Cleidocranial dysplasia-dental disorder treatment and audiology diagnosis

Abstract

<jats:title>Abstract</jats:title><jats:p>A review of numerous case reports was made, in order to demonstrate the possibilities for treatment of dental disorders in patients with Cleidocranial dysplasia (CCD). In this paper, our own report, including a diagnosis of the effect on the auditory system, is presented. In addition to the triad of CCD symptoms that include hypoplastic or aplastic clavicles, impacted and supernumerary teeth, delayed closure of fontanelles and cranial sutures, impairment of the hearing system resulting in conductive hearing loss also occurs. Our own report is based on the case of a 12-year-old CCD patient, in whom Cone Beam Computed Tomography (CBCT) revealed the presence of 12 supernumerary teeth. Furthermore, a clinical examination pointed to the presence of retained deciduous teeth and a delayed eruption of permanent teeth. Orthodontic-surgical procedures were implemented, in accordance with the literature. During the course of the orthodontic treatment, a decrease in auditory sensitivity was observed, for which reason hearing tests were also performed. Conductive hearing loss was detected. As such, it is important to remember that in such cases, auditory check-ups need to be performed between the many surgical and orthodontic interventions, which usually last a few years.</jats:p>

Cleidocranial dysplasia

“…RUNX2 mutations result in defective intramembranous and endochondral ossification [4]. Besides patent anterior fontanelle, late closure of cranial sutures and absent or rudimentary clavicles, patients with CCD frequently present with late erupting secondary dentition, impacted and supernumerary teeth, an inverted pear-shaped calvaria, hypertelorism, general midface retrusion, high palate, mandible prognathism, brachydactyly, pubic bone abnormalities, such as wide pubic symphysis and short stature [1,3,5,[7][8][9]. There is no clear phenotype-genotype correlation and a wide spectrum of clinical features ranging from dental abnormalities to all CCD manifestations exists in the affected children [2,3,5,6,9].…”

“…There is no clear phenotype-genotype correlation and a wide spectrum of clinical features ranging from dental abnormalities to all CCD manifestations exists in the affected children [2,3,5,6,9]. Individuals with CCD spectrum disorder are at increased risk of developing recurrent sinus and ear infections leading to conductive hearing loss [1,3,7]. There is no causal therapy in CCD; management of CCD patients consists predominantly of preventive measures, such as trauma prevention, skull protection and efficient treatment of otitis and sinusitis.…”

“…There is no causal therapy in CCD; management of CCD patients consists predominantly of preventive measures, such as trauma prevention, skull protection and efficient treatment of otitis and sinusitis. Dental care is represented by orthodontic treatment, removal of the supernumerary teeth and, eventually, orthognathic preprosthetic surgery [1,3,[5][6][7]. In case of stunted growth, growth hormone treatment was reported as beneficial [8].…”

“…Cleidocranial dysplasia (CCD) is a rare (prevalence 1:1,000,000) autosomal dominant disorder resulting in skeletal anomalies, such as patent fontanels, late closure of cranial sutures and rudimentary or absent clavicles [1][2][3][4][5][6][7][8][9]. The diagnosis of CCD remains incidental due to the rare occurrence of this disorder.…”

Disostosis cleidocraneal a tres años de tratamiento ortopédico

“…Durante el transcurso del tratamiento ortodóntico se observó disminución en la sensibilidad auditiva, el autor recomienda realizar controles auditivos regulares, para evitar la pérdida de función. 16 Roberts T, en su investigación reunió múltiples reportes de casos y enumeró las posibles opciones de tratamiento de acuerdo con la anomalía dental que presente el paciente, coincidiendo con tratamientos quirúrgicos, expansión con aparatología ortopédica y ortodóncica. 17…”