Audiologic Findings in Achondroplasia

Abstract

Achondroplasia, the autosomal dominant form of skeletal dysplasia that is characterized by short stature, short limbs, a variety of cranio-facial anomalies including a shortened cranial base, frontal bossing of the skull and many midfacial anomalies, is also often characterized by otologic and audiologic disorders as well. A high incidence of otitis media has already been reported by Gorlin (3) and Cohen (1). In 1981, Glass (2) studied a series of patients with skeletal dysplasias, twenty-eight of whom had achondroplasia and ten of whom had other skeletal dysplasias. The objective of this study was to determine whether or not there was a higher incidence of skeletal dysplasia. In addition, the characteristics of hearing loss in those patients with achondroplasia were studied. Glass and his colleagues found a variety of types of hearing loss in their sample, which ranged in age from twenty months to sixty-three years.