Audiological Profile of Children and Young Adults With Syndromic and Complex Craniosynostosis

Jong, T. de; Toll, Martijn S.; Gier, H. H. W. de; Mathijssen, Irene M.J.

doi:10.1001/archoto.2011.115

Cited by 25 publications

(23 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the study by De Jong et al mild or moderate hearing loss (mostly conductive) was found in 28.5% of patients with Crouzon syndrome (De Jong et al, 2011).…”

Section: Crouzon Syndromementioning

confidence: 89%

Genetic Hearing Loss Associated with Craniofacial Abnormalities

Lunardi¹,

Forlì²,

Michelucci³

et al. 2012

Hearing Loss

View full text Add to dashboard Cite

“…In the study by De Jong et al mild or moderate hearing loss (mostly conductive) was found in 28.5% of patients with Crouzon syndrome (De Jong et al, 2011).…”

Section: Crouzon Syndromementioning

confidence: 89%

Genetic Hearing Loss Associated with Craniofacial Abnormalities

Lunardi¹,

Forlì²,

Michelucci³

et al. 2012

Hearing Loss

View full text Add to dashboard Cite

“…Vision is routinely checked with a Snellen chart by our pediatric ophthalmologist. Data of hearing was cross-sectionally gathered as part of another study [5]. Hearing was tested by a pediatric audiologist with pure tone audiometry.…”

Section: Methodsmentioning

confidence: 99%

Health-related problems and quality of life in patients with syndromic and complex craniosynostosis

et al. 2012

Self Cite

View full text Add to dashboard Cite

PurposeWe conducted this study to gauge the health-related problems, quality of life and the performance of the Health Utility Index Mark 3 (HUI-3) in patients with syndromic and complex craniosynostosis. Patients with syndromic and complex craniosynostosis have various physical and mental problems. More insight on these problems, per syndrome, could provide guidance to improve patient treatment and follow-up.MethodsA cross-sectional, comparative study on 131 patients and their parents was performed. Health-related quality of life was measured with the HUI-3 and the Visual Analogue Scale (VAS). All data were compared to a normative Dutch population. Vision, hearing and intelligence were objectively measured.ResultsThe HUI-3 and the VAS were significant lower compared to the normative Dutch population. All syndromes have a high prevalence of vision and speech problems. Cognitive problems were mainly reported in patients with Apert, Crouzon and Muenke syndrome. Ambulation and dexterity problems were seen in Apert, Crouzon, Saethre–Chotzen and complex craniosynostosis. Only patients with Apert syndrome scored significantly worse on pain. The HUI-3 had a medium to strong correlation with the objectively measured outcomes.ConclusionsThe overall quality of life is lower in patients with syndromic and complex craniosynostosis. To improve quality of life, more attention is needed for problems with vision and speech.

show abstract

“…Conductive hearing loss is common in Apert syndrome. This results from chronic otitis media and occasionally from middle ear anomalies (9). The ears are set low and may be enlarged.…”

Section: Treatment Timing and Multidisciplinary Approach In Apert Synmentioning

confidence: 99%

Treatment timing and multidisciplinary approach in Apert syndrome

Ierardo

Ladniak

et al. 2015

ads

View full text Add to dashboard Cite

SummaryApert syndrome is a rare congenital disorder characterized by craniosynostosis, midface hypoplasia and symmetric syndactyly of hands and feet. Abnormalities associated with Apert syndrome include premature fusion of coronal sutures system (coronal sutures and less frequently lambdoid suture) resulting in brachiturricephalic dismorphism and impaired skull base growth. After this brief explanation it is clear that these anatomical abnormalities may have a negative impact on the ability to perform essential functions. Due to the complexity of the syndrome a multidisciplinary (respiratory, cerebral, maxillo-mandibular, dental, ophthalmic and orthopaedic) approach is necessary in treating the psychological, aesthetic and functional issues. The aim of this paper is to analyse the different functional issues and surgical methods trying to enhance results through a treatment plan which includes different specialities involved in Apert syndrome treatment. Reduced intellectual capacity is associated to the high number of general anaesthesia the small patients are subject to. Therefore the diagnostic and therapeutic treatment plan in these patients has established integrated and tailored surgical procedures based on the patients' age in order to reduce the number of general anaesthesia, thus simplifying therapy for both Apert patients and their family members.

show abstract

Audiological Profile of Children and Young Adults With Syndromic and Complex Craniosynostosis

Cited by 25 publications

References 25 publications

Genetic Hearing Loss Associated with Craniofacial Abnormalities

Genetic Hearing Loss Associated with Craniofacial Abnormalities

Health-related problems and quality of life in patients with syndromic and complex craniosynostosis

Treatment timing and multidisciplinary approach in Apert syndrome

Contact Info

Product

Resources

About