Congenital deafness is described as deafness that exists at birth. Factors responsible for this condition include those present during pregnancy (such as hereditary factors), as well as factors present after pregnancy. Hereditary congenital deafness can be transmitted, sensorineural or even a combination of the two. The extent or progression of this type of hearing loss varies depending on each individual's case. According to Richard Smith, congenital hearing loss is "syndromic (associated with malformations of the external ear or other organs or medical problems involving other organ systems) or non-syndromic (no associated visible abnormalities of the ear external or any associated medical problem)…” More than 400 genetic syndromes are associated with congenital deafness. These include Treacher Collins, an autosomal dominant disorder, and Down syndrome, an X-linked hearing loss. Although congenital hearing loss can be difficult to live with, hearing aids, surgery, and therapy are all available as forms of treatment. Congenital deafness must be treated as early as possible to avoid as many delays in the child's language development as possible. Congenital deafness can be the result of several causes. As mentioned earlier, this is a type of hearing loss that is present at birth. There may be hereditary factors that play into this type of hearing loss, as well as other prenatal or birth factors. Fifty percent of congenital hearing incidents in children are all caused by genetic factors. These can be autosomal dominant, autosomal recessive, or linked to the sex chromosome corresponding...... middle of paper ......n the processing process because it is the primary means of delivering undistorted sound waves directly to the inner ear. The problem for patients with this disorder tends to be malformations of the outer and middle ear. By bypassing these two areas, sound can be successfully transmitted directly to the inner ear via a bone anchored hearing aid (BAHA). The BAHA consists of a sound processor that captures sound waves and transmits them to the external pillar. The vibrations then pass through this pillar and enter the titanium implant which works by direct bone conduction, via the skull bone which is embedded inside and stimulates the nerve fibers of the inner ear. Malformations of the outer and middle ear can be treated with reconstructive surgery. The success of this approach varies depending on the severity of different aspects of the individual's abnormalities...