I went to an ear, nose and throat doctor to discuss surgery for my four-year-old's protruding ears, and I mentioned that his speech isn't good. The doctor said there was a deformity in his uvula. It looks more like a "w" than a "u". He said some children are born with cleft palate or cleft lip and that this was somewhat similar. Could you please explain this? How will this affect him in the future? Should this have been determined when he was an infant? What could have been done earlier?

Karyn

What you are describing is a "bifid uvula." "Bifid" means divided or forked. Bifid uvula is not a significant problem in and of itself; it is mostly significant for its association with a dysfunctional palate.

Before birth, the palate begins as two divided shelves of tissue. The shelves grow toward each other and eventually join in the midline. ("Midline" refers to an imaginary line that divides our bodies into left and right halves.) There is muscle within these shelves; when the shelves join, the muscles interdigitate (interlock).

If the shelves fail to join, the child is born with a cleft palate. When the shelves join but the muscles fail to interdigitate, the child is said to have a submucous cleft palate. In other words, below the mucous membrane, the muscular portion of the palate is still separated. Frequently, bifid uvula is a clue to the presence of a submucous cleft palate.

Interdigitation of the muscle is important to the palate's function, so submucous cleft palates are inefficient compared with normal palates. This can cause problems with speech, swallowing and eustachian tube function (leading to frequent ear infections).

Should this have been noticed at birth? Not necessarily; bifid uvula can be a difficult to spot in a newborn, and often submucous cleft palate can be detected only by careful palpation (by feeling it with the hand). As the infant grows into a toddler, these problems are easier to detect. It is not uncommon for bifid uvula and/or submucous cleft palate to go unnoticed until the toddler years.

If it had been detected, could anything have been done about it? One can operate on a cleft palate, but submucous cleft palate cannot be repaired. It is certainly useful to know that one's child has this condition. (By the way, bifid uvula is not always associated with submucous cleft palate. Your son needs to be appropriately examined to determine this.) Armed with this knowledge, you would be more likely to seek early intervention for problems with speech, swallowing and ear infections.

One last comment: You also mentioned that your son has protruding ears. If he has protruding ears and a bifid uvula, there is a chance that he has a congenital (inborn) syndrome. There are hundreds of syndromes that involve subtle malformations of facial and oral anatomy. In many of these syndromes, there may be subtle abnormalities of other organs. For example, one might find kidney malformations together with the ear/uvula malformations you have described. These other abnormalities could have great significance to his health, now or in the future. For this reason, I recommend that you take your son to a medical geneticist for a thorough evaluation.