What is cleft lip and palate?
Cleft lip is a term used to describe a notch, opening or separation in the upper lip. Cleft palate describes an opening or separation in the roof of the mouth, which is either easily seen, or is covered by the lining of the roof of the mouth (submucous cleft palate).
Cleft lip and palate are common and affect about one baby in every 700 births. The degree to which children are affected and the extent of the clefts and their impact on the child varies.
Cleft lip and palate can each occur alone or together. Of the babies born with cleft lip and/or palate, around:
- 45% have a cleft palate
- 24% have a cleft lip
- 31% have both a cleft lip and palate.
A cleft lip, with or without a cleft palate, is more common in boys. Cleft palates on their own are more common in girls. Cleft lip and palate, and cleft palate alone may be more common in babies with close relatives who also have it.
Children who were born with a cleft lip alone are not normally at a greater risk of long-term deafness than the general population. However, children with cleft palate or cleft lip and palate are likely to have longer-term difficulties with their hearing.
What is the cause of cleft lip and/or palate?
We all start out life with a cleft lip and palate. During early pregnancy these clefts normally fuse together. The exact reasons for isolated cleft lip and/or palate are unclear, although it is usually a random, one-off event.
Sometimes cleft lip and/or palate are diagnosed during routine antenatal scans. Sometimes they aren't known about until birth. When the cleft lip and/or palate are diagnosed you will be referred to a specialist regional cleft lip and palate service.
A team of professionals will be involved in the care and treatment of your child. This team may include:
- a specialist cleft nurse
- an audiovestibular (hearing and balance) physician
- an ear, nose and throat (ENT) surgeon
- a maxillofacial (head, neck, face, jaws) surgeon
- a plastic surgeon
- a speech and language therapist
- an orthodontist
- an audiologist
- a psychologist
- a paediatrician.
The team will also have access to a geneticist and other professionals who may need to be involved in the overall care of your child. The team will discuss with you the treatments your child will need and together you will develop a care plan.
As part of your child’s care plan, it’s important that they have their hearing assessed. This may be done at the regional centre for cleft palate or they may refer you to your local ear, nose and throat (ENT) and audiology service.
This will normally be done routinely shortly after birth. Your audiology team will regularly assess your child’s hearing. This should be at least once a year during the first three years, and again around the time your child starts school, and at ages five and 10 years.
Types of deafness and cleft palate
Glue ear is the most common type of deafness in children with cleft palate. It's a type of conductive deafness – when sound can’t pass efficiently through the outer and middle ear to the inner ear (cochlea and auditory nerve).
Glue ear is a build-up of sticky fluid in the middle ear. It may not cause any problems in hearing or it may cause a mild to moderate deafness (20–60 dB) in the affected ear.
For most children without a cleft palate, glue ear is a temporary condition that they grow out of by around eight years old as their eustachian tube and other cavities grow larger.
However, in children with a cleft palate there are likely to be additional structural abnormalities of the eustachian tube and the muscles may not work as well. Almost all children with a cleft palate will get glue ear before the age of one and it may persist much longer than for other children. For this reason children should be monitored closely.
Sensorineural deafness is unusual in children with a cleft palate. This type of deafness may indicate an associated syndrome or, rarely, a co-existing condition (two or more unrelated conditions happening together).
Mixed deafness is when children who have conductive deafness, such as glue ear, also have sensorineural deafness.
Grommets and cleft palate
Your surgeon may recommend putting in grommets at the same time as the surgery to repair the cleft palate.
In older children grommets may be offered if the glue ear hasn’t cleared up spontaneously following the palate muscle repair.
Children who have a cleft palate are much more likely to have recurring glue ear after the grommets have come out. It is possible to put in grommets up to three times but it isn’t good practice to carry out repeated grommet surgery after this. This is because every time a grommet is put in the eardrum has to be punctured, and every time one falls out it leaves a tiny scar.
Cleft palate and hearing technology
Some ear, nose and throat (ENT) doctors and audiologists recommend hearing aids as the preferred management for children with glue ear who also have a cleft palate.
The type of hearing aid that will be suitable for your child will depend on the type and level of deafness they have and whether the outer ear is fully formed.
A hearing aid sits on the top of the pinna (the outside part of the ear) and is connected to an earmould that is specially made to fit in the child’s ear. Children who have a syndrome associated with a cleft palate may have a very small pinna that can make it difficult to keep a behind-the-ear hearing aid in place. Smaller ear canals can sometimes make it difficult to get well-fitting earmoulds.
Narrow ear canals can cause sound from a behind-the-ear hearing aid to ‘bounce’ back off the wall of the ear canal, causing whistling or ‘feedback’ from the hearing aids. If your child has any of these problems, talk to their audiologist about possible solutions.
Children who have conductive hearing loss and have problems wearing behind-the-ear hearing aids may benefit from a different type of hearing device that allows the child to hear sounds using bone conduction.
Technology at school
Children with a cleft palate may be more prone to middle ear infections. This is caused by a combination of factors that can encourage bacterial growth in the middle ear. Ear infections can occur with or without glue ear.
What to do if your child has an ear infection
Any discharge (leak) from the ear is abnormal. If it occurs you should see your GP, who may prescribe oral antibiotics or antibiotic drops. Any prolonged discharge from the ear should be assessed by your ear, nose and throat (ENT) doctor.
Swimming and ear infections
Some children appear to get ear infections triggered by swimming. Most doctors feel it’s important for children to learn to swim and enjoy swimming rather than worry about possible infection. If your doctor permits swimming, try to stop dirty water entering your child’s mouth or being swallowed. Avoid letting your child swim in lakes or non-chlorinated pools as the water usually has a high bacteria count.
Most children with a cleft palate or cleft lip and palate have no other medical problems associated with it. However, cleft lip and palate occurs in more than 400 syndromes. ‘Syndrome’ means a collection of signs or symptoms that commonly occur together, which doctors recognise as being related.
How do syndromes occur?
Syndromes may be caused by changes to genes, known as mutations, which are inherited (passed on) from one or both parents to the child. Sometimes the change in the gene happens in the child for the first time. This is known as a new or spontaneous mutation.
About 30% of children born with cleft lip and/or palate have a syndrome. Here is a list of the commonest syndromes and where you can go for more information and support.
- Van der Woude syndrome – www.clapa.com
- Pierre Robin Sequence (also known as Pierre Robin syndrome or PRS) – www.clapa.com
- 22q11.2 deletion syndrome (sometimes known as DiGeorge syndrome and Velocardiofacial syndrome) www.maxappeal.org.uk
- Stickler syndrome – Stickler Syndrome Support Group www.stickler.org.uk
- Down’s syndrome – and visit the Down’s Syndrome Association’s website www.downs-syndrome.org.uk
- CHARGE syndrome – The CHARGE family support group www.chargesyndrome.org.uk
- Hemifacial microsomia (Goldenhar syndrome) – Goldenhar UK www.goldenhar.org.uk
- Treacher Collins syndrome – Treacher Collins Family Support Group www.treachercollins.net
- Kabuki syndrome – Kabuki UK www.kabukiuk.org.uk.
Acknowledgements and references
We are grateful to Mr Raouf Chorbachi, Consultant Audiovestibular Physician with Great Ormond Street Hospital for Children NHS Trust and North Thames Regional Specialist Cleft Lip and Palate service for his guidance in preparing these webpages.
Full references for this webpage are available by emailing [email protected].