Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

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

doi:10.1177/0145561321996839

Ear Nose Throat J

2021

DOI: 10.1177/0145561321996839

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Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

Wenqi Liang

Line Wang

Xinyu Song

et al.

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 MYH… Show more

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A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency

Maturi,

Noij,

Babu

et al. 2024

Otol Neurotol

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Objective To assess hearing and speech outcomes in pediatric patients with bilateral cochlear nerve deficiency (CND) who underwent cochlear implantation (CI) and to identify factors associated with improved outcomes. Databases reviewed PubMed, Embase, Web of Science, and Cochran databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Methods Studies that reported hearing and speech outcomes of pediatric patients with bilateral CND who underwent CI were included. Demographics, comorbidities, inner ear abnormalities, CND classification (aplasia or hypoplasia), details of diagnostic workup, and outcomes data were extracted. Outcomes were assessed using the four-level auditory performance level (APL) scale. Meta-analysis, using Cochran–Armitage tests, was performed on patients with individual data to assess factors associated with performance. Results A total of 314 papers were screened, and 40 papers with 378 total patients met inclusion criteria. A total of 339 patients had patient-level data and were included in the meta-analysis. Of the 339 patients, 19% (n = 63) of patients had no measurable stimulation, 28% (n = 95) had improved detection, 22% (n = 76) achieved closed-set speech perception, and 31% (n = 105) achieved open-set speech perception. Patients with cochlear nerve aplasia (p = 0.016) and syndromes (p < 0.001) had significantly worse APL scores relative to patients with cochlear nerve hypoplasia and patients without syndromes, respectively. Conclusions While most patients with bilateral CND benefit from CI and almost one-third of patients achieved open-set speech perception, outcomes were heterogenous and one-fifth of patients did not experience measurable benefit from CI.

show abstract

A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency

Maturi,

Noij,

Babu

et al. 2024

Otol Neurotol

View full text Add to dashboard Cite

show abstract

Heterozygous MYH9 Mutations in 2 Children With Cochlear Nerve Canal Stenosis

et al. 2022

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MYH9 is a gene that encodes for a subunit of the myosin heavy chain IIA protein. Mutations in MYH9 are associated with hematologic abnormalities, renal dysfunction, and hearing loss. Bony cochlear nerve canal stenosis (CNCS), which is diagnosed on computed tomography (CT) imaging, has been associated with congenital deafness, cochlear nerve aplasia/hypoplasia, and inner ear malformations. We report two cases of CNCS presenting with profound congenital hearing loss whom we diagnosed with mutations in MYH9 and discuss the genotype-phenotype association and implications for management.

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Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

Cited by 2 publications

References 20 publications

A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency

A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency

Heterozygous MYH9 Mutations in 2 Children With Cochlear Nerve Canal Stenosis

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