suspected otosclerosis from January 1996 to June 2002. The demographic data was analyzed. The procedure most commonly performed was stapedotomy. The closure of air bone gap after surgery was good at 2 kHz and 4 kHz compared to frequency below 2 kHz. Closure of air bone gap to within 10 dB was achieved in 74.3%. About 7.5% of our patients did not gain in average air conduction. The outcome of the procedure is comparable to those reported by authors who used the same evaluation criteria. No serious complications occurred in our patients.

Adult↗

[Postoperative complications in stapes surgery. An analysis of medical and economic aspects].

With the establishment of DRGs in the German hospital financing system, knowledge of therapy costs and the suitability of therapies for reservation planning and medical cost management becomes important. Stapes surgery and its postoperative treatment is relatively standardised. The timing of intervention can be planned and serious complications are rare. We carried out a retrospective evaluation of all stapedectomies performed in Bochum University ENT department over 1 year ( n=46). The course of treatment and differences in expenditure between standard and complicated cases were recorded. We used the official calculation model of the Deutsche Krankenhausgesellschaft and the homogeneneity of the investigated patient groups was evaluated statistically. A total of 6.52% of all patients had complications, mainly temporary inner ear hearing deficit and vertigo, resulting in additional therapy. The average cost of a stapedectomy was 964 EUR for regular cases. The costs if complications occurred ranged from 1,754 EUR to 3,504 EUR. Statistical evaluation showed a strong homogeneity in the group of patients without complications, especially for the costs of surgery. Stapedectomy is suitable for flat rates due to the standardisation of therapy and the homogeneity of the patients. The profitability depends on the future DRG-flat-rate. Stapedectomy surgery seems to be appropriate for resource and cost management. To avoid unfair future losses for the hospitals, a mechanism of compensation for very expensive medical complications should be developed.

Adolescent↗

Safety of gold in stapes surgery.

Gold prostheses in middle ear surgery were found to have a higher extrusion rate than prostheses made from titanium. Incidences of deafness after insertion of a gold piston into the vestibule during stapes surgery have been observed. The aim of this study was to find out to what degree gold cations can diffuse from the prosthesis piston into the perilymph. For this, gold prostheses were incubated in artificial perilymph for four months, after which the gold content of the perilymph was analysed. As gold exhibits a special behaviour in complex fluids like the perilymph, a new analysing method was developed. The results show that gold does leak out of the pistons, that it can be reliably measured and that the amount of gold reaching the perilymph depends on the contact area. As the amount of gold measured in the perilymph stays far below the toxic level, it is very unlikely that the gold cations diffusing from a stapes prosthesis into the perilymph have a toxic effect on the inner ear hair cells. Inflammatory or allergic reactions to gold induced by direct tissue contact, however, cannot be excluded.

Animals↗

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↗

[Imaging of postoperative failures and complications in stapes surgery for otosclerosis].

Otosclerosis (OS) is a dysplasia of the otic capsule located in most cases on the anterior margin of the oval window or fissula ante fenestrum. Progressive conductive hearing loss is the major clinical symptom, due to stapedovestibular ankylosis. Stapes surgery is the only effective treatment of OS, with excellent functional results in more than 90% of cases. However, failures and complications of the surgery may be observed. In theses cases, the etiologic work-up includes imaging evaluation (CT and MRI). Imaging findings are extremely useful in the therapeutic decision. Surgical failure represents 80% of the causes for surgical revision. The main causes of failure are: displacement of the prosthesis, fibrosis of the oval window, erosion of the long process of the incus, incudo-mallear dislocation, obliterative otosclerosis. CT is essential for diagnosis. MR imaging is rarely indicated in the work-up of surgical failures. Labyrinthine complications account for less than 20% of surgical revisions. Etiologies of labyrinthine complications are: intravestibular penetration of the prosthesis, perilymphatic fistula, intra-vestibular granuloma, labyrinthitis and intravestibular bleeding. CT and MRI are complementary for the work up of these complications.

Adult↗

Long-term follow-up of tinnitus in patients with otosclerosis after stapes surgery.

We prospectively studied 48 otosclerosis patients over a 2-year interval. Forty-four had stapedotomy, and four had stapedectomy. Demographics, clinical history, and pre- and postoperative audiometry results were recorded. A questionnaire asking about tinnitus (intensity, pitch) and including a visual analog scale quantifying the degree (1-10) of annoyance caused by the tinnitus was completed pre- and postoperatively (4-10 months and 14-48 months, respectively). Ninety-one percent of the otosclerosis patients reported the presence of tinnitus, and 39.58% reported severe disabling tinnitus (SDT; 7-10 on the visual analog scale). High-pitch (whistle) tinnitus was present in most patients, and white noise (radio static, waterfall, rain) was present in others. No patient described the tinnitus as a low-tone noise (buzzing). Ninety-one percent of patients reported tinnitus reduction or total remission after surgery. Ten of 19 patients with SDT reported complete remission of tinnitus, 7 reported improvement postoperatively, and 2 reported no change. No patient reported worsening of tinnitus. Small postoperative air-bone gaps (four tonal average) correlated with remission or reduction of tinnitus in SDT patients. Tinnitus pitch, gender, and age of patients were not related to decrease of tinnitus postoperatively. A larger preoperative air-bone gap correlated with larger reduction of tinnitus after successful surgery. A lower preoperative bone conduction level correlated with more intense tinnitus before surgery and greater reduction postoperatively. We contacted 25 patients from 14 to 48 months after surgery, and all reported that their tinnitus status had not changed since the early follow-up. Tinnitus is very prevalent in otosclerosis patients; almost one-half of patients have SDT preoperatively, and stapes surgery improves this symptom in 91% of cases. Preoperative air-bone gap and bone conduction level and a postoperative airbone gap seem to influence the degree of postoperative tinnitus reduction.

Adolescent↗

Pneumolabyrinth: a late complication of stapes surgery.

As temporal bone imaging techniques continue to improve, it is likely that we will see an increase in the detection of pneumolabyrinth. Several mechanisms have been proposed to explain how air enters the labyrinth. A small number of authors has reported an association between pneumolabyrinth and temporal bone fractures, perilymphatic fistulae, and displaced stapes prostheses. In this article, we describe a new case of pneumolabyrinth that was seen as a late complication of stapes surgery, and we summarize what is known about this rare condition.

Ear, Inner↗

Stapes surgery: implications for training.

The worldwide decline in surgery for otosclerosis had been reflected by a similar pattern here in Great Britain. Unless we are prepared to accept poorer results than have formerly been achieved, we will have to adopt measures to ensure that the skills of stapes surgery are transferred to the next generation of surgeons. Using the results of our experience, at the Queen Elizabeth Hospital, over the past 18 years, I have examined the implications for surgical training and for the management of future patients with otosclerosis.

Clinical Competence↗

Stapes surgery.

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Nursing↗

[Long-term results of stapes surgery--over 1000 patients].

Authors present audiometric results of 1200 patients with otosclerosis post stapedotomy. Air-conduction, bone-conduction and air-bone gap was measured prior the surgery and 12 months post stapedotomy for frequencies 0.5, 1.0, 2.0 and 3.0 kHz. In the analysed group of 1200 patients a postoperative air-bone gap 0-10 and 11-20 dB was obtained in 93.7% patients. The result of stapedotomy in reference to tinnitus based on the patients' subjective opinion showed that 78.0% patients were either free from tinnitus or tinnitus was subjectively decreased. Authors stress that some anatomical abnormalities in the tympanic cavity might influence on the result of stapes surgery.

Audiometry, Pure-Tone↗

The knotted wire in stapes surgery: one possible factor in postoperative sensori-neural hearing loss.

In four instances of revision stapedectomy in patients with sensori-neural hearing loss, with or without dizziness, a wire prosthesis was found with an extention beyond the knot which could impinge on the contents of the vestibule. Four commercially manufactured knotted wire prostheses were examined, and the end of three was found projecting beyond the knot. The wire piercing the membranous labyrinth appears to be one of the factors in causing further sensori-neural hearing loss after stapedectomy. A wire loop or piston prosthesis should be considered to eliminate the hazard of a trailing end of a knotted wire in stapes surgery.

Adult↗

The effect of preoperative bone conduction threshold levels on the results of stapes surgery.

The effect of preoperative bone conduction (BC) threshold levels on the results of stapedectomy was evaluated in 378 adult patients with otosclerosis. The mean follow-up period was 8.8 years. Both short-term and long-term postoperative hearing levels were found to be significantly dependent on preoperative BC levels. Only 2 of 31 patients (7%) with preoperative BC thresholds > 40 dB had postoperative hearing levels of 30 dB or better. However, hearing still improved by > or = 21 dB as often in patients with elevated preoperative BC thresholds as in patients with normal BC thresholds. These findings support the use of stapes surgery being worthwhile even in patients with highly elevated BC thresholds.

Adult↗