Schwannomatosis in a child--or early neurofibromatosis type 2.

A case of multiple cervical schwannomas in a five-year-old boy, without other evidence of neurofibromatosis type 2, is described. Schwannomatosis is a disorder characterized by the presence of multiple schwannomas in the absence of neurofibromatosis type 2 that has only been recognized in the last 15 years. The clinical and genetic features of neurofibromatosis types 1 and 2 and schwannomatosis are compared and contrasted. This patient with possible schwannomatosis is presented to illustrate the potential pitfalls of making this diagnosis in the paediatric age group and to increase awareness of the debate on whether this is a distinct entity or a form fruste of neurofibromatosis type 2.

Cavernous haemangioma of the internal auditory canal.

Cavernous haemangiomas are rare lesions of the cerebello-pontine angle that can mimic the more commonly occurring vestibular schwannoma. A case report involving a patient with a cavernous haemangioma of the internal auditory canal (IAC) highlights this as a diagnostic possibility for lesions of the IAC by comparing and contrasting the clinical and radiological findings with the more commonly occurring vestibular nerve and facial schwannomas. Symptoms such as hearing loss and facial paralysis that are disproportionate to the size of the lesion or fluctuate with hormonal changes such as those seen in pregnancy are suggestive of haemangioma. Radiological imaging demonstrating a lesion enhancing with gadolinium and containing areas of calcification is also suggestive of haemangioma. It is important to consider the possible diagnosis of haemangioma as early recognition of this entity may improve the chances of preserving the functional integrity of the facial nerve.

The nasal valves: changes in anatomy and physiology in normal subjects.

There is confusion in the literature concerning the physiology and pathology of the nasal valve, and some debate as to whether there is one valve or two. In an attempt to clarify these uncertainties we have measured nasal function by assessing nasal minimum cross-sectional area, inspiratory resistance and peak inspiratory flow under baseline conditions and after the application of a topical vasoconstrictor. These measurements were then repeated following the application of external and internal nasal splints. Whatever test was employed the results showed that vasoconstriction tended to be the most potent stimulus which changed nasal function producing significant expansion of the minimum cross-sectional area, a decrease in inspiratory resistance and an increase in peak inspiratory flow. External splints also increased the minimal cross sectional area but they had no effect on inspiratory resistance or on the tendency of the vestibular rim to collapse at high inspiratory flow rates. The tendency for lower lateral cartilage collapse was, however, prevented by internal splintage using alar dilators. The results of this study suggest that there is an internal valve at the nasal isthmus where the principal alterations in airway patency follow changes in mucosal congestion, and a mobile external valve where airflow is limited by the tendency of the alar cartilages to collapse. These should be considered as separate entities with differing pathophysiology and these differences should be taken into account when treating patients with airway obstruction due to pathology at these sites.

Recent advances in the use of lasers in otolaryngology.

The laser (Light Amplification by Stimulated Emission of Radiation) has been used in Otolaryngology for over 20 years and is by now an accepted part of the armamentarium. A tremendous amount of work is being done in refining existing techniques and developing new ones, and this review discusses some of the recent advances.