Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.
نویسندگان
چکیده
OBJECTIVE The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome. METHODS We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013. RESULTS This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction. CONCLUSIONS Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice.
منابع مشابه
Results of the implantation of bone-anchored hearing aids in patients with treacher-collins syndrome
INTRODUCTION Treacher-Collins syndrome is characterized by craniofacial malformations, narrowing of the external auditory canal (EAC), and, in 30% of cases, agenesis of the canal and ossicular chain defects. The use of hearing aids (HA) is not possible in cases in which agenesis or stenosis of the EAC accompanies conductive deafness. In contrast, bone conduction implants such as the Bone Ancho...
متن کاملThe Prevalence of Ear Disease in Sensorineural Hearing Impaired Children Below 18 Years-Old in Deaf Welfare Clinic of Molavi Rehabilitation Center
Objectives: Hearing impairment in children is considered as an important public health problem. Auditory function in these children is poor and depends on their hearing aids efficacy and also their ears’ status. Hearing aids are very expensive and ear disease deteriorates its performance. Therefore detecting the ear disease and offering proper treatment is necessary. The aim of this study is to...
متن کاملMild Form of Treacher Collins Syndrome Imitating Juvenile Otosclerosis
Treacher Collins syndrome (TCS) is an inherited developmental disorder. More than 40% of individuals with TCS have conductive hearing loss attributed to external and middle ear anomalies. Mild cases of TCS often pass undiagnosed at birth or early childhood. The disease may be manifested as conductive hearing loss in teenagers and may resemble juvenile otosclerosis. Patients could suffer from sl...
متن کاملEar surgery in Treacher Collins syndrome.
The autosomal dominant hereditary Treacher Collins syndrome manifests itself phenotypically in dysmorphogenesis of particularly the first, but also the second branchial arch system. Consequently, 50% of patients with Treacher Collins syndrome have a congenital, generally pure conductive hearing loss resulting from a major or minor ear anomaly. The outcome of surgery to improve patients' hearing...
متن کاملFiberoptic tracheal intubation through a laryngeal mask airway in a child with Treacher Collins syndrome.
Treacher Collins syndrome (TCS) is a rare inherited condition characterized by bilateral and symmetric abnormalities of structures within the first and second bronchial arches. The mechanism of inheritance is autosomal dominant with variable expressivity. Because of this variability in expression, some affected individuals exhibit virtually no overt clinical manifestations. However, most childr...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Acta otorrinolaringologica espanola
دوره 67 3 شماره
صفحات -
تاریخ انتشار 2016