Microtia is a congenital deformity of the outer ear. It results in a very small and underdeveloped pinna and often affects the canal and eardrum as well. This deformity is most often unilateral, found in males, and occurs 1 in every 8,000-10,000 births. There are a variety of types and suspected causes of this deformity as well as treatment options and complications.
Types of these are divided into four grades, with I being the least severe. Grade I includes a noticeably small pinna with identifiable structures and intact external canal. Grade II consists of a small partial lobe with a stenotic or closed external ear canal and results in conductive hearing loss. Grade III is the most common type and includes those with a small vestigal structure. Grade IV is the most severe and is also called anotia: total absence. Microtia occurs more commonly in males and most often on the right side. Approximately 10% of cases may occur bilaterally.
While this may occur as an isolated congenital deformity, it often presents with a spectrum of other minor or major defects. Syndromes that typically include microtia include hemifacial microsomia, Goldenhar syndrome, and Treacher-Collins syndrome.
This disorder does not have a specifically defined cause and potential theories are difficult to prove. A decreased blood supply to the developing embryo in-utero may be to blame and can be caused by anything from smoking to excess caffeine intake. Medications such as Accutane, Thalidomide, and retinoic acid have also been identified as potential causes and many environmental factors are under suspicion.
Treatment options for this deformity vary and are dependent on whether the inner ear is intact and the hearing is normal. Children born with bilateral microtia are managed more aggressively and often earlier than children with intact audibilty on one side. This is to ensure the children receive bone-conduction aids within the first few months of life if necessary. Surgery may even be started as early as 4 years of age. Many vital and important developmental milestones rely on intact hearing in the first few years of life.
Children born with unilateral microtia often do not receive surgery until 8 or 10 years of age. Many surgeons feel this is an appropriate age for rib cartilage grafts and closer to the adult ear size. Surgical options include auricular reconstruction to restore aesthetic appearance or implantation of a bone-conduction aid to restore hearing. Very often choosing aesthetics and correction of hearing loss is not possible in the same ear. Parents of children with intact audibility in the opposite side may forego the aid and just choose appearance.
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