[Recent surgical options for vestibular vertigo]. / Aktuelle operative Therapie des vestibulären Schwindels.

Vertigo is not a well defined symptom but a heterogenous entity diagnosed and treated mainly by otolaryngologists, neurologists, internal medicine and primary care physicians. Most vertigo syndroms have a good prognosis and management is predominantly conservative, whereas the need for surgical therapy is rare, but for a subset of patients often the only remaining option. In this paper, we describe the development of surgical therapy for hydropic inner ear diseases, Menière disease, dehiscence syndroms, perilymphatic fistulas, and benign paroxysmal vertigo. At the end, we shortly introduce the most recent development of vestibular implants. Surgical vestibular therapy is still indicated for selected patients nowadays when conservative options did not reduce symptoms and patients are still suffering. Success depends on the correct diagnosis and indication for the different procedures going along with an adequate patient selection. In regard to the invasiveness and the possible risks due to the surgery, in depth individual counselling is necessary. Ablative and destructive surgical procedures usually achieve a successful vertigo control, but go along with a high risk for hearing loss. Therefore, residual hearing has to be included in the decission making process for a surgical therapy.

Impact of endolymphatic mastoid shunt surgery on saccule and lateral semicircular canal function.

Endolymphatic mastoid shunt surgery (EMSS) is widely performed in patients with medically intractable Meniere's disease. Although many patients report an improvement of symptoms after surgery, the mechanisms which are responsible for the relief of complaints are not known. To date, only few studies exist which studied the influence of EMSS on vestibular function. The present study examines the effect of EMSS on saccule function by measuring vestibular evoked myogenic potentials and the effect on lateral semicircular canal function by sinusoidal harmonic acceleration (SHA) testing. No changes in vestibulo-collic reflexes were found after surgery compared to before surgery. SHA testing resulted in comparable phase lag and gain pre- and postoperatively. Although central compensation is clinically evident no effect in specific vestibular diagnostic testing is seen. Modulations of canal-otolith interaction might suggest a change of symptoms. The only method so far to evaluate the success of EMSS is the patient's subjective assessment.

Perineal hypospadias repair with preservation of a coincidental vagina or perineal utricle in boys with disorders of sex development.

INTRODUCTION: An enlarged utricle in patients with proximal hypospadias or disorders of sex development (DSD) is common. The utricle orifice is usually in the posterior urethra near the verumontanum, but in exceptional cases located on the perineum. Concurrence of a perineal hypospadias and perineal utricle or vagina is also known as male vagina, pseudovagina, or blind vaginal pouch. The utricle in such cases is usually excised either before or concomitant with hypospadias repair. The authors developed an alternative approach in which the vagina or perineal utricle is retained during hypospadias repair and report on the results in four patients. OBJECTIVE: To report a novel technique for perineal hypospadias repair while retaining a concurrent vagina or perineal utricle. PATIENTS AND METHODS: Between 1999 and 2014, four neonates presented with perineal hypospadias. In all patients, a second perineal opening providing access to either an enlarged utricle or a vagina was identified. Karyotype in peripheral blood was in two patients 46,XY and in the other two 45,X/46,XY of which one showed a complex mosaicism in gonadal tissue. No genetic cause was identified on DNA evaluation in the two patients with 46,XY DSD. All patients were raised as boys. Hypospadias repair was performed in two stages at prepubertal age. During the second stage of surgery, performed between the age of 1.5 and 5 years, the vagina or utricle orifice was incorporated into the neo-urethra, resulting in a retained 'built-in' vagina or utricle. RESULTS: Surgical procedures were uneventful, and patients remained asymptomatic during a mean postoperative follow-up of 8.5 (range 2-13.5) years. One patient was lost to follow-up after the age of 8 years. At their last visit, the remaining patients, at the age of 4, 15, and 17 years, were able to void in standing position without dribbling. Both adolescent patients reported erections without ejaculations and identified themselves as males without signs of gender dysphoria. CONCLUSION: Hypospadias repair in boys with perineal hypospadias while leaving a male vagina or perineal utricle in situ has not been reported previously, and the study's preliminary results are favorable. One of the benefits of this approach is that inadvertent injury to adjacent anatomic structures such as urethral sphincter, neurovascular bundles, ureters, vas deferens, and rectum is avoided. The main rationale for adopting this conservative approach however is to minimize genital tissue removal in children with a not yet definite gender identity, which will certainly facilitate unforeseen future gender reassignment surgery.

Menière symptoms induced by an otologic implant during MR.

A patient who had had sacculotomy with placement of a stainless steel sacculotomy tack 20 years earlier experienced vertigo and auditory sensations during MR imaging. The safety of these prosthesis in MR is questionable. A simple method of determining ferromagnetic interaction is proposed.

Cochleosacculotomy for the treatment of Menière's disease in the elderly patient.

Cochleosacculotomy is a surgical option for the treatment of incapacitating vertigo in elderly patients with Menière's disease. This procedure was performed in nine patients (mean age: 73 years) for control of vertigo and/or drop attacks. The average follow-up period was 29 months. Vertigo was controlled in eight of nine patients, but hearing worsened in all but one patient. No patient developed a transient increase in vertigo in the postoperative period. Cochleosacculotomy should be considered in elderly patients with recurrent vertigo and severe non-fluctuating sensorineural hearing loss who would otherwise be candidates for labyrinthectomy.

Surgical management of Ménière's disease with the endolymphatic subarachnoid shunt: A five-year study.

The majority of individuals with Ménière's Disease can be successfully managed by medical means; however, there are some patients who become refractory to this form of therapy. It is this group of patients who are candidates for some type of surgical procedure. This paper reviews 109 patients who underwent 112 endolymphatic subarachnoid shunt procedures for the control of their Ménière's Disease. Medical therapy, indications for surgery, surgical technique, results, and complications are covered in detail.

Endolymphatic sac surgery.

There is a disturbing variation in the reported success rate in Endolymphatic Sac Surgery in the literature. Relatively long-term (24 months to 10 years) follow-ups are given in a series of 66 patients in this paper. Results reveal only a 55% long-term relief of vertigo in these patients. Only 30% of this series of cases had any improvement in cochlear function. There was a definite trend towards recurrence of vertigo in the longer follow-up cases. It is concluded that Endolymphatic Sac Surgery still is important in the treatment of idiopathic endolymphatic hydrops, but is of questionable value early in the course of the disease. Total removal of the endolymphatic system remains the best single surgical approach in a large number of patients with Ménièr's disease.

Surgical treatment of chronic otitis media and Menière's syndrome.

Menière's symptom-complex in conjunction with chronic otitis media (COM) has occasionally been encountered in otologic practice. This study compares the effects of various surgical techniques on patients with COM and Menière's syndrome, primarily on incapacitating vertigo. One group of patients with incapacitating vertigo received endolymphatic system drainage procedures, either endolymphatic sac surgery (83 patients) or cochleosacculotomy (12 patients) simultaneously with surgery for COM. Another group (14 patients) had surgery for COM only. A comparison of the results for vertigo control between the two approaches indicates a significant difference in success rate in favor of endolymphatic system drainage procedures (80% versus 20%). COM is regarded as one of the causes of endolymphatic hydrops (EH). The results of this study seem to indicate both 1. the likelihood of the cause-and-effect relationship between COM and EH, and 2. the effectiveness of endolymphatic shunting procedures on control of incapacitating vertigo, thus supporting the contentions of other authors with regard to pathogenesis and sac enhancement in patients with COM and Menière's syndrome.

Otosclerosis and Meniere's syndrome: diagnosis and treatment.

Occasionally a patient with otosclerosis and a conductive hearing loss will develop typical findings of Meniere's syndrome in the involved ear, years later. A review of clinical and pathological studies in the literature and in our laboratory and clinic indicates a likely cause-and-effect relationship for these cases. The pathology and pathogenesis of the syndrome of otosclerosis and Meniere's syndrome is discussed. A stapedectomy/sacculotomy was used to treat 17 patients, 13 of whom acquired a satisfactory result in terms of improvement of hearing and control of vertigo. This technique and findings are described and discussed.

Cochleosacculotomy revisited. Long-term results poorer than expected.

Cochleosacculotomy has been described as a simple, efficacious treatment for relief of vertigo in patients with Meniere's disease in whom medical therapy has failed. We reviewed records of 11 elderly patients with good vestibular function who were thought to be ideal candidates for this procedure. Average follow-up was 17 months. Contrary to previous reports, long-term control of vertigo was poor, and more than 80% of the patients suffered a significant hearing loss from this procedure. Four of 11 patients required a second surgical procedure to control their vertigo. Audiometric measures revealed statistically significant postoperative increases in puretone thresholds at all frequencies and speech reception threshold, and a decrease in discrimination scores. Based on the results of this study, we no longer plan to use cochleosacculotomy for the treatment of elderly patients with Meniere's disease.

Treatment of endolymphatic hydrops with cochleosacculotomy. Clinical results and experimental findings.

Cochleosacculotomy was performed on 25 patients with Meniere's syndrome. Long-term relief of vertigo was obtained in 19 out of 23 (82%). Postoperatively dead ears occurred in three cases. The same operation was performed on 12 guinea pigs in which hydrops had been surgically induced by blockage of the endolymphatic duct and sac. All fistulas were healed and cochleosacculotomy did not decrease or prevent the induced endolymphatic hydrops in these animals. Although histologic confirmation of persistent fistulas in human ears is lacking, the relief of vertigo in patients may not be caused by "drainage" but, rather, by a nonspecific effect on the inner ear. Cochleosacculotomy gives results comparable with other nondestructive surgical procedures performed to suppress vertigo in Meniere's syndrome.

Ossicular fixation caused by bone dust after saccus decompression surgery.

HYPOTHESIS: To highlight the clinical and histologic features of ossicular fixation produced by bone dust during mastoid surgery. BACKGROUND: Bone dust deposition in the middle ear may occur during temporal bone surgery, but the ossicular fixation that may result from it remains underreported. METHODS: A case is reported of delayed conductive hearing loss after saccus decompression surgery resulting from ossicular fixation by bone dust deposition. RESULTS: Six months after the initial surgery, the patient experienced a 45 dB conductive hearing loss. The findings on second-look tympanotomy were stapes fixation and round window niche occlusion by newly formed tissue. Histologic examination of the latter showed fibrosis and new bone formation in reaction to particles of bone dust. New bone formation is a frequent concomitant of fibrosis in the middle ear and is probably not a growth from the bone dust. CONCLUSION: The use of high-speed drilling of bone in temporal bone surgery carries the risk of bone dust particle deposition in the middle ear. Measures are discussed that can be used to prevent this complication.

Pathology of Ménière's disease as it relates to the sac and tack procedures.

The rationale for endolymphatic sac and tack operations is to prevent the further accumulation of endolymph, the former by draining endolymph into extralabyrinthine tissues and the latter by shunting endolymph into the perilymphatic space. While the basic concepts are reasonable enough, the probability of achieving these objectives seems remote. There appears to be adequate clinical evidence, however, to show that these procedures are of therapeutic value in selected cases. It is tempting to speculate that these surgical insults to the labyrinth, with the associated inflammatory and biochemical changes, alter the function of cells which control fluid physiology and thus improve the symptoms of Ménière's disease.

Current status of surgical decompression and drainage procedures upon the endolymphatic system.

At the present time, neither the etiologic factors nor the pathophysiologic mechanisms that lead to the development of endolymphatic hydrops or Ménière's disease are known; however, the anatomic-pathologic alterations in the labyrinth that are produced by the disease are well-documented. Undoubtedly, what is not known is responsible for the controversial status and questionable benefit that surgical decompression and drainage procedures have upon the endolymphatic system.

Effect of fistulae on endolymphatic hydrops.

An experiment was performed on 31 guinea pigs to study the effect of fistulae of the horizontal canal, superior canal, common crus, and utricle on the course of experimentally induced endolymphatic hydrops. The effect of fistulae on corresponding parts of normal ears was also studied in an additional 17 animals. The results indicated a remarkable consistency of healing of the fistulae in both groups of animals. Fistulae had no significant effect on the course of endolymphatic hydrops, irrespective of whether the fistulae were made immediately before or several months after obliteration of the endolymphatic duct or whether a polyethylene strut was introduced into the membranous labyrinth. Once again, the functional significance of the endolymphatic sac was clearly apparent, for ablation of the sac consistently produced severe hydrops and atrophic changes in sensory and neural structures. Assuming that the mechanisms of labyrinth repair in the guinea pig are comparable to the higher mammalian ear, it is apparent that procedures designed to surgically fistulize the membranous labyrinth are of questionable value and are probably not rational therapeutic approaches to the management of Ménière's disease.

Cochleostomy or endolymphatic sac surgery for advanced Meniere's disease.

The operation of cochleostomy is described. The surgical technique is vital for the true assessment of results. If the cochlear duct is punctured, the risk to hearing is unacceptably high. If only the osseous spiral lamina is punctured, the results are acceptable in the short term, though the rationale cannot be explained. In the management of difficult patients with more advanced Meniere's disease, some variant of cochleostomy should probably be included in the therapeutic options. The results of endolymphatic sac surgery are significantly better in a matched group of patients.

The sacculotomy procedure.

In patients who have unilateral idiopathic endolymphatic hydrops with intractable, severe episodic vertigo and established sensorineural deafness that cannot be helped by a hearing aid, the Cody tack procedure is the conservative procedure of choice. Local anesthesia is used, morbidity is unusual, vertigo is controlled in almost 80% of patients, and hearing can be maintained or improved.

Sac surgery on the only or better hearing ear.

In a neuro-otological practice, many patients are seen with bilateral Meniere's disease. Some of these poor prognostic cases start in childhood or adolescence. Of all the conservative operations, endolymphatic sac surgery alone should be considered for the only or better hearing ear. The risk of surgical anacusis is less than 2 per cent, a risk outweighed by the significant short and long term benefit for these difficult patients. Including patients who undergo revision surgery, control of vertigo is very acceptable, and hearing gain, especially in only hearing ears, is often essential for rehabilitation. The best results probably are obtained when surgery is performed early in the course of the disease.

Laser labyrinthectomy in humans.

This paper describes the feasibility of labyrinthectomy of only the vestibular part using argon laser in humans. In animal experiments, the utricular and saccular maculae were completely destroyed using argon laser with the otoprobe of the HGM system via the oval window, following stapedectomy. The human utricular and saccular maculae can be approached in a similar manner. Several approaches to the nerves and ampullae of the anterior and lateral semicircular canals are discussed. The most appropriate method to abolish the function of the ampullae is to sever the ampullary nerves. This can be done through the widened oval window with the argon laser otoprobe. After laser application, the oval window is covered by a piece of perichondrium. A stapes prosthesis is placed on the long process of the incus. Laser singular neurectomy may be used to sever the nerve to the posterior ampulla.

Recovery of the otolith-ocular reflex after unilateral deafferentation of the otolith organs in squirrel monkeys.

The enhancement of the vestibulo-ocular reflex (VOR) gain in the eccentric rotation is mediated by the otolith organs. Functional recovery of the otolith-ocular reflex after deafferentation of the otolith organs was examined in squirrel monkeys, using the enhancement of the eccentric VOR gain as an indicator of the reflex. After unilateral deafferentation of the otolith organs, the enhancement of the eccentric VOR gain decreased and then recovered completely within eight weeks. However, the eccentric VOR gain was not enhanced after contralateral side lesions. These findings demonstrate that functional recovery of the otolith-ocular reflex is achieved after unilateral deafferentation of the otolith organs, and that afferents from the remaining otolith organs are necessary for the functional compensation.