Middle and inner ear malformations in two cases of velocardiofacial syndrome

• Main Authors: Tabith Junior, Alfredo, Haetinger, Rainer Guilherme, Silva, Fernando Leite de Carvalho e, Gudmon, Monica de Castro
• Format: Article
• Language: English
• Published: Thieme Revinter Publicações Ltda. 2009-03-01
• Series: International Archives of Otorhinolaryngology
• Subjects: Ear; Hearing loss; Velocardiofacial
• Online Access: http://www.arquivosdeorl.org.br/conteudo/acervo_eng.asp?Id=593

Objective: To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Method: Audiometric evaluation, computerized tomography of the temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome. Results: Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the latter with the use of reformations based on multislice acquisitions on of the computerized tomography of the temporal bones. Conclusion: We consider it to be highly important to carry out a thorough evaluation and monitoring of the hearing evolution, as well as the occurrence of symptoms related to the vestibular function in patients with velocardiofacial syndrome. From the radiological point of view, attention should be given to the use of high quality techniques for the tomographic study of temporal bones.