HEARING IN KNIEST SYNDROME

Abstract

Kniest syndrome is a type of dwarfism that is characterized by unusually short arms and legs,a round face with hollow or depressed areas, swelling and stiffness of the joints and a stiff drawing up (contractures) of the fingers. Cleft palate, abnormal curvature of spine (scoliosis), and vision and/or hearing problems may also occur. Intellect is usually normal in people with this syndrome.In this case report we present the audiologic findings of an eight-year-old boy with Kniest syndrome.Key Words: Kniest syndrome, Hearing loss, Audiological evaluation