Research progress on incomplete partition type 3 inner ear malformation.
Eur Arch Otorhinolaryngol
; 281(8): 3943-3948, 2024 Aug.
Article
em En
| MEDLINE
| ID: mdl-38498189
ABSTRACT
PURPOSE:
This review aims to provides a comprehensive overview of the latest research progress on IP-III inner ear malformation, focusing on its geneticbasis, imaging features, cochlear implantation, and outcome.METHODS:
Review the literature on clinical and genetic mechanisms associated with IP-III.RESULTS:
Mutations in the POU3F4 gene emerge as the principal pathogenic contributors to IP-III anomalies, primarily manifesting through inner ear potential irregularities leading to deafness. While cochlear implantation stands as the primary intervention for restoring hearing, the unique nature of the inner ear anomaly escalates the complexity of surgical procedures and postoperative results. Hence, meticulous preoperative assessment to ascertain surgical feasibility and postoperative verification of electrode placement are imperative. Additionally, gene therapy holds promise as a prospective treatment modality.CONCLUSIONS:
IP-III denotes X-linked recessive hereditary deafness, with cochlear implantation currently serving as the predominant therapeutic approach. Clinicians are tasked with preoperative assement and individualized postoperative rehabilitation.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Implante Coclear
/
Fatores do Domínio POU
/
Orelha Interna
Limite:
Humans
Idioma:
En
Revista:
Eur Arch Otorhinolaryngol
Assunto da revista:
OTORRINOLARINGOLOGIA
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
China