Congenital deafness is deafness that occurs at birth. It is affects 1 in every 1,000-2,000 births. Congenital deafness can be due to non-syndromic and syndromic causes. Non-syndromic means that the deafness is caused due to genes and is not involved with other disorders. This is about 80% of the congenital hearing loss. It can be due to autosomal dominant genes where the gene is passed directly from the generations and usually only one parent needs to express it or autosomal recessive where both parents must express the gene. Syndromic accounts for the other 20% of congenital deafness and can happen because of many other disorders other than the involvement of genes. Some of these syndromes include mitochondrial diseases, Waardenburg syndrome, X-linked CMT, and Alport syndrome, just to name a few.