Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes.

Wenqi Liang, Line Wang, Xinyu Song, Fenqi Gao, Pan Liu, Tiffany H Lee, Kevin A Peng

Ear, nose, & throat journal 2021 Jun

filter terms:

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.

Citation

Wenqi Liang, Line Wang, Xinyu Song, Fenqi Gao, Pan Liu, Tiffany H Lee, Kevin A Peng. Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes. Ear, nose, & throat journal. 2021 Jun;100(3_suppl):343S-346S