Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes.
Ear Nose Throat J
; 100(3_suppl): 343S-346S, 2021 Jun.
Article
em En
| MEDLINE
| ID: mdl-33683976
ABSTRACT
The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças do Nervo Vestibulococlear
/
Nervo Coclear
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Cadeias Pesadas de Miosina
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Miosina Tipo II
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Perda Auditiva Neurossensorial
Tipo de estudo:
Risk_factors_studies
Limite:
Female
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Humans
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Infant
Idioma:
En
Revista:
Ear Nose Throat J
Ano de publicação:
2021
Tipo de documento:
Article