Newborn Hearing Screening for Patients with Orofacial Clefts

Emily Gallagher, MD, MPH
Assistant Professor | Craniofacial Center
Department of Pediatrics
Seattle Children’s

Cleft lip and palate is one of the most common birth defects, occurring in about 1 in 750 births.  An intact palate is crucial for normal feeding, speech, and hearing, yet determining a child’s hearing status at birth may be complicated if a cleft palate is present.  Children with cleft palate usually have Eustachian tube dysfunction and develop middle ear effusions with conductive hearing loss.   They also have a slightly increased risk of having sensorineural hearing loss.  Tympanostomy tubes are usually placed with the palate repair (around a year of age), but this treatment is not effective until the palate has been closed.  Children with cleft palate who do not pass the newborn hearing screen may not have a definitive hearing diagnosis until after a year of age.

There have been few publications about newborn screening results for patients with orofacial clefts, and the range of severity of hearing loss during the first year of life has not been well characterized.  Some small studies have reported that 25-28% of newborns with cleft palate do not pass the newborn hearing screen[i],[ii].   Although providers may be tempted to blame the results on the cleft palate, these patients should be referred for diagnostic testing and to Early Intervention by 6 months.  Most patients will continue to have hearing followed through a multidisciplinary craniofacial team, but patients receiving care without an established team may not have adequate management of hearing loss.

In 2016 in Washington State, there was a discrepancy in age at diagnosis of hearing loss for children with craniofacial malformations.  These children were less likely to have a definitive diagnosis of hearing loss before 3 months of age compared to children without craniofacial conditions and were older when they received a diagnosis.  Children with cleft lip and palate have an increased risk for speech delay and poor academic outcomes compared to unaffected peers.  While there are many potential reasons for these differences, hearing loss may be a key factor.  Providers must be vigilant about timely referrals to Early Intervention Services and medical support for this vulnerable group to help them reach their full developmental potential.

[i] Jordan VA and Sidman JD. Hearing Outcomes in Children With Cleft Palate and Referred Newborn Hearing Screen. 2014; The Laryngoscope; 124:(e384-388).

[ii] Tan EEK, Hee KYM, Yeoh A, Lim SB, Tan HKK, Yeow VKL, Daniel LM.  Hearing Loss in Newborns with Cleft Lip and/or Palate. 2014; Ann Acad Med Singapore; 43(7):371-377.