Diagnosis and treatment of speech disorders in children with Moebius syndrome

Int J Pediatr Otorhinolaryngol. 2020 Nov:138:110316. doi: 10.1016/j.ijporl.2020.110316. Epub 2020 Aug 15.

Abstract

Background: Moebius syndrome (MS) is characterized by congenital bilateral paralysis of the facial and abducens nerves. Clinical features include feeding problems, dysarthria, dysphagia, sialorrhea, strabismus, and lack of facial expression. Patients with MS frequently present with dysphagia during infancy. Further on during childhood a severe speech disorder is a common feature. However, articulation deficits in patients with MS are scarcely reported in the related scientific literature.

Objective: The aim of this study is to describe speech deviations, intelligibility and sialorrhea in patients with MS.

Material and methods: Eighty-seven patients with MS were prospectively studied. Age ranged from 4 to 18 years. A complete Speech and Language Pathology (SLP) evaluation was performed in all cases. The evaluation focused on articulation placement, sialorrhea and intelligibility of speech.

Results: Sialorrhea was detected in 23% of the patients. Abnormal articulation placement of bilabial phonemes was observed in 68% of the patients. Another 50% of the patients presented with articulation placement errors in other phonemes. Intelligibility was classified as adequate in 18% of the cases. Mildly affected intelligibility was found in 51% of the patients. Speech was considered moderately unintelligible in 20% of the cases. Unintelligible speech was found in 11% of the patients.

Conclusions: From the results of this prospective study it can be concluded that a high percentage of patients with MS are at high risk of presenting with moderate to severe speech disorders. Thus, an early SLP intervention should be provided for this population in order to enhance speech development and reducing the risk of severe oral communication impairments.

Keywords: Congenital anomalies; Facial paralysis; Moebius syndrome; Speech.

MeSH terms

  • Adolescent
  • Articulation Disorders
  • Child
  • Child, Preschool
  • Humans
  • Mobius Syndrome* / complications
  • Mobius Syndrome* / diagnosis
  • Mobius Syndrome* / therapy
  • Prospective Studies
  • Speech
  • Speech Disorders / diagnosis*
  • Speech Disorders / etiology
  • Speech Disorders / therapy
  • Speech Intelligibility