Some affected children show delays in attaining developmental and speech milestones. Mental ability and motor coordination is normal or borderline normal. Most people with MGS have normal intelligence and learning ability.
The lifespan of those with MGS may depend on the severity of the symptoms experienced by each person. The oldest documented adult is a 65-year-old woman. Most people with MGS have a normal life expectancy.
Facial features may include full lips and a narrow nose with a small jaw and mouth. In addition to those mentioned above, facial features can include a triangular face, an arched roof of the mouth and/or droopy eyelids. Specifically, hands can have the fifth finger be abnormally bent, and/or one or more fingers may be permanently flexed. There have also been patients with detachment of cartilage and bone tissue from the surface of a bone, unusually extended joints, and abnormally stiff joints that may lock in position. There have been some patients with an absent or abnormally shallow depression on the upper arm bone located where the bone meets the shoulder. Elbows can be dislocated, collar bones can be hook-shaped and/or long bones can have flat ends. These may include slender, missing, or unusually shaped ribs with slender arm and leg bones. People with MGS may have differences in their bone development. The following symptoms are associated with MGS, but not everyone diagnosed with MGS will have all of these symptoms. This syndrome has a range of symptoms with severity anywhere from mild to severe. As a result, these babies have low birth weight and can have feeding difficulties that result in a slower development and short stature. Short stature is due, in part, to the slow growth that can occur before and after birth in babies with MGS. This may cause an unstable knee joint which could lead to chronic knee pain. The kneecaps may be small or even missing. They may have narrower or absent ear canals which could affect their hearing. The ears can also be low-set and/or unusually formed.
People with MGS can have small or absent outer ears (microtia). These features can be seen at birth and are discussed in more detail below. The main clinical features of MGS are small ears, kneecap abnormalities and short stature. 5 Myths About Orphan Drugs and the Orphan Drug Act.Information on Clinical Trials and Research Studies.View our Photo Gallery of Before and After Infant Ear Molding at EarWell Centers of Texas. Please view our list of educational ear deformity videos in the EarWell Video Gallery. The EarWell™ Infant Ear Correction System, an FDA-approved, non-surgical, painless solution for correcting ear deformities in the first two weeks of life may have precluded one from having to operate on children with Stahl’s ear. Please view the photo of adult (older child) Stahl’s ear by Yotsuyanagi requiring surgical release of the offending muscle and then the difficult process of cartilage reshaping. At the same time the normal superior crus is restored. EarWell Infant Ear Correction System therapy stretches out the offending muscle (auricular transverse muscle) and restores cartilage shape so as to eliminate the transverse crus thus avoiding the angulated and pointed Spock ear look. Adult Stahl’s ear requiring surgical releaseĪdult anatomic and surgical studies (Yotsuyanagi PRS July 2015) have affirmed that the Stahl’s deformity is largely related to the abnormal insertion of the auricular transverse muscle which creates the abnormal transverse crus crossing the scapha. Please compare the normal ear in photo to right in to Stahl’s ears in gallery below. The Stahl’s infant ear deformity (Spock Ear or Elf Ear) is characterized by a transverse crus (see arrow in photo) extending outward from the antihelix rather than continuing upward in a gentle bend as the superior crus helix. Anatomy of the Stahl’s Ear Deformity in Newborn Infants Stahl’s ear deformity in newborn infants (also called Spock’s Ear or an Elf Ear) is an infant ear deformity characterized by an extra horizontal cartilage fold which gives the ears a pointed shape. Congenital Ear Deformity Microtia and Anotia.