Vestibular investigations in acoustic neuroma.

Forty-eight patients with surgically proven acoustic neuroma were examined preoperatively with a comprehensive battery of vestibular tests. In 87% of the patients, reduced caloric responses were measured on the side of the lesion. In 60% of the patients a non-responsive labyrinth was found. Spontaneous nystagmus was observed in 29% of the patients. A significant correlation with the size of the tumour was found in the occurrence of abnormal saccades in the calibration test, pathological smooth pursuit movements and the occurrence of gaze nystagmus, respectively. There was no correlation between the magnitude of the caloric response reduction and the mean hearing loss, which suggests that the hearing loss and the vestibular function loss do not occur synchronously. This study shows that in most cases of acoustic neuroma, the peripheral part of the vestibular system is severely disturbed. Abnormalities in the central and peripheral part of the vestibular system are more easily recognized with increasing tumour size. We conclude that a complete battery of vestibular tests is essential in combination with audiometric, neurologic and radiologic analysis for the early clinical diagnosis of acoustic neuroma.

Secondary syphilis of the tonsils.

Secondary syphilis of the tonsils without accompanying signs in the mouth or pharynx is rare. A case report of a patient presenting with papular tonsillitis as a result of secondary syphilis is described.

Hereditary Angio-Edema (HAE).

Hereditary Angio-Edema (HAE) is manifested by recurrent attacks of acute circumscribed edema of the extremities, face, trunk, and respiratory or abdominal tract, occurring after minor physical or emotional trauma, or spontaneously. The clinical diagnosis can be confirmed by low serum-levels of the inhibitor of the activated first component of complement (C1-INH). A family of 35 members over six generations was investigated. About 40 per cent of the members were HAE-patients. An autosomal dominant inheritance with high penetrance was present. Four patients died by suffocation from laryngeal edema. In all HAE-patients the C1-INH serum-level was diminished, and also in two children who have yet had no complaints. Adequate medical advice, including the provision of a medical alert card and the prescription of C1-INH concentrate (for short-term prophylaxis and the treatment of attacks of laryngeal edema), is necessary. In patients with frequent serious attacks, long-term prophylaxis with Danazol is indicated.

Follow-up vestibular examination in Menière's disease.

A retrospective study was carried out on repeated audio-vestibular examinations in 53 patients with unilateral Meniere's disease, between the vertiginous spells. Most patients showed no change in the caloric response reduction of the afflicted ear in the subsequent tests compared with the first one. These patients had a comparatively long illness duration, a small percentage of fluctuations in the pure tone audiogram and mostly flat or falling type curves. A minority of patients showed an increase in or a fluctuating caloric test reduction. This group consists of patients with a short disease duration, mostly a fluctuating pure tone audiogram and rising or dome-shaped curves. The hearing loss and the caloric test reduction appear to deteriorate mainly in the first years of the disease. A certain parallelism between the course of vestibular and audiological damage seems to be present.

Vestibular investigations in Meniere's disease.

151 patients with unilateral Meniere's disease were investigated with a vestibular test battery in the interval between vertigo attacks, and the results were compared with those obtained in a control group. In 73.5% of the patients a caloric response reduction of the afflicted ear was observed. The mean caloric response reduction was 34.3%; a major response reduction or a total absence of the caloric response of the afflicted ear was rare. A correlation was found between the amount of caloric response reduction and the duration of the disease. Spontaneous nystagmus was found in 47% of the cases (mean value 3 degrees/sec) and a mainly direction fixed positional nystagmus in 78% (mean value 4.0 degrees/sec). No correlation was found between the mean hearing loss and the magnitude of caloric response reduction on the day of investigation.

The median nasal sinus and dermoid cyst.

The median nasal sinus and the dermoid cyst of the nose are relatively rare congenital anomalies. The most likely hypothesis for their ontogeny seems to be the retention of ectodermal fragments during closure of the anterior foramen of the neural tube early in embryonal development. The results of the surgical management of fourteen patients with a median dermoid cyst of the nose are discussed. Presenting symptoms were recurrent abscess formation on the dorsum of the nose, a secreting fistula in the median line of the dorsum of the nose, or meningitis. The draining site of the sinus varied less than the location of the dermoid cyst. Treatment consisted in radical excision in a quite stage, using the binocular microscope followed by primary reconstruction of nasal skeletal defects. Anomalies are due not only to development disturbances in the surrounding structures owing to the presence of the sinus and the cyst, but also to growth of the cyst and to infection. Partly for that reason, early surgical management is indicated. It should be preceded by proper radiological examination, because this can provide important clues as to the site of the dermoid cyst. If an endocranial extension has been established preoperatively, a combined ORL and neurosurgical operation is necessary.

Vestibulo-toxicity of tobramycin.

The case histories are described of two patients with normal renal function who developed permanent complete bilateral loss of peripheral vestibular function during tobramycin therapy. Despite the low incidence of the cochleotoxic and and vestibulotoxic side-effects of tobramycin, it seems advisable to monitor patients on tobramycin therapy for signs of vestibular dysfunction and hearing loss.

[Vestibular testing in Ménière's syndrome]. / Vestibulair onderzoek bij het syndroom van Ménière.

Extensive vestibular examination was done in fifty patients with unilateral Menière's disease in the interval between the attacks of vertigo. Horizontal and vertical eye-movements were recorded by electro-oculography. In 80% of the significant difference was found in the slow phase velocity of the nystagmus due to caloric stimulation between the normal and pathological ear (mean difference 41% +/- 24,2). A spontaneous nystagmus was found with closed eyes in 58% of the patients with a mean value of 2,5 degrees/sec +/- 1,6 and a positional nystagmus in 76% with a mean value of 2,3 degrees/sec +/- 1,5. Also in the silent phase (between the vertigo-attacks) distinct abnormalities can be found in the vestibular examination tests.

External septorhinoplasty.

One of the disadvantages of the usual endonasal technique of rhinoplasty is the poor surveyability of the surgical field. By joining the left and right marginal incision via a transverse columellar incision and elevating the skin of the columella and the dorsum nasi, the infrastructure of the nose can be exposed. The good exposition results in a meticulous surgery of the nasal dorsum, precise correction of the triangular cartilages, good access to the septum and more a-curate augmentation procedures of the prespinal area, columella or nasal dorsum. This "external" approach is more or less a synthesis of the subcutaneous endonasal method and surgery under visual control, with the only sacrifice of a small scar which has marked advantages on the other hand. The results of the external approach to septorhinoplasty in 30 patients with cosmetic and functional complaints are discussed. No complications attributable to the method were seen. Especially for difficult cases as revisional operations, congenital nasal deformities and multiple traumatic damaged noses the external approach appears to be very useful.

Surgical treatment of the infected preauricular sinus.

Preauricular sinus is a frequent occurring congenital abnormality (circa 0.25% in the white race), but gives mostly no symptoms. In a certain amount of cases symptoms can arise as recurring purulent secretion or a preauricular abscess. If the disease is recognized an adequate therapy can be accomplished. Sometimes the disease is not recognized and diagnosed as a sebaceous cyst or a furunculus. Also, if the sinus is recognized, it is necessary not to underestimate the disease. In several clinical studies a recurrencerate of 15--33% is quoted. Singer published in 1966 a technique of extirpation of the infected preauricular sinus with excellent results. This technique was adopted with addition of the use of the operating microscope. In nine patients with an infected preauricular sinus extirpation in toto was accomplished in a silent period. Under these nine patients were two recurrences after previous inadequate surgery. After a minimal follow-up of 2 years the patients were symptom-free. The quoted technique of extirpation of the infected preauricular sinus in toto seems to be an improvement for the therapeutic armamentarium.

Juvenile fibrous hamartoma of the pharynx.

A case of juvenile fibrous hamartoma of the pharynx in a three-year-old boy is described. It is a rare tumour, mostly occurring in the subcutaneous tissues in the infant. A pharyngeal localisation has not been described before. Complete local excision appeared sufficient for cure.

Changes in compensatory eye movements after unilateral labyrinthectomy in the rabbit.

Compensatory (slow phase) eye movements elicited by sinusoidal oscillation on a torsion swing were measured in rabbits, 6 months after destruction of the left labyrinth. A range of combinations of stimulus frequencies (0.048-1.8 Hz) and amplitudes (1-25 degrees) were used. Gain (amplitude of cumulative slow phase eye movement/amplitude of swing), phase (eye position vs. swing position - 180 degrees) and directional asymmetry were calculated from averaged records. With eyes covered, gain was much less than half of normal and phase lead was increased by at least 20 degrees. Spontaneous drift was minimal or absent; a slight asymmetry of reactions (preponderance of smooth movements to the intact side) was found. In the presence of vision, reactions were slightly improved, but only for low stimulus velocities. It is concluded that although the acute effects of unilateral labyrinthectomy in rabbits subside and a static equilibrium is achieved, dynamic performance of vestibular reactions remains much below normal.

Eye movements due to linear accelerations in the rabbit.

1. Compensatory vertical or torsional eye movements of rabbits caused by linear accelerations along the transverse or sagittal axis were measured. Sinusoidal accelerations (parallel swing) in a frequency range of 0-068--1-22 Hz and acceleration steps (linear track) of 0-02--0-11 g were applied. 2. On the parallel swing, properties of the maculo-ocular reflexes were similar for transverse and sagittal acceleration. Gain (rotation of eye/rotation of the resultant linear vector) proved to be very low: about 0-1 for 0-3 Hz and smaller than 0-01 for frequencies above 1-0 Hz. The decrease in gain was accompanied by an increase in phase lag to about 180degrees. No non-linearity was revealed by the use of different amplitudes (10--30 cm). 3. On the linear track, eye deviation after an acceleration step took many seconds to develop fully. Gain increased with time and was about 0-65 after 5 sec. 4. The results indicate that the responses of the otoliths, as reflected in maculo-ocular reactions, are very slow. Fluctuations in the direction of gravity seem to be averaged over several seconds by the system. This may explain that erratic linear accelerations(frequency greater than 1 Hz) during locomotion or transport do not lead to eye movements or disorientation.