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Revista CEFAC

Print version ISSN 1516-1846On-line version ISSN 1982-0216

Abstract

GOMES, Isabela et al. Speech-language aspects on Crouzon syndrome: case study. Rev. CEFAC [online]. 2008, vol.10, n.3, pp.303-310. ISSN 1982-0216.  https://doi.org/10.1590/S1516-18462008000300005.

BACKGROUND: to describe the results of speech, language, cognition, stomatognathic system and hearing assessments of a case of Crouzon syndrome at the age 6:4 years. PROCEDURE: the subject carried out the following evaluations: ABFW, Test of Receptive Vocabulary, Language-Cognition Development Evaluation, Evaluation of Structures and Functions of the Stomatognathic System, pure-tone audiometry threshold, immitance measures and vocal audiometry. RESULTS: the pure-tone audiometry identified bilateral moderate conductive hearing loss, compatible with vocal audiometry's and immitance measures' results. The stomatognathic system evaluation showed that the structures had reduced tonus and altered posture and mobility. Suction, chewing, deglutition and breathing functions were also altered. Phonologically, the following processes were identified: Cluster Simplification, Stopping of Fricatives and Others. In the Fluency evaluation, subject's performance was below the expected scores for matched age and gender. In the Pragmatics test, the child had 14.4 acts per minute and, predominantly, gestural communication. The Receptive Vocabulary Test showed scores 7.1% below reference. In the Expressive Vocabulary Test, data indicated a performance compatible to the reference values of 4 and 5 year-old children, below the expected scores for the subject's age. Regarding language and cognition, the analysis indicated a gap between the child's performance and the developmental level. CONCLUSION: the deficits caused by the syndrome are diffuse and interconnected. The present study had the aim to present the Speech-Language and Hearing Pathology associated aspects of a Crouzon syndrome case and to provide initial data to further investigate these aspects and the intervention process.

Keywords : Craniosynostoses; Child Language; Cognition; Stomatognatic System; Hearing.

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