Dk Gupta

The syndrome of Goldenhar (oculo-auricular dysplasia).

The syndrome of Goldenhar is characterized by a triad of anomalies comprising epibulbar dermoids, accessory auricular appendages and aural fistulae. The condition is unilateral, occurs only sporadically and is sometimes associated with other ocular anomalies like coloboma of the upper lid, microphthalmos and ocular colobomata. More rarely other facial and widespread nervous, muscular and skeletal anomalies may be in association with the ocular dermoids and aural deformities (Duke-Elder, 1964). Though external aural deformities are a part of this syndrome middle ear anomalies giving rise to congenital deafness must be very rare. We have not come across any reference to this in the literature available to us. Moreover, the association of congenital deafness and facial hemiatrophy has an interesting embryological explanation worth recording.

ORBITAL AFFECTION IN NASAL AND PARANASAL NEOPLASMS

Neoplasms of the nose and paranasal sinuses affecting the orbit comprise a heterogenous group which are encountered quite infrequently by the average practising ophthalmologist. Though such neoplasms usually affect the orbit in the late stages, involvement of it at an early stage is not unknown. The prognosis is much better where the orbital involvement is merely due to associated inflammatory reaction but in most cases the tumours grow to encroach upon the orbit or actually involve the orbital tissues. Eye findings are not only indicative of the extent but also of the site of the primary lesion which itself may be inaccessible to clinical examination. In some eases the orbital involvement is as a result of treatment of the tumour, surgical or irradiational, and it is the duty of the ophthalmologists to apprise their E. N. T. colleagues of such post-operative and post-radiational orbital and ocular complications that may accrue eventually.