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Children with cleft palates and craniofacial defects may have an increased incidence of ear disease and hearing loss.
The hearing loss may occur intermittently or could become permanent and varies from mild to severe. Hearing loss can have a significant adverse influence on speech and language development, and educational and psychological status.
Children with cleft palates require regular ear exams and audiological evaluations beginning at 1 month of age and continuing through adolescence by the Cleft-Craniofacial Center team and their pediatrician.
Before we schedule a cleft-lip and/or palate repair, most infants see an Ear, Nose, and Throat (ENT) specialist at the Cleft-Craniofacial Center. We evaluate whether to include ear tube placement with their cleft repairs during this appointment. Any other procedure for airway concerns may also be able to be coordinated.
ENT physicians from UPMC Children's Hospital of Pittsburgh see patients within the Center when available or we may coordinate evaluations within the ENT clinic on the same day as your Center appointment. You may also make an appointment at UPMC Children's Hospital or at one of our outreach locations to see one of the doctors. Our ENT specialists see patients throughout the community and at multiple UPMC Children's outpatient locations.
Children with cleft palates require frequent hearing tests due to the increased risk of hearing loss, which may occur with middle ear fluid or pressure changes in the middle ear space.
Due to the child's structural differences, they are at risk for recurrent middle ear fluid problems. Doctors need to monitor children with tubes to ensure that they remain in place and are open to function properly.
Fluctuating hearing loss frequently occurs in children with clefts and must be monitored to prevent damage to the ear or problems with speech, language, and learning.
At UPMC Children's, audiologists test hearing many different ways depending on a child's age and development. We typically test infants and toddlers in a sound-treated booth by presenting sounds and words through speakers. Young children respond in specific ways that allow the audiologist to determine what they hear and at what loudness level. Some of these evaluated behaviors may include:
As the child grows older, they may begin to identify body parts, point to pictures in the booth, or repeat words with or without earphones in place depending on their maturity level.
Once the child tolerates headphones, they will begin to respond by indicating whether he hears a sound by raising their hand or placing marbles in a bowl. They also may be asked to repeat words to determine their hearing level for speech.
Besides testing the hearing level, the audiologist also performs a middle ear test and tympanometry. Doctors place a small rubber probe in the ear during this exam, which emits some air pressure and an audible tone. The machine can then measure the middle ear space's pressure, how well the eardrum moves, and ear canal volume.
These measurements allow the audiologist to determine if there is fluid or abnormal pressure in the middle ear space, impacting hearing. Additionally, the canal volume measure can evaluate whether a ventilation tube is in place and functioning correctly. This information allows the nurse practitioner and doctor to manage any ear problems identified.
The soft palate is part of the velopharyngeal mechanism, made up of the palate, sides, and back of the throat. These are muscular structures that move together to form an airtight seal during the production of specific speech sounds and to prevent nasal regurgitation of foods and liquids into the nasal cavity during swallowing.
During the production of all English phonemes except the nasal sounds (m, n, ng), the palate moves up and back to close against the back of the throat. At the same time, the sidewalls of the throat squeeze inward to form a complete seal. This gives a complete separation from the mouth and nose.
Velopharyngeal insufficiency results from incomplete closure of the velopharyngeal mechanism. It results in nasal air emission during speech, hypernasality (talking through the nose), and altered speech sound production due to this inability to maintain air pressure in the mouth.
Approximately 20 percent of children with a repaired cleft palate will develop some symptoms of velopharyngeal insufficiency.
Surgeons correct this problem with a second procedure to lengthen the palate or to block airflow into the nose.
Children with clefts do not always have difficulty with speech. Many children with clefts develop speech on a normal developmental timeline and never require speech therapy.
Most children diagnosed with cleft-lip without cleft palate have regular speech unless they have dental issues associated with their gum ridge.
If the teeth are out of place or there are dental gaps, speech sounds produced in that region, (s, z, t, d) may be somewhat distorted.
These same sounds may be distorted in children with cleft lip and cleft palate as well. If these children substitute different sounds for intended sounds, they may require some speech therapy to obtain the correct placement for producing specific speech sounds.
Children with only cleft palate are at risk for speech and language delays. They may also be diagnosed with velopharyngeal insufficiency (VPI). VPI may appear in about 20 percent of children with repaired cleft palates with or without a cleft lip. Speech sound development or articulation may be negatively impacted by velopharyngeal insufficiency.
Speech can be affected in several different ways by VPI. Still, the main characteristics are:
VPI may impact articulation and speech sound development if the child can not maintain good air pressure in the mouth while talking. The child may omit speech sounds move them back in the mouth or into the throat or nose. A direct result of VPI, we refer to these placement changes as articulation compensations. VPI treatment is surgical. It typically involves procedures to rearrange the muscles in the palate to normalize anatomy, palatal lengthening procedures or pharyngeal procedures.
Many children with VPI may require speech therapy before and after surgery to obtain correct placement of articulation. Your speech pathologist at the Cleft-Craniofacial Center and your surgeon will determine the most beneficial treatment plan based on your child's needs.
Speech therapy may be provided at UPMC Children's or through an educational agency or clinic in your home area. If you have questions about the need for speech therapy or how to obtain services contact the Center.
The speech pathologist monitors all patients with cleft palates regularly to ensure adequate speech and language skills and palate function. These evaluations determine if your child will need additional palate surgery or speech therapy services.
At times, we may recommend diagnostic tests for velopharyngeal competency. They may include an x-ray study called a Televex or an endoscopic procedure called a Nasendoscopy.
Both of these assessments allow us to see the velopharyngeal mechanism structures to determine how they work during speech. These tests allow for the most effective diagnosis and treatment planning.
A Televex brochure and Nasendoscopy brochure are available if you have any questions about these tests. The brochures outline the procedures and what to expect during an evaluation.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
UPMC Children’s Hospital of Pittsburgh
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
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