Sequential treatment of speech disorders in velocardiofacial syndrome patients: an 8-year retrospective evaluation.

Background: Speech disorders are the most common presentations of patients with velocardiofacial syndrome (VCFS) and are difficult to be treated with very good treatment outcome. The purpose of this study was to evaluate the clinical diagnosis and outcomes of sequential treatment of therapy for VCFS.

Methods: A retrospective study of 120 patients (ages ranged from 4.3 to 38 years old, with a mean age of 10.2 years) was conducted and thoroughly reviewed retrospectively oral speech evaluation, oral examinations, and lateral cephalometry of 33 patients. Comparison was made in 33 patients (age range, 4-17 years; mean age, 7.24 years); patients were compared with and age-matched controls, using IQ scores and speech intelligibility tests. A Chinese speech intelligibility test and blowing test were also used to evaluate the outcomes of modified pharyngeal flap surgery, behavioral therapy, and speech therapy.

Results: The average age of the primary diagnosis for VCFS was 13.9 years. No cleft palate and reduced mobility in pharyngeal function were found by oral examination. Speech intelligibility in the 33 patients with VCFS was 33%, with an average IQ test score of 67. Chinese speech intelligibility of 33 patients who underwent pharyngoplasty and speech therapy was improved from 47% to 98%, and the duration of blowing test increased from 17 to 38 seconds. The average length of therapy period was estimated to be 8 months.

Conclusions: Hypernasality and reduced mobility in palate and pharyngeal structures of VCFS patients could be measured with Chinese speech intelligibility test and blowing tests. The pharyngeal flap surgery and the behavioral therapy are proved to be an effective protocol for VCFS.