SAN DIEGO—Parents often bring their children to see pediatric otolaryngologists, distraught that their child’s speech is hard to understand. These can sometimes be delicate situations, and may or may not be cases for which otolaryngologists are best suited, an expert said here in January at the Triological Society Combined Sections Meeting.
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April 2020Sometimes, these children will show signs of autism, and it is critical to understand what to look for so parents can be properly guided to appropriate care, said Anna Messner, MD, chief of otolaryngology-head and neck surgery at Texas Children’s Hospital in Houston. Her tips came in a session on pediatrics that also touched on the critical ways in which pediatric otolaryngology differs profoundly from adult care, and on how to manage laryngeal cleft.
Addressing Concerns
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The most important features to look for in communication differences among children with autism are not saying any words by 16 months; not responding to their name being called but responding to other sounds, such as a honking car horn; possibly having a good rote memory, especially for numbers, letters, or songs; and losing language or other milestones between 15 and 24 months of age, a worrisome development known as regression.
Dr. Messner said it’s important to be tactful when interacting with parents.
“If everything else checks out, tell the family, ‘This looks like a developmental issue,’” she said. “I personally do not bring up the word ‘autism’ unless the family does first.” She suggested recommending that the family speak to a pediatrician. When she is particularly concerned, especially in cases in which speech or other developmental traits are actually getting worse, she might call the pediatrician herself.
Dr. Messner had other tips for handling speech concerns.
In cases of suspected tongue-tie, or ankyloglossia, it is important to assure parents that it typically does not affect speech, according to an upcoming clinical consensus statement from the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). The statement is expected to be published in May, according to the AAO-HNS.
When parents are concerned that their child can’t say words with “R” or “S,” they should be told that those sounds are “late” sounds, and that it’s normal for those sounds not to be spoken correctly until up to age six, on the same timeline as the sounds “z,” “j,” and “th.”
An important feature to look for in children with difficult-to-understand speech is 22q11 deletion syndrome, brought on by a small deletion from chromosome 22. In these children, the most consistent feature is a fairly flat and fairly long midface, Dr. Messner said.
The syndrome is now easy to diagnose, with fluorescence in situ hybridization (FISH) testing widely available. These children frequently have palatal abnormalities, velopharyngeal insufficiency, and speech and language impairments, but treatments and services such as speech therapy can often help, Dr. Messner said.
“This is something you really don’t want to miss.”
Lasting Consequences
Blake Papsin, MD, MSc, otolaryngologist-in-chief at the Hospital for Sick Children in Toronto, said the crucial ways in which pediatric otolaryngology differs from adult otolaryngology are causes and effects. He encouraged the audience to remember that their care can have far-reaching consequences.
Dr. Papsin pointed to the way screening in newborns has transformed care and dramatically improved outcomes. Early detection and early intervention, a 2010 study showed, can result in near-normal hearing for children with severe to profound sensorineural hearing loss (JAMA. 2010;303:1498-1506).
If everything else checks out, tell the family, ‘This looks like a developmental issue.’ I personally do not bring up the word ‘autism’ unless the family does first. —Anna Messner, MD
Advances in our understanding of the genetic causes of diseases can be an asset in surgery, giving an early alert that a procedure is likely to be more difficult than might otherwise be expected, Dr. Papsin said. He pointed to surgery for branchio-oto-renal (BOR) syndrome and CHARGE, a complex genetic disorder involving an array of physical anomalies, sensory deficits, and behavior traits.
Pediatric otolaryngologists also have to remember the long-term effects that imaging can have, Dr. Papsin said. The field has advanced in being able to get enough information from MRI, he noted, so less CT imaging, which involves radiation exposure, is needed.
“We irradiate a child and their developing brain—they have a lifetime risk of cancer associated with that radiation,” he said. “It’s not big—but it’s not zero.”
Dr. Papsin pointed to “spatial intelligence,” the way in which children gain knowledge through a range of processes, from hearing to vision to proprioception to vestibular function.
“We are the pediatric otologists that have to preserve the capacity of the organ to get language,” he said.
Laryngeal Cleft
Reza Rahbar, DMD, MD, director of the Center for Airway Disorders at Boston Children’s Hospital, said that his outlook and approach in the management of laryngeal cleft, a space between the esophagus and windpipe that allows food and liquid into the airway, has changed over the years.
Often, even after the defect is closed, swallowing problems persist—about half the time in the most severe cases. Dr. Rahbar said he used to explain away these difficulties by pointing to the array of problems seen in patients with laryngeal cleft: pneumonia, G tubes, and cardiac issues, among others.
“I kept telling myself that they fail because these are complex patients,” he said.
Now, he knows that there is more to the story, Dr. Rahbar said. The children aspirate not only because of the anatomic defect, but also because their neuromuscular function is not properly coordinated.
His approach is now more nuanced, he said, with a treatment timeline that depends on function, an appropriate length of time to allow medical management to work, and factoring in parent expectations and choosing an endoscopic or open surgical approach depending on the severity of the case.
“This really changed the whole management of these patients from my perspective.”
Sometimes, he said, “despite our best surgical approach to correct the hole,” swallowing problems persist.
“I have no ability to change this except providing feeding therapy—and time.”
Thomas Collins is a freelance medical writer based in Florida.