The Mondini dysplasia--from early diagnosis to cochlear implant.

The early diagnosis of the Mondini dysplasia by means of electrocochleography and temporal bone polytomography, and the prevention of further hearing loss through the endolymphatic subarachnoid shunt operation, brought new data on the follow-up of patients with this malformation. They were found to present specific medical and educational problems. Children with concomitant serous otitis media were found to develop profound hearing loss following insertion of ventilation tubes due to fistula formation in the oval window. One cochlear implant, employing the technique developed by William House, was performed in a 22-year-old patient in whom the dysplasia had not been previously recognized. The importance of early diagnosis and treatment will be emphasized.

Sudden vertigo of central origin.

Intense vertiginous attacks with sudden onset and of several months' duration were observed in a group of patients following viral infection. Neurotological examination revealed different types of signs of involvement of the central vestibular apparatus. All of these patients became dizzier when medicated with vestibular depressor drugs, and could obtain symptomatic relief with corticosteroids. They became asymptomatic after a period of 3-4 months from the onset of the disease, but the signs of central vestibular involvement persisted for 6-12 months.

The perilymphatic hypertension syndrome.

Perilymphatic hypertension is usually related to gushers which occur in the operating room when the stapes footplate is punctured or removed. These patients present defects in the modiolus, or enlarged cochlear aqueducts, which allow easy access of spinal fluid to the perilymphatic space. This syndrome was detected in children and appears to present consistent audiological patterns. They present a mixed hearing loss and in spite of the presence of an air-bone gap the stapedial reflexes are present. These children benefit from the use of hearing aids but show very little insertion gain in the low frequencies when tested with real-ear measurements.

High frequency audiometry and chronic renal failure.

High frequency audiometry with pure tones from 9 to 18 kHz was performed in 37 patients with chronic renal failure undergoing conservative medical treatment and in a control group of 96 patients (age range 30 59 years for both groups). It was noted that the high frequency thresholds were significantly higher for the patients with chronic renal disease, and further deterioration of hearing function was observed at the examination performed after 1 year of follow-up. It was concluded that high frequency audiometry is a sensitive method for detecting hearing changes in patients with chronic renal failure and can be used to monitor these patients.

Cognitive potentials in children with learning disabilities.

A group of 16 children, aged from 8 to 14 years, with learning disabilities, were studied by means of a series of conventional and sensitized audiological tests, including recording of the late cognitive electrical responses (P300). They had no otolaryngological or neurological complaints but expressive language disorders (difficulties in speaking and/or writing), receptive language disorders (difficulties in reading and text comprehension), and lack of concentration and/or restlessness. Their audiograms, speech discrimination and immitance tests were normal. The P300 responses, as compared with those found in 20 normal controls within the same age group, occurred at significantly longer latency periods.

Vestibular disorders caused by defective enzyme mechanisms in the small intestine.

A group of patients suffering from long periods of unsteadiness and presenting hyperreflexia in response to torsion swing and/or caloric stimulations were found to have abnormal results in glucose and lactose tolerance tests, but normal or reduced insulin liberation. Biopsies of the jejunal mucosa have shown abnormal enzyme activity or deficiencies in transport mechanisms. The use of a diet suited to each specific deficiency resulted in a high percentage of relief from the vestibular symptoms of these patients.

Glucose, insulin and inner ear pathology.

One hundred consecutive patients with a clinical suspicion of metabolic inner ear disorder were submitted to a 5-hour glucose tolerance test with simultaneous titration of insulin. It was found that 82% of the patients presented abnormal glucose and/or insulin values. The analysis of the results revealed hypo- or hyperglycemia were present in only 41% of the cases, and that all patients had normal fast values for glucose. Hyperinsulinemia was the most frequent abnormality and was found to constitute the determining factor of the inner ear disorders. 26 of the patients presented Meniere's disease. The pathophysiological model for insulin-induced Meniere's disease is discussed. Follow-up data indicate that these patients respond well to a diet with reduced intake of small molecule carbohydrates.

[Cavernous hemangioma of the internal auditory canal. Apropos of a case]. / Hémangiome caverneux intracanalaire. A propos d'un cas.

A case of cavernous hemangioma in the internal auditory canal was reported. There were no specific symptoms or physical or neurotological findings to differentiate from acoustic neuroma. The clinical and surgical aspects of this rare tumor were reviewed and discussed.

Calcium antagonists as a peripherally acting labyrinthine suppressant in humans.

A total of 908 patients with peripheral vestibular disorders were treated with calcium antagonists for periods of 10 to 120 days in a series of controlled clinical trials using a placebo or other medication for comparison. The cumulative analysis of the results indicates that cinnarizine, flunarizine and bencyclan have produced good clinical results, particularly in patients with vascular problems. A comparison between different doses used in different trials revealed a correlation of good clinical results to smaller doses of some of these medications.

Ménière's disease.

The authors review some of the contrasts that Ménière's disease presents to the practicing otologist and describe recent findings in electrocochleography (ECochG) and vectornystagmorgraphy that have been of help in establishing the diagnosis in unusual cases. ECochG has also been useful in detecting the presence of the disease in the patient's better ear. The goals of medical and surgical treatment are discussed in relation to these findings.

Flunarizine and cinnarizine as vestibular depressants. A statistical study.

The authors analysed the vestibular depressant activity of flunarizine and cinnarizine, as compared to a placebo, in 58 patients with peripheral labyrinthine disorders. The activity of the medications was judged by the reduction of the average velocity of the slow phase of the post-caloric nystagmus, before and after treatment. In spite of being administered in a smaller dosis, flunarizine appeared to have a more intense vestibular supressant activity, as judged by different statistical tests.

The Mondini dysplasia - a clinical study.

The authors describe a clinical approach to the diagnosis and treatment of sensory-neural hearing loss associated to the Mondini dysplasia. The first element of diagnostic suspicion was represented either by a history of fluctuant and progressive hearing loss or by electrocochleography patterns showing multiple-peaked acoustic nerve action potentials similar to those observed in Ménière's disease. Confirmation of the diagnosis was obtained by temporal bone polytomography. An endolymphatic subarachnoid shunt was performed in 22 ears of 18 patients. The results, although preliminary, suggest that the shunt effectively contributes to stabilize the hearing and arrest the further progression of the hearing loss.

Vestibular decruitment. A study of the phenomenon in a normal population.

Vestibular decruitment is a phenomenon characterized by the presence of less intense responses following stronger vestibular stimulation, and its presence has been considered as indicative of central vestibular pathology. In this study conducted in 24 normal individuals, with normal caloric responses, decruitment was observed in 58.3% of the cases. These decruitments were of types I and II as defined by Torok. Type III decruitment was not seen in these patients. In view of the present findings the actual meaning of the phenomenon must be investigated in larger numbers of patients with peripheral or central vestibular disorders.

Controlled clinical trial of pentoxifylline versus cinnarizine in the treatment of labyrinthine disorders.

Seventy-six patients with labyrinthine diseases of vascular origin were treated in a 6-week double-blind comparative study with either 400 mg pentoxifylline ('Trental') or 75 mg cinnarizine 3-times daily. Clinical evaluations, supported by audiological tests and vectornystagmography, were carried out before and after treatment. Statistical analysis of the results showed pentoxifylline to be globally superior to cinnarizine and especially to have a more intense antivertiginous effect. No significant differences were observed between the two drugs in respect of tinnitus and hearing loss therapy. Side-effects were occasional, mild and well tolerated in the pentoxifylline group, and more pronounced and frequent with cinnarizine.

Da velocidade angular real maxima da componente rapida do nistagmo pos-calorico em individuos normais. / On the maximum real angular velocity of the rapid component pos-caloric nystagmus in normal subjects

A prova calorica foi realizada em 38 individuos normais com o intuito de se avaliar o comportamento dos valores absolutos e relativos da velocidade angular real maxima da componente rapida do nistagmo pos-calorico, a prova de FITZGERALD & HALLPIKE (1942) com vecto-electronistagmografia.Verificou-se que foi possivel estabelecer limites criticos normais dos valores absolutos e relativos desse parametro, que podem ser uteis para a identificacao de alteracoes quantitativas na patologia otoneurologica