Marfan's Syndrome and Hearing Organ

Abstract

An eleven-month-old girl, delivered prematurely in breech position, presented multiple congenital anomalies summing up to Marfan's syndrome (arachnodactyly, a mesodermal dystrophy). The most conspicuous abnormal finding, in the sectional series of the right hearing organ, was a bony lip that protruded from the external aperture of the vestibular aqueduct and caused considerable narrowing of this orifice. It could be considered, by damming up the endolymph current, the cause of dilatation of the utricle and rupture of the saccular wall. Marfan's syndrome is generally associated with the hearing organ only when deformities of the external ear are observed. Study of the middle and the inner ear may find in these areas components of the syndrome, enroling the latter among the sources of congenital deafness.