Surgical capping of superior semicircular canal dehiscence.

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.

Cogan's syndrome: clinical evolution of deafness and vertigo in three patients.

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.

Vestibular function in patients with cochlear implantation.

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.

Long-term evolution of subjective visual vertical after vestibular neurectomy and labyrinthectomy.

Subjective visual vertical (SVV) tilt, observed after vestibular neurectomy and labyrinthectomy, is considered to be due to the deafferentation of the otolithic organs. The aim of this study was to determine the long-term evolution of the SVV up to 4 years after surgery. Between 1993 and 1998 the SVV was measured in 35 patients (18 men, 17 women) aged from 21 to 71 years (mean 36 years). Vestibular neurectomy was performed in 30 patients and labyrinthectomy in 6. SVV was measured with a binocular test (vertical frame) and a monocular test (Maddox rod). Immediately after operation, the SVV showed a 10-30 degrees tilt toward the operated ear with the vertical frame (normal 0 +/- 2 degrees) and a 5-22 degrees tilt with the Maddox rod (normal 0 +/- 4 degrees). After labyrinthectomy, SVV returned to normal values after 1 year in all patients. SVV also returned to normal within 1 year after vestibular neurectomy in 20 patients; in the other 10 patients SVV was still slightly tilted 1-4 years after neurectomy: > 2 degrees with the vertical frame and > 4 degrees with the Maddox rod, particularly on the eye ipsilateral to the operated ear. Some of these 10 patients also had persisting disequilibrium.

Subjective visual vertical in peripheral unilateral vestibular diseases.

In humans, the perception of vertical is provided by input from various sensorineural organs and pathways: vision, eye-movements, and proprioceptive and vestibular cues, particularly from the otolithic organs and graviceptive pathways. Well known in several types of brainstem lesions, subjective visual vertical (SVV) abnormalities may also be observed after peripheral vestibular lesions, such as surgical deafferentation, with a deviation directed toward the operated ear. Subjective visual vertical abnormalities are presumably related to a lesion of the otolithic organs and/or to changes in the afferent graviceptive pathways. The goal of this prospective study was to measure the SVV and to define the influence of the otolithic organs in patients suffering from various types of peripheral vestibular diseases: unilateral sudden cochleo-vestibular loss, so-called "viral labyrinthitis" (VL), sudden idiopathic unilateral peripheral vestibular loss, so-called "vestibular neuritis" (Ne). Data were compared with findings after unilateral surgical deafferentations such as vestibular neurectomy (VN) and labyrinthectomy (Lab). Subjective visual vertical was measured with a binocular test (vertical frame) and a monocular test (Maddox rod). In all patients, after VN and Lab, the SVV showed a 10-30 degrees tilt with the vertical frame (N: 0 +/- 2 degrees), 5-15 degrees with the Maddox rod (N: 0 +/- 4 degrees). With the vertical frame, SVV was tilted > 2 degrees in VL (47%) and in Ne (37%); the Maddox rod showed a > 4 degrees tilt in VL (41%) and in Ne (42%). The tilt was directed toward the affected ear. Our results demonstrate that SVV is frequently tilted in acute peripheral vestibulopathies such as VL and Ne. These findings suggest that otolithic function is implicated in the deficit depending on the extent and/or the localisation of the peripheral vestibular lesion.

Patients with CHARGE association: a model to study saccular function in the human.

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.

[Effect of antiretroviral treatment on early electroencephalographic and otoneurologic manifestations in HIV infection and prognostic importance of verified perturbations]. / Effet du traitement antirétroviral sur les manifestations électroencéphalographiques et otoneurologiques précoces de l'infection à VIH et signification pronostique des perturbations constatées.

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.

Diplopia from skew deviation in unilateral peripheral vestibular lesions.

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.

Ocular tilt reaction associated with a sudden idiopathic unilateral peripheral cochleovestibular loss.

We recently observed a female patient who was suffering from acute right peripheral cochleovestibular loss associated with a marked vertical diplopia. Otoneurological examination showed profound deafness, and absence of nystagmic response to caloric and pendular rotatory test in the right ear. Neuroophthalmological examination showed skew deviation with right hypotropia, excyclotorsion, and tilt of the static visual vertical directed to the right side. Immunological and serological examinations were normal. Neurological examination and extensive neuroradiological investigations failed to demonstrate any central nervous system involvement. In this patient, skew deviation and tilt of the static visual vertical were interpreted as signs of an acute unilateral otolithic dysfunction, due to a sudden idiopathic peripheral vestibular loss.

Bilateral idiopathic loss of peripheral vestibular function with normal hearing.

From 1982 to 1993, 52 electronystagmograms (FNG) revealed an absent nystagmic reaction on both caloric (44 30 and 10 degrees C water irrigation) and rotatory pendular testing (0.05 Hz/peak velocity 60 degrees/s), which represents 0.6% of all FNG performed during this period. Among these examinations, 14 patients (27%) presented a bilateral idiopathic loss of vestibular function (BILVF) with normal hearing and without associated neurological symptoms. Two different groups emerged: one group with simultaneous onset of BILVF ( 10 patients), with sudden imbalance and disequilibrium, worse in darkness, with an absence of bilateral caloric and pendular response. The other group (4 patients) was characterized by sequential onset of BILVF. These patients experienced several episodes of acute vertigo with persistent imbalances caloric and pendular responses showed unilateral, then contralateral loss with or without recovery of function. Eleven were controlled with a follow-up from 1 to 7 years. Patients of both groups remained permanently or episodically symptomatic, but only 4 patients complained of persistent oscillopsia. Viral infections, systemic diseases (hypertension, hypothyroidism, asthma), immune reactions (vaccination) and toxic factors (herbicide exposure) may play a role in the etiology of this rare bilateral peripheral vestibulopathy.

[Vestibular neuritis: a frequently unrecognized cause of diplopia]. / La névrite vestibulaire: une cause souvent insoupçonnée de diplopie.

The vestibular function plays an essential role in the stabilization of the image on the retina. In addition, when the head is tilted, it contributes to maintain horizontally the plane of the gaze. Vestibular changes can result in oscillopsia and/or diplopia. The latter is related to occurrence of a skew deviation. The authors emphasize the frequent occurrence of diplopia following disorders of the vestibular nerve, specially after vestibular neuritis. In clinical practice, the causal relationship between vestibular neuritis and diplopia is often unrecognized.

Skew deviation after vestibular neuritis.

We treated five patients with vestibular neuritis who had strabismus. Three of them spontaneously noted vertical diplopia. During the following weeks and months, strabismus progressively resolved, indicating the recently acquired nature of the oculomotor condition. In three of these individuals, a change in visual vertical and cyclo-torsion of the globes suggested that strabismus was a form of skew deviation that occurred as a part of an ocular tilt reaction resulting from the peripheral vestibular lesion. Strabismus appears to occur frequently in this common vestibular condition.

Vertigo as manifestation of vertebral artery dissection after chiropractic neck manipulations.

We recently observed a case of vertebral artery (VA) dissection following chiropractic neck manipulations. The first manifestation was unusual; in the form of vertigo. Therefore, the patient was referred to the otoneurologist. A VA dissection should be suspected in a case of vertigo following chiropractic neck manipulations, and vestibular tests should be done carefully, avoiding Rose's positions. In our case, the diagnosis was based on Doppler sonography and angiography. Chiropractic neck manipulations are the most frequent cause of traumatic VA dissection. Practitioners should be aware that, in some cases, these manoeuvres are life threatening.

[Clinical evaluation of otolithic function by the measurement of ocular cyclotorsion and skew deviation]. / Evaluation clinique de la fonction otolithique par mesure de la cyclotorsion oculaire et de la "skew deviation".

The ocular tilt reaction (OTR) consists in an association of an eye-head postural reaction, alteration of vertical perception, conjugated eye cyclotorsion, skew deviation and head tilt. We observed this condition following unilateral vestibular neurectomy and labyrinthectomy performed in patients suffering from severe Meniere's disease. All patients described postoperative vertical diplopia and vertical perception tilt. One illustrative case is reported in detail here. Changes in visual vertical were measured using a vertical frame with a pivoting pointer and the Maddox rod calibrated with a spirit level. The actual eye torsion was measured by a photography of eye-fundus. In the follow-up, it was found that vertical diplopia disappeared within a few days while conjugated eye cyclotorsion lasted weeks to months. Mechanisms involved in the OTR could be the consequence of a unilateral deafferentation of the utricular and saccular organs. We feel that the techniques of examination which we used with this patient might be a useful measurement for the clinical evaluation of unilateral otolithic dysfunction.

[Strabismus induced by peripheral vestibular lesions]. / Strabismes induits par des lésions vestibulaires périphériques.

Little attention has been paid in the ophthalmological literature to strabismus resulting from lesions located in the peripheral vestibular system. However, this phenomenon is commonly encountered in clinical practice. As a rule, this kind of strabismus shows a prominent vertical component. It is generally combined with a change in perception of verticality, conjugate cyclotorsion of the eyes, and head tilt. This association is known as "ocular tilt reaction". It occurs in a number of clinical settings which are believed to be related with alteration in the otolithical and/or vertical semicircular canal pathways. Strabismus occurring as a feature of ocular tilt reaction might result from different mechanisms according to the location of the lesion, e.g. in the utricles, in the midbrain tegmentum, and in the dorso-lateral medulla oblongata. This phenomenon is illustrated here with the report of a patient suffering from Ménière disease, who underwent selective vestibular neurectomy. Methods of ophthalmological evaluation in such cases is described.

Neuro-otological manifestations in different stages of HIV infection.

A systematic investigation of the incidence and type of peripheral and central auditory and vestibular disorders occurring in different stages of HIV infection was performed: 43 HIV-seropositive patients (14 were symptomatic with manifestation of AIDS or related complex and 29 were clinically asymptomatic) were compared with a matched control group of 33 HIV-seronegative homosexual volunteers. Among the symptomatic patients 8 (57%) out of 14 and among the asymptomatic HIV-positive patients, 13 (45%) out of 29 had abnormal results in various audiological, vestibular and electrophysiological tests. Only minor anomalies were found in 4 (12%) of the 33 seronegative homosexuals. Our results indicate a high incidence of neuro-otological disorders among patients suffering from AIDS. The numerous electrophysiological abnormalities found in asymptomatic HIV-seropositive persons suggest furthermore an early subclinical involvement of the auditory and vestibular systems.

A controlled study of early neurologic abnormalities in men with asymptomatic human immunodeficiency virus infection.

BACKGROUND: Although neurologic complications are frequent in the acquired immunodeficiency syndrome, their incidence and progression in early human immunodeficiency virus (HIV) infection remain controversial. The goal of this study was to assess neurologic manifestations in asymptomatic carriers of HIV. METHODS: We studied 29 HIV-seropositive homosexual men and 33 seronegative homosexual controls by means of neurologic and neuropsychological examinations, magnetic resonance imaging (MRI), and electrophysiologic tests (electroencephalography, multimodal evoked-potential tests, and otoneurologic tests). After six to nine months, the tests were repeated in 27 seropositive men and 30 controls. The investigators were blind to the serologic status of the subjects. RESULTS: The seropositive subjects had a mean CD4+ lymphocyte count of 635 X 10(6) per liter. Neurologic and neuropsychological examination, MRI, and measurements of pattern visual evoked potentials did not show significant differences between the two groups. The latencies of the median-nerve somatosensory evoked potentials were slightly prolonged in the seropositive men. The initial electroencephalogram was judged abnormal in 8 of 27 of the seropositive subjects (30 percent) as compared with none of the controls, with a slowing of fundamental activity, anterior spread [corrected] of alpha rhythm, subnormal reactivity, and unusual anterior theta activities. These findings were confirmed by computerized spectral analysis. The second electroencephalogram was abnormal in 10 of 25 of the seropositive men (40 percent). The otoneurologic evaluation identified abnormalities in the central auditory or vestibulo-ocular pathways in 34 percent of the seropositive men (10 of 29), as compared with 6 percent of the controls (2 of 33), on the first examination and in 44 percent (12 of 27) and 7 percent (2 of 30), respectively, on the second examination. Altogether, electrophysiologic abnormalities were found in 67 percent of the seropositive men (18 of 27) and 10 percent of the controls (3 of 30) (P less than 0.00005). CONCLUSIONS: In persons with asymptomatic HIV infection, electrophysiologic tests may be the most sensitive indicators of subclinical neurologic impairment. Electrophysiologic abnormalities are far more common in asymptomatic carriers of HIV than in controls and tend to progress over time.