[Round window microcatheter administered microdose of gentamycin: an alternative in the treatment of tinnitus in patients with Menière's disease]. / Round-Window-Mikrokatheterassistierte Mikrodosierung von Gentamycin: Alternative in der Behandlung des Tinnitus bei Patienten mit Morbus Menière.

BACKGROUND: In this study, we review the results of Menière's disease treatment using microdose gentamycin delivered directly to the round window using a new microcatheter system. PATIENTS AND METHODS: 11 patients were treated by 1.25 mg gentamycin on the 3rd and 7th day after insertion of the new microcatheter at the niche of the round window membrane, while a second group of 7 patients was treated by a gentamycin dosage of 1 microliter/h continuously applied by a minipump over a period of 10 days. Electrocochleography was derived by an integrated electrode and the microcatheter was removed after 10 days. The results were analysed with a follow-up ranging from 6 to 12 months. RESULTS: In 15 of 18 patients (83%) tinnitus was improved significantly throughout the follow-up period. Vertigo was eliminated in all patients, and pressure was relieved in 17 of 18 (94%). CONCLUSIONS: These preliminary data suggest that gentamycin delivered by the Round-Window-Microcatheter is a safe and effective treatment for the reduction of tinnitus, vertigo, and pressure associated with Menière's disease.

Demonstration of autoantibodies to the endolymphatic sac in Meniere's disease.

Recent evidence suggests that immune mechanisms may underlie some cases of Meniere's disease. This study was conducted to determine whether an autoimmune mechanism is involved. Sera from 30 patients with Meniere's disease were reacted with human endolymphatic sacs and examined by indirect immunohistochemistry and fluorescence microscopy. Three of the samples (10%) showed positive staining, indicating immunoglobulin G (IgG) binding against the sac. No positive staining occurred when sera from healthy individuals or phosphate-buffered saline was used as a control. Clinical data showed an association between immunoreactivity and extent of disease (worse hearing over a shorter disease course and bilateral involvement). This study suggests that, in some cases of Meniere's disease, autoantibodies directed against human endolymphatic sac are present in the sera, supporting the theory that a specific autoimmune reaction takes place in a minority of patients with Meniere's disease.

Pathophysiological mechanisms in immune inner ear disease.

Immune inner ear disease is a somewhat controversial entity which describes cochleovestibular dysfunction that is related to immune-mediated mechanisms. The diagnosis of this disease is based on clinical presentation and response to various treatment protocols. Unfortunately, the presentation is variable and the treatment empirical, and this has caused much confusion in the diagnosis and management of the condition. To elucidate the variable nature of the disease, it is important to understand that more than one mechanism of immune injury may be involved. This paper attempts to classify clinical and experimental cases of immune inner ear disease with regard to the Gell and Coombs classification scheme of immune-mediated injury. By understanding the different pathophysiological mechanisms involved, the clinician should be better able to diagnose and manage this difficult problem in a directed fashion. The ramifications of the proposed classification system on the diagnosis, treatment and future research of immune inner ear disease are discussed.

Pathophysiologic theories in the etiology of Meniere's disease.

The etiology of the attacks of vertigo that occur in Meniere's disease is discussed in this article. None of the current theories remain tenable in view of recent findings concerning the physiology and pathophysiology of the cochlea and endolymphatic sac. A new theory suggests that a narrowed duct becomes obstructed by debris that is cleared by a combination of the secretion of hydrophillic proteins within the sac and a hormone, saccin, that increases the volume of endolymph within the cochlea. It is proposed that the sudden restoration of longitudinal flow initiates the attacks of vertigo.

Immune mechanisms in Meniere's syndrome.

Immune-mediated inner ear disease is a nonspecific term used to describe cochleovestibular dysfunction that results from immune-mediated injury. The ear may be involved directly or secondarily by the immune reaction, and evidence exists to implicate all four of the Gell and Coombs mechanisms of immune injury. The clinical presentation may be variable, with the diagnosis based on presentation, laboratory studies, and response to therapy. Current research is directed toward determining antigen-specific laboratory tests to improve diagnostic capabilities, examining human temporal bones to elucidate the pathophysiologic mechanisms involved, and developing a clinical laboratory and pathologic correlation.

Outbreak of vertigo in Wyoming: possible role of an enterovirus infection.

An epidemiologic investigation was conducted to characterize and evaluate the possibility of a viral aetiology of an outbreak of acute vertigo in Hot Springs Country, Wyoming, during autumn 1992. Case-finding identified Hot Springs County residents who sought medical attention for new onset vertigo during 1 August, 1992-31 January 1993. Thirty-five case-patients and 61 matched controls were interviewed and serum specimens were obtained during January 1993. Case-patients were more likely than controls to report symptoms (e.g. fatigue, sore throat, fever, diarrhoea) of antecedent acute illness. Case-patients did not have a significantly greater prevalence or mean titre of IgG antibodies to respiratory syncytial virus, parainfluenza viruses, Epstein-Barr virus, and cytomegalovirus than controls. Serologic evidence of recent enterovirus infection (IgM antibodies) was found for 74% of case-patients compared with 54% of controls (P < 0.05), suggesting a possible association between vertigo and enterovirus infection. Future studies are needed to define the role of enteroviruses in innerear diseases.

Normal evoked otoacoustic emissions with a profound hearing loss due to a juvenile pilocytic astrocytoma.

An interesting case is reported of an 11-year-old girl who experienced a sudden, profound right-sided hearing loss at the age of 5 years after hearing a "pop" and the sound of rushing water in her right ear. At that time the patient was thought to have Mondini's deformity, and a perilymphatic fistula of the oval window in the right ear. Six years later she was diagnosed with a juvenile pilocytic astrocytoma of the pons with an exophytic component extending into the cerebellopontine angle. Of particular interest in this case is the presence of evoked otoacoustic emissions in the right ear with a profound neural hearing loss. The presence of transient evoked and distortion product otoacoustic emissions confirmed normal sensory outer hair cell function and an intact peripheral auditory system in a clinically deaf ear, thus indicating hearing loss due to a neural component instead of a sensory component, which was previously assumed.

Fluorescein as an easy, low-cost, indirect, or reverse intraoperative marker to rule out perilymph versus local injection.

Clear fluid found in the dependent portions of the middle ear (round and oval windows) is generally assumed to be perilymph (PL). However, all clear fluid observed at middle ear exploration is not necessarily PL. PL should be distinguished from local injection. An accumulation of the local injection could be confused with perilymph fistula (PLF) diagnosis. There is no standard way of distinguishing perilymph from local injection, as both are clear, watery fluids. Fluorescein, tagged with a mixture of xylocaine and epinephrine (FLOTAX) was used as the local injection in 10 patients undergoing middle ear exploration for possible PLF.FLOTAX was injected into the vascular strip and ear canal skin in routine fashion. Transtympanic endoscopy and special Zeiss custom fluorescein filters were used to document any accumulation of fluid in the dependent portions of the middle ear and confirm whether any fluorescein (from the FLOTAX) was present. The middle ear was checked both before (endoscopically) and after raising the tympanomeatal flap. After the "local" injection, FLOTAX was observed to accumulate in dependent portions of the middle ear before raising the tympanomeatal flap in six of 10 ears. In the other four ears, FLOTAX slowly seeped into the middle ear cleft after the tympanomeatal flap was elevated and manipulated. Fluorescein can be used as an inexpensive, indirect intraoperative marker or "reverse test" for possible PLF. By eliminating a likely and common offender (local injection), any accumulation or reaccumulation of clear fluid that is not fluorescein tagged may more confidently be identified as possibly perilymph. The use of FLOTAX helps eliminate the confusion between the local injection and potential PLF. It does not in any way eliminate the possibility of other transudates from incisions, allergic reactions, or other manipulation of the tympanomeatal flap or middle ear mucosa.

CT imaging of radiopaque shunt catheter after endolymphatic duct surgery.

Until now, shunt catheters used in endolymphatic sac (ELS) and duct surgery have not been visible at computed tomography (CT) imaging. This report summarizes findings at postoperative CT in 50 patients who underwent endolymphatic system decompressive surgery with a new opaque shunt catheter placed at or into the endolymphatic duct (ELD). The catheter was visible in all 50 cases, and the best projection for visualization was the semicoronal view. The postoperative visualization of the opaque shunt catheter allows determination of the exact placement of the catheter (a) in the bony vestibular aqueduct (within the membranous ELD); (b) at the external aperture of the bony vestibular aqueduct (within or at the membranous ELD at its junction with the ELS); or (c) outside the bony vestibular aqueduct system or aperture but presumably within the membranous ELS. These objective findings may be used as a marker to help predict outcomes from this type of nondestructive inner ear surgery, as well as to determine exactly where the surgeon placed the shunt-tube catheter tip.

Immunoperoxidase study of the endolymphatic sac in Menière's disease.

A growing body of evidence suggests that some cases of Menière's disease may be mediated by immune mechanisms. Because endolymphatic sac dysfunction is believed to be an underlying cause of Menière's disease, this study used immunohistochemical techniques to demonstrate the presence of immune complex deposition in the sacs of patients with Menière's disease. Positive immunoglobulin G (IgG) staining was noted in 10 of 23 sac biopsies from Menière's patients, with 2 specimens showing perivascular deposition. Only 1 of 5 control specimens was only slightly positive for IgG. Clinical correlation showed a statistically significant increase in disease bilaterality (P < .05), larger summating potential/action potential (SP/AP) ratios with electrocochleography (ECoG), and a tendency toward worse hearing and more progressive disease among the immunopositive Menière's patients. The results provided histological evidence of immune injury in the endolymphatic sacs of patients with Menière's disease.

Diagnosis of vestibular Menière's disease with electrocochleography.

The existence of vestibular Menière's disease, once considered a variant of classic Menière's disease, but without hearing loss, has been questioned because of lack of objective evidence that endolymphatic hydrops is involved with the disease process. Transtympanic electrocochleography (TT ECoG) has emerged as a useful tool for electrophysiologic monitoring of the inner ear, and is especially valuable in assessing endolymphatic hydrops. A retrospective chart review was performed to identify those patients with a diagnosis consistent with vestibular Menière's disease in order to determine the presence or absence of endolymphatic hydrops using TT ECoG. A total of 15 patients were identified. Using established norms for the summating to action potential ratio (SP:AP) with click stimulus, 73 percent demonstrated values consistent with endolymphatic hydrops. The most commonly associated symptom was aural fullness (60%), followed by tinnitus (33%), and the disease was bilateral 53 percent of the time. The similarities between this clinical entity and classic Menière's disease are noted, and theoretic considerations are made.

Intraoperative electrocochleography of endolymphatic hydrops surgery using clicks and tone bursts.

Seventy-seven ears of patients with Menière's disease (n = 69) and other ears with secondary endolymphatic hydrops (n = 8) underwent valved shunt surgery, and were monitored with intraoperative electrocochleography (ECoG) using clicks stimuli (n = 77), and both clicks and tone bursts (n = 37). Of the 41 ears in which the "baseline" measurement was > 35% summating potential to action potential (SP/AP) amplitude click ratio, 23 ears (56%) showed a significant ratio decrease (improvement). Twenty-six ears out of the 37 that were monitored by both clicks and tone bursts had abnormal absolute SP tone bursts amplitudes at "baseline" and 8 ears (31%) changed to normal at "closing". When tone burst ECoG information is combined with click stimuli, our basic understanding of click ECoG is enhanced and our ability to assess electrophysiologic changes intraoperatively is improved.

Delayed endolymphatic hydrops following acoustic tumor removal with intraoperative and postoperative auditory brainstem response improvements.

A 36-year-old female with delayed endolymphatic hydrops (ELH) after acoustic tumor removal of the same ear is reported. She presented at our clinic with pressure, fullness, and tinnitus involving her right ear. She had an abnormal auditory brainstem response (ABR), and a computed tomography showed a right acoustic tumor. She underwent a complete tumor removal from a suboccipital incision with significant intraoperative and postoperative ABR improvements. Preoperatively, there was no fluctuation in her hearing and her electrocochleography (ECoG) was normal. She did well postoperatively, but 18 months after the tumor surgery she complained of noticeable fluctuating hearing loss with increased tinnitus and aural fullness of her right operated ear and nausea but no vertigo. Neurotological examinations showed a positive right urea dehydration and positive right ECoG for enhanced summating potential to clicks. She was diagnosed as cochlear Meniere's disease with delayed ELH, failed medical management and then underwent a valved shunt surgery for ELH to stabilize her hearing. Eight years after her acoustic tumor surgery and 6 and a half years after her ELH surgery she has stabilized hearing with normal ABR and ECoG. Her clinical course is presented and a pathogenesis of ELH is discussed.

Intraoperative electrocochleographic monitoring of inner ear surgery for endolymphatic hydrops. A review of cases.

The purpose of this review of cases was to determine whether or not the SP/AP amplitude ratio changes during unidirectional inner ear valved shunt surgery for the decompression of the hydropic labyrinth in Meniere's disease. A series of 62 patients underwent shunt surgery over a 20 month period. In 43 cases (43 ears), ECoG responses were successfully recorded throughout the duration of surgery. A pair of two-tailed paired t-tests were computed for SP/AP amplitude ratios acquired during intraoperative ECoG monitoring at the baseline and closing stage of surgery. The first t-test compared the mean SP/AP amplitude ratios at baseline and closing for those cases in which the SP/AP amplitude ratio at baseline was abnormal (baseline > 35%). The t was significant, t(19) = 4.63, p < 0.01). The second t-test compared the mean SP/AP amplitude ratios at baseline and closing for those cases in which the SP/AP amplitude ratio at baseline was WNL (baseline < or = 35%). The t was insignificant, t(20) = 0.31, p > 0.05. Variations between baseline and closing measurements were categorized as either a reduction, no change (very stable over time), or an increase in the SP/AP amplitude ratio. A change in the SP/AP amplitude ratio from baseline to closing of > or = 7% (x = 2%, SD = 2%) was considered statistically significant. Overall (43 cases), 49% of the variations fell into the no change category, with 42% and 9% of the cases placed in the reduction and increase categories, respectively. Of the cases (22) in which the baseline measurement was outside the limits of normal, 64% showed a reduction, 32% showed no change, and 4% showed an increase in the SP/AP amplitude ratio at closing. Changes in the SP/AP amplitude were observed at various stages of the surgical procedure. Reductions were found at each of the five surgical steps identified, with the majority of the changes almost evenly divided between the stages of mastoid drilling and opening sac. Increases in the SP/AP amplitude ratio were found in four cases. The increases were observed during drilling of the mastoid bone in two cases and sac decompression and opening the sac in the remaining cases.

Vincent's violent vertigo. An analysis of the original diagnosis of epilepsy vs. the current diagnosis of Meniére's disease.

The authors propose to correct the historical misimpression that Vincent van Gogh's medical problems resulted from epilepsy. Rather, the authors propose his main medical problem was Meniére's disease. The authors have reviewed the 796 personal letters written by van Gogh. The symptoms of his Vertigo attacks, their presentation and duration as described in these letters, taken as a whole, are consistent with the clinical picture of Meniére's disease, not epilepsy. They point out that Prosper Meniére's description of his syndrome was not well known at the time of van Gogh's death, and was often misdiagnosed as epilepsy. During the last years of his life, van Gogh was labeled epileptic, although no rigid criteria for this diagnosis are evident. This diagnosis is still prevalent in the art history literature today. His symptoms included episodic vertigo and dizziness, physical imbalance, hearing symptoms, ear noises (tinnitus) as well as a presumed secondary psychological reaction to his physical symptomatology. van Gogh's diagnosis of epilepsy is based on written diagnosis in his medical records in 1889 when he was interred (voluntarily) in St. Remy at an asylum for epileptics and lunatics.

Van Gogh had Menière's disease and not epilepsy.

We intend to correct the historical error that Vincent Van Gogh's medical problems resulted from epilepsy plus madness, a diagnosis made during his life but for which no rigid criteria are apparent. Review of 796 personal letters to family and friends written between 1884 and his suicide in 1890 reveals a man constantly in control of his reason and suffering from severe repeated attacks of disabling vertigo, not a seizure disorder. His own diagnosis of epilepsy was made from the written diagnosis by Dr Peyron, the physician at the asylum of St Remy (France), wherein on May 9, 1889, Van Gogh voluntarily committed himself to the asylum for epileptics and lunatics. However, the clinical descriptions in his letters are those of a person suffering from Meniere's disease, not epilepsy. The authors point out that Prosper Meniere's description of his syndrome (an inner-ear disorder) was not well known when Van Gogh died and that it often was misdiagnosed as epilepsy well into the 20th century.