Major Ear Aplasia and Cochleovestibular Dysplasia: Rare Congenital Malformation about a Case  

作　　者：Mouangue-Mbonjo Louise Epée Ngoué Jeannette Mantho Fopa Pauline Njifou Njimah Amadou Mouangue-Mbonjo Louise;Epée Ngoué Jeannette;Mantho Fopa Pauline;Njifou Njimah Amadou(Faculty of Medicine and Pharmaceutical Sciences (FMSP), Department of Surgery and Specialties, University of Douala, Douala, Cameroon;ENT and Maxillofacial Surgery Department, Yaoundé Central Hospital, Yaoundé, Cameroon;Department of Pediatrics, Faculty of Medicine and Biomedical Sciences University of Yaoundé I, Yaoundé, Cameroon)

机构地区：[1]Faculty of Medicine and Pharmaceutical Sciences (FMSP), Department of Surgery and Specialties, University of Douala, Douala, Cameroon [2]ENT and Maxillofacial Surgery Department, Yaoundé Central Hospital, Yaoundé, Cameroon [3]Department of Pediatrics, Faculty of Medicine and Biomedical Sciences University of Yaoundé I, Yaoundé, Cameroon

出　　处：《International Journal of Otolaryngology and Head & Neck Surgery》2023年第6期397-404,共8页耳鼻喉（英文）

摘　　要：Introduction: The congenital malformations of the ear are rare and often isolated, may be unilateral or bilateral, and can be associated with another syndromic malformation. Such malformations so not necessarily impact aesthetics and social relations. Case Presentation: The authors report the case of Samuel M, male born at 38 WA, who is the first child of healthy parents from the same socio-cultural area. His birth weight was 2800 g and he did not have any risk factors for deafness or concept of obstetrical trauma. He presented with congenital malformation manifesting as bilateral ear aplasia with unilateral facial paralysis. Computed tomography revealed abnormalities of the inner ear;functional explorations, such as PEA and OEA, showed findings in favor of bilateral cophosis. ASSR (Auditory Steady-State Responses) was not performed. The announcement of the serious diagnosis of deafness requires multidisciplinary care in order to plan a therapeutic program to limit the impact on the development of language, schooling, and consequently, the socio-professional future of children. Conclusion: This clinical case underlines the fact that interest of the clinical interview before possible multiple surgery does not always guarantee the satisfaction of the desire for repair in this type of patient.Introduction: The congenital malformations of the ear are rare and often isolated, may be unilateral or bilateral, and can be associated with another syndromic malformation. Such malformations so not necessarily impact aesthetics and social relations. Case Presentation: The authors report the case of Samuel M, male born at 38 WA, who is the first child of healthy parents from the same socio-cultural area. His birth weight was 2800 g and he did not have any risk factors for deafness or concept of obstetrical trauma. He presented with congenital malformation manifesting as bilateral ear aplasia with unilateral facial paralysis. Computed tomography revealed abnormalities of the inner ear;functional explorations, such as PEA and OEA, showed findings in favor of bilateral cophosis. ASSR (Auditory Steady-State Responses) was not performed. The announcement of the serious diagnosis of deafness requires multidisciplinary care in order to plan a therapeutic program to limit the impact on the development of language, schooling, and consequently, the socio-professional future of children. Conclusion: This clinical case underlines the fact that interest of the clinical interview before possible multiple surgery does not always guarantee the satisfaction of the desire for repair in this type of patient.

关 键 词：Ear Malformations Congenital Malformations Major Aplasia Congenital Facial Paralysis Congenital Deaf-Mutism 

分 类 号：R76[医药卫生—耳鼻咽喉科]