Surgical plugging and resurfacing are well established treatments of superior semicircular canal dehiscence, while capping with hydroxyapatite cement has been little discussed in literature. The aim of this study was to prove the efficacy of the capping technique. Charts of patients diagnosed with superior semicircular canal dehiscence were reviewed retrospectively. All patients answered the dizziness handicap inventory, a survey analyzing the impact of their symptoms on their quality of life. Capping of the dehiscent canal was performed via the middle fossa approach in all cases. Ten out of 22 patients diagnosed with superior semicircular canal dehiscence were treated with surgical capping, nine of which were included in this study. No major perioperative complications occurred. In 8 out of 9 (89%) patients, capping led to a satisfying reduction of the main symptoms. One patient underwent revision surgery 1 year after the initial intervention. Scores in the dizziness handicap inventory were lower in the surgically treated group than in the non-surgically treated group, but results were not statistically significant (P = 0.45). Overall, capping is a safe and efficient alternative to plugging and resurfacing of superior semicircular canal dehiscence.

El vértigo es un síntoma consistente en una sensación de movimiento, que se manifiesta por una impresión de rotación y/o de ebriedad. Suele acompañarse de signos neurovegetativos, como náuseas, vómitos y sudores fríos. El paciente permanece consciente durante la crisis. El vértigo se debe a una disfunción de las aferencias del sistema vestibular (oído interno, nervio vestibular y vías vestibulares centrales) y a conflictos sensoriales relacionados con el sistema visuooculomotor y propioceptivo. El vértigo puede obligar al paciente a modificar su modo de vida, incluso en ausencia de este síntoma. El diagnóstico permite sobre todo proponer un tratamiento adecuado, que puede ser farmacológico (antivertiginosos, corticoides) o con rehabilitación vestibular e incluso con cirugía o psicoterapia. Es fundamental identificar las urgencias vitales para el paciente y las urgencias vitales para el laberinto.

The aim of this study was to evaluate the clinical symptoms, the otoneurological examinations, the treatment and the clinical course of three patients suffering from Cogan’s syndrome, a rare disease based on the clinical association of a non-syphilitic interstitial keratitis with a cochleo-vestibular deficit. This case series involved three patients with follow up. The clinical course of the three patients (aged 30, 48 and 49 years) with Cogan’s syndrome during a follow-up period of 2 to 6 years is reported. All patients underwent complete otoneurological, ophthalmologic and rheumatologic examinations and were treated with immunosuppressive therapy such as glucocorticoids and cyclophosphamide in two and glucocorticoids and methotrexate in one patient. Using immunosuppressive therapy, ophthalmologic symptoms disappeared rapidly in two patients. Hearing improved only in one and stabilized in a second patient. One patient died after 6 years of treatment because of complications of generalized vasculitis. Early diagnosis and rapid initiation of a combined immunosuppressive therapy such as corticosteroids and cyclophosphamide seem to be important in controlling the disease and avoiding persistent deafness. Whether systemic complications and a fatal outcome also can be prevented is still questionable.

Purpose: Otoneurologic findings in patients with superficial siderosis (SS) of the central nervous system are described. A 20-year-old man with acute vertigo, dizziness, with a history of head trauma in childhood; an 87-year-old woman with severe disequilibrium appearing after peridural anesthesia; and a 55-year-old woman with recurrent episodes of positional vertigo and progressive ataxia, suffering from a lumbar ependymoma are described; all patients complained of progressive bilateral hearing loss and tinnitus.Methods and Results: Otoneurologic examination showed bilateral sensorineural hearing loss, disturbed ocular pursuit and optokinetic nystagmus, incomplete visual suppression of the vestibulo-ocular reflex during rotatory pendular testing, right hyporeflexia, and bilateral caloric areflexia. Cerebral magnetic resonance imaging (MRI) revealed rims of hypointensity surrounding the brainstem and linear hypointensities following the surface of the cerebellar folia.Conclusions: The etiology stems from subarachnoid hemorrhage, but the source of bleeding may remain obscure. Bilateral hearing loss is described in 95% and disequilibrium in 90% because of peripheral vestibular deficit and cerebellar ataxia. In patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS. (C) 2004 Elsevier Inc. All rights reserved.

We report on a patient with bilateral cochlear implants (a Med-El Combi40 and a Med-El Combi40+), as well as considerable experience in scuba diving with both of his implants. After having been exposed to 68 and 89 dives, respectively, in depths of up to 43 m, both cochlear implants are in working order and the patient continues to receive excellent speech recognition scores with both cochlear implant systems. The presented data show that scuba diving after cochlear implantation is possible over a considerable number of dives without any major negative impact on the implants.

We report 3 patients who had acute peripheral vestibular dysfunction minutes to hours after a car collision with whiplash injury without head trauma. The accident was a frontal collision in 1 case, a rear impact in the second, and lateral in the third. All patients complained immediately of cervicalgia, headache, acute vertigo with a sensation of erroneous body movements, and slipping of image with head movements. A sudden sensation of tilting of the environment when driving, tinnitus, and hyperacusis were also described. The otoneurologic findings showed bilateral canalolithiasis in 1 patient and an acute peripheral vestibular deficit in 2 patients. Tilt of the subjective visual vertical was measured in all patients. Cerebral magnetic resonance imaging yielded normal findings. As angular and linear accelerometers, the vestibular organs are directly exposed to high forces generated by whiplash mechanisms. Vertigo generated by peripheral vestibular lesions is probably underestimated in whiplash injuries and may often be incorrectly attributed to cervical or cerebral lesions.

OBJECTIVES To assess the prevalence, presentation, treatment, and evolution of Menière's disease in elderly patients (> or = 65 yr old). STUDY DESIGN Retrospective clinical study and case report. SETTING A neuro-otology referral center at the university hospital in Bern, Switzerland, and a neurotologic practice in Geneva, Switzerland. PATIENTS Patients were selected from the hospital clinic and otolaryngologic practice registers. Among 8423 neurotologic checkups performed between 1988 and 1998, 432 (5.1%) patients had definite Menière's disease and 66 (15.3%) of these patients were > or = 65 years old. INTERVENTION The files of these 66 patients were analyzed. RESULTS Menière's disease in the elderly had 2 modes of presentation: reactivation of longstanding Menière's disease (40.9%) and de novo Menière's disease (59.1%), where the first dizzy spells, tinnitus, and hearing loss appeared after 65 years of age. Drop attacks occurred in 11.1 and 25.6% of the cases, respectively, and could be responsible for misdiagnosis of strokes of the brainstem. The patients were treated by mild antivertiginous drugs (betahistine, cinnarizine). Twenty-seven patients (41%) underwent surgery: transtympanic ventilation tubes (19 patients), sacculotomy (4 patients), vestibular neurectomy (3 patients), surgical labyrinthectomy (1 patient). The vertigo spells disappeared in 73.3 to 100% of the cases according to the type of surgery performed. CONCLUSION Our study shows that Menière's disease in the elderly is not at all uncommon. It can appear as a de novo disease or as a reactivated longstanding disease. Drop attacks are more frequent than reported in general patient populations with Menière's disease and can mimic a stroke of the brainstem. Medical and surgical treatments have to be cautious because many of these elderly patients are fragile.

The aim of this work was to determine the influence of cochlear implantation (CI) on vestibular canal and otolithic function. Between 1995 and 1999, 15 patients (6 females, 9 males; 9-77 years old) underwent a vestibular examination before and after CI. Electronystagmography was performed between 5 and 8 days after CI in 9 patients, and with a time delay of 2-24 months in 10 patients. Pre- and postoperative evaluation included electronystagmography with caloric (44 degrees C, 30 degrees C, ice-water) and pendular rotatory testing. Otolithic function was measured postoperatively using off-vertical axis rotation (OVAR) in six patients. Preoperative data (n = 14) showed areflexia on caloric and rotatory pendular testing in deafness cases due to meningitis (n = 2) and in 2/5 patients with sudden idiopathic bilateral deafness. Two patients suffering from an idiopathic deafness had a unilateral hyporeflexia. Vestibular function was normal in the other eight patients. Immediately following CI, among patients with normal preoperative canal function, three developed vertiginous symptoms with spontaneous nystagmus, which disappeared within days to weeks. Later, postoperative canal evaluation was normal in 5/8 patients (62%) with initially preserved vestibular function: areflexia was measured ipsilaterally to the implanted ear in 1 patient and contralaterally in 2 patients. Hyporeflexia was measured ipsilateral to the implanted ear in two patients. OVAR examination, performed 2-19 months after surgery, showed a preserved otolithic function in all 6 tested patients. Transient vertigo on electrical CI stimulation was described in only one patient during the first postoperative weeks. The following conclusions can be drawn. Patients with deafness due to meningitis had an eradicated vestibular function. In other etiologies, vestibular function was most often preserved. CI did not usually abolish vestibular function, but the canal function was disturbed temporarily in 20% of cases. Otolithic function was preserved in all six CI patients tested in this series.

The term CHARGE association refers to a combination of congenital malformations, the mnemonic CHARGE designating the most frequently occurring anomalies in the constellation. "C" indicates coloboma of the retina, "H" heart defects, "A" choanal atresia, "R" retarded growth and/or central nervous system anomalies, "G" genital hypoplasia, and "E" ear anomalies and/or deafness. The inner ear anomaly consists of a specific form of labyrinthine dysplasia that includes Mondini dysplasia of the pars inferior (cochlea and saccule) and complete absence of the pars superior (utricle and semicircular canals). We observed the development of a child with CHARGE association up to the age of 10 years. There was complete absence of nystagmic response to bithermal caloric and rotatory pendular stimuli. A nystagmic reaction was elicited by the off-vertical axis rotation test, indicating stimulation of the saccular macula, the sole remaining vestibular sense organ in this dysplasia. This reaffirms that the saccule is a vestibular organ, even though it is located in the pars inferior. In spite of the severe bilateral vestibular deficit and coloboma of the retina, the child was able to walk at the age of 2 years. The delay in the development of walking was not due to central nervous system anomalies, as suggested by the "R" of the acronym CHARGE, but rather, to the severe sensorineural visual and vestibular deficits.

Electrophysiologic tests may be abnormal in asymptomatic HIV-1-infected individuals. Our study was aimed at determining whether these findings have a prognostic value and could be corrected by antiviral treatment. In 18 patients, followed for 34 or 43 months, these findings were not progressive. Only one patient developped Aids dementia complex (ADC). Three have died (one with normal, two with abnormal rests at baseline). To study the effect of antiviral treatment, another group of seven asymptomatic patients was included into a cross-over double-blind study with either eight week zidovudine or eight weeks placebo, separated by eight more week without treatment. Electrophysiological evaluation was also performed in a group of 15 patients before antiviral therapy with zidovudine or didanosine was started and again after a mean of three and 13 months treatment. Results did not suggest that treatment reverses early electroencephalographic and otoneurological changes seen in HIV-1 infection.

Electrophysiologic tests may be abnormal in asymptomatic HIV-1-infected individuals. Our study was aimed at determining whether these findings have a prognostic value and could be corrected by antiviral treatment. In 18 patients, followed for 34 or 43 months, these findings were not progressive. Only one patient developed Aids dementia complex (ADC). Three have died (one with normal, two with abnormal tests at baseline). To study the effect of antiviral treatment, another group of seven asymptomatic patients was included into a cross-over double-blind study with either eight weeks zidovudine or eight weeks placebo, separated by eight more weeks without treatment. Electrophysiological evaluation was also performed in a group of 15 patients before antiviral therapy with zidovudine or didanosine was started and again after a mean of three and 13 months treatment. Results did not suggest that treatment reverses early electroencephalographic and otoneurological changes seen in HIV-1 infection.

Vertical diplopia from skew deviation is well described in brainstem lesions. The phenomenon can also result from peripheral vestibular lesions. During the past years, we have observed these ocular changes in the acute stage following unilateral vestibular neurectomy and labyrinthectomy (n = 13), as well as in series of patients suffering from idiopathic sudden unilateral peripheral vestibular or cochleo-vestibular deficit (n = 5). Diplopia from skew deviation was noted immediately following ablative vestibular procedures; in patients with idiopathic vestibular deficit, it was observed as an associated sign. In all patients, clinical evaluation revealed an acute unilateral peripheral vestibular loss, with spontaneous nystagmus toward the unaffected ear and absence of nystagmic response to caloric testing on the affected ear. Skew deviation was measured using the Hess-Weiss test, which is based on the haploscopic principle. Static visual vertical was evaluated with the original methods of vertical frame and Maddox rod techniques. Photographs were made of the ocular fundi, to measure the degree of cyclotorsion of both eyes. In our patients, we found skew deviation with hypotropia of the eye that was ipsilateral to the affected ear and conjugated cyclotorsion and tilt of the static visual vertical on the side of the affected ear. Skew deviation was the first sign to disappear within a few days; conjugated cyclotorsion and tilt of the static visual vertical persisted for weeks to months. The eye-head postural reaction, consisting of head tilt, conjugated eye cyclotorsion, skew deviation, and alteration of vertical perception directed toward the side of the lesion, is known as the Ocular Tilt Reaction (OTR). The mechanism is presumably related to a lesion of the otolithic organs and/or to changes in the afferent graviceptive pathways. In man, the OTR is often mild and unrecognized, masked by spontaneous nystagmus and marked neuro-vegetative symptoms. Our observations indicate that skew deviation, as a part of the OTR, occurs in patients with sudden peripheral vestibular lesions, whether surgical or non-surgical in origin.