Audiological findings in 100 USH2 patients.

Bilateral sensorineural hearing loss (HL), classically described as mild to severe with a typically down-sloping audiometric configuration, is the earliest symptom occurring in Usher syndrome type II (USH2). Audiological findings were analyzed in a total of 100 USH2 patients (92 families) divided into three groups according to the gene involved: 88 USH2A, 10 GPR98 and 2 DFNB31 patients. A fine analysis of audiograms was performed (pure tone average, degree of severity, configuration). The median age of HL diagnosis was 5 years (range 8 months-31 years) although the median age at USH2 diagnosis was 34.5 (range 8-76). Moderate HL was predominant (76%) and a gently down-sloping configuration characterized most audiograms (66%). No statistically significant difference was found between USH2A and GPR98 patients but a tendency was clearly noted for more GPR98 patients to present with severe hearing loss. It is not possible to predict the mutated gene from audiograms.

[Chemical labyrinthectomy: results and applications]. / Labyrinthectomie chimique. Résultats et intérêt.

GOAL OF THE STUDY: Unilateral Menière's disease can be treated by chemical labyrinthectomy. The goal of this work was to report our clinical experience with chemical labyrinthectomy, and to discuss the respective value of labyrinthectomy and of vestibular neurotomy for treating unilateral Menière's disease. METHODS: Twelve patients underwent chemical labyrinthectomy using middle ear gentamicin infusion. Before treatment, patients had a pure tone audiogram and a vestibular caloric testing. After treatment, patients had a clinical assessment at 1 and 6 months after treatment (pure-tone audiogram, vestibular caloric testing), and answered a questionnaire at 1 and 2 years following the treatment (number of vertigo attacks, ataxia, tinnitus, subjective assessment of the method by the patient). RESULTS: At two years post-treatment, the vertigo cure rate was 91.6% (ABC using AAOO criteria), vestibular function was abolished in 70% of cases, ataxia was reported in 42% of patients, 16.6% of patients had hearing deterioration above 20 dB HL (C following AAOO criteria), tinnitus was decreased in 50% of cases. Of 12 patients, 8 were satisfied. CONCLUSION: Chemical labyrinthectomy cures vertigo in Menière's disease, with similar cure rate than vestibular neurotomy. Minor complications can occur with chemical labyrinthectomy. This method should be used in more patients.

[Rotatory impulse test does not replace caloric tests]. / L'épreuve rotatoire impulsionnelle ne remplace pas les tests caloriques.

We sought to determine whether the rotatory impulsional test was capable of exploring the canalar function with sufficient precision to replace the caloric test, as it has been recently affirmed. We first compared the observed preponderance from this test with that measured during a sinusoidal (20 and 4 s) test. We observed that, in the case of a significant preponderance for a given test, there was complete redondance with the preponderance observed with any other test. The rotatory impulsional test does not present any specific advantage compared to other kinetic test as far as the observation of the preponderance phenomenon. We then compared the preponderance with the results of the caloric tests and came to the following conclusions i) the absence of preponderance does not allow us to predict the absence of vestibular deficit, due to the fact that 37% of the deficits were compensated for including acoustic neuroma; ii) the presence of a preponderance does not allow a priori to say whether it is of vestibular, cervical, or central origin and systematic caloric tests shows that almost one fourth of preponderance observed is not associated with unilateral weakness iii) supposing that a clinical argument allow us to conclude as to the probable vestibular origin of a vestibular preponderance, the direction of this preponderance does not allow us to determine which side is involved. In fact, if the undercompensated deficits are 3 times more frequent than overcompensated deficits, the proportion of preponderance not linked to a significant deficit indicates that the probability of encountering a preponderance related to a specific undercompensated deficit is approximately 50%. We thus did not find in the rotatory impulsional test any specific advantage allowing us to predict the laterality of a vestibular lesion.

A protective effect of 5-HT3 antagonist against vestibular deficit? Metoclopramide versus ondansetron at the early stage of vestibular neuritis: a pilot study.

OBJECTIVES: Ondansetron is an antiemetic 5-HT3 receptor antagonist with proven efficacy in central balance disorder. A pilot study investigated impact on acute unilateral vestibular neuritis. PATIENTS AND METHODS: A randomized clinical trial included 20 vestibular neuritis patients. Subjects received methylprednisolone-valacyclovir, associated to 5 days' metoclopramide (30 mg/d; group M, n=10) or ondansetron (8 mg/d; group O, n=10). Assessment was based on early and 1 month videonystagmography, duration of hospital stay and time to first independent walking. Blinded intention-to-treat analysis used univariate (Student test) and multivariate (linear logistic regression) analysis. RESULTS: Early caloric vestibular deficit was significantly lower in group O than group M (56.53% versus 84.38%; P=0.03). Vestibular preponderance did not differ between groups (8.2°/s in O versus 10.34°/s in M). At 1 month, trends were observed for vestibular deficit (43% in O versus 63.4% in M; P=0.07) and preponderance (1.67°/s in O versus 1.74°/s in M; P=0.4). Hospital stay and time to first independent walking were significantly shorter in O (2.88 versus 4.5 days (P=0.03); and 1.25 versus 2.25 days (P=0.001), respectively). CONCLUSION: Early treatment with ondansetron associated to corticosteroids and antiviral treatment reduced vestibular deficit in acute-phase vestibular neuritis as compared to reference histamine H1 receptor antagonists. The treatment did not affect central compensation. Benefit includes improved tolerance of vertigo syndrome and reduced hospital stay. These results should be confirmed on a larger series, particularly to determine the mechanism of action of 5-HT3 antagonists on vestibular function.