Speech, Language and Cognitive Delays in Patients with Non-Syndromic Craniosynostosis
Sanjay Naran, MD, Matthew Miller, BS, Benjamin Ware, BS, Liliana Camison, MD, Jesse Goldstein, MD, Joseph Losee, MD.
University of Pittsburgh, Pittsburgh, PA, USA.
Non-syndromic craniosynostosis (NSCS) patients are at risk of neurodevelopmental delay. While many metrics have been analyzed, few have directly examined early language acquisition and speech development. Our purpose was to determine whether infants with NSCS have normal language acquisition and speech development.
Patients with NSCS presenting from 2000-2014 were queried. Those with identified syndromes were excluded. Data elements included; age, gender, Pittsburgh Weighted Speech Score (PWSS), evaluation for anatomic motor delay, language/speech delay, articulation/phoneme deficiency, hypernasality, velopharangeal insufficiency (VPI) or borderline competency, and whether speech therapy was recommended. Diagnosis of a sub-mucous cleft palate (SMCP) was noted. All patients were evaluated by a certified speech and language pathologist.
165 patients met our criteria. 58.2% were male. Average age at time of most recent speech evaluation was 6.2 years (Range: 1.3-17.95). 48.5%% had normal speech/language metrics. 51.5% had one or more abnormalities, including anatomic motor delay/disorder (20.6%), speech/language delay (21.2%), articulation/phoneme deficiency (6.1%), hypernasality (12.1%), and VPI or borderline competency (20.0%). 27.9% (n=31) of patients were recommended to have speech therapy. 17.6% were diagnosed with a SMCP. Of those patients for whom speech/language therapy was prescribed, the average PWSS at most recent follow-up was 2.9 (Range 1-5). Two patients were documented to have global cognitive delay.
We find that almost 1 in 5 patients with NSCS carry a diagnosis of SMCP. Defective speech and language development necessitating therapy occurred in 1 in 4 patients with NSCS; a prevalence 2-3 times higher compared to the general population.
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