Loeys-Dietz syndrome: a Marfan-like syndrome associated with aggressive vasculopathy.

Loeys-Dietz syndrome is a recently-characterised genetic disorder with an autosomal-dominant inheritance due to mutations in the transforming growth factor beta-receptor Type 1 or Type 2 genes. We present a Chinese female neonate with genetically-confirmed Loeys-Dietz syndrome, cleft palate, hypertelorism, and an early dilatation of the aortic root and ascending aorta. This syndrome is associated with an aggressive arteriopathy, with an increased risk of dissection and rupture. Early diagnosis, close monitoring and early surgery may prolong the life in affected individuals. Losartan is an emerging therapy that may help slow down the rate of arterial dilatation.

A self-controlled trial to evaluate the use of active hearing defenders in the engine rooms of operational naval vessels.

INTRODUCTION: Active Hearing Defenders are established hearing protectors with in-built electro-acoustics that shut-off ambient noise while allowing effective communication between users. METHODS: A blinded, self-controlled trial was conducted among naval servicemen to compare the effectiveness of two types of active hearing defenders (Howard-Leight Thunder TM and COM-55) in relation to passive hearing defenders in an operational environment. RESULTS: Subjects felt that the active hearing defenders were more comfortable, durable, and that the active hearing defenders helped them work better. When subjects were tested with a speech discrimination battery (Central Institute of the Deaf, Spondee Word lists), there was a significant difference (p value of 0.04, using the Kruskall-Wallis ANOVA test) between the two active and the passive defenders. However, no significant difference was found between the two types of active hearing defenders. CONCLUSION: Active hearing defenders are an acceptable and efficacious means of hearing protection in noisy environments.