section_path: Clinical significance/Congenital abnormalities
section_name: Congenital abnormalities
paragraph_text: Congenital abnormalities
  
  Anomalies and malformations of the auricle are common. These anomalies include chromosome syndromes such as ring 18. Children may also present cases of abnormal ear canals and low ear implantation. In rare cases, no auricle is formed (atresia), or is extremely small (microtia). Small auricles can develop when the auricular hillocks do not develop properly. The ear canal can fail to develop if it does not channelise properly or if there is an obstruction. Reconstructive surgery to treat hearing loss is considered as an option for children older than five, with a cosmetic surgical procedure to reduce the size or change the shape of the ear is called an otoplasty. The initial medical intervention is aimed at assessing the baby's hearing and the condition of the ear canal, as well as the middle and inner ear. Depending on the results of tests, reconstruction of the outer ear is done in stages, with planning for any possible repairs of the rest of the ear.
  
  Approximately one out of one thousand children suffer some type of congenital deafness related to the development of the inner ear. Inner ear congenital anomalies are related to sensorineural hearing loss and are generally diagnosed with a computed tomography (CT) scan or a magnetic resonance imaging (MRI) scan. Hearing loss problems also derive from inner ear anomalies because its development is separate from that of the middle and external ear. Middle ear anomalies can occur because of errors during head and neck development. The first pharyngeal pouch syndrome associates middle ear anomalies to the malleus and incus structures as well as to the non-differentiation of the annular stapedial ligament. Temporal bone and ear canal anomalies are also related to this structure of the ear and are known to be associated with sensorineural hearing loss and conductive hearing loss.