ENT Aspects of Marfan Syndrome

Abstract

The clinical findings in the ear, sinuses, nose and throat display the same great variability in expression as found throughout the rest of the body in Marfan syndrome [1]. Important skeletal characteristics which contribute to ENT problems include a long narrow face and skull (dolichocephaly, often associated with mandibular prognathism, high arched palate, crowded teeth, prominent supra-orbital ridges, deep-set eyes and frontal bossing [2]. The ears are often large, simple, low-set and posteriorly rotated. Ear canals can be narrow and angulated upward and forward. Unilateral hearing loss is not uncommon, although the cause is obscure, possibly due to ossicular malformation during development, but most likely due to recurrent or chronic otitis media in childhood.