Bony cochlear nerve canal stenosis and speech discrimination in pediatric unilateral hearing loss

2015 ◽  
Vol 125 (7) ◽  
pp. 1691-1696 ◽  
Author(s):  
Patricia L. Purcell ◽  
Ayaka J. Iwata ◽  
Grace S. Phillips ◽  
Angelisa M. Paladin ◽  
Kathleen C. Y. Sie ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Nerve ◽  
Unilateral Hearing Loss ◽  
Speech Discrimination ◽  
Canal Stenosis

scholarly journals Progression of Unilateral Hearing Loss in Children With and Without Ipsilateral Cochlear Nerve Canal Stenosis

2017 ◽  
Vol 38 (6) ◽  
pp. e138-e144 ◽  
Author(s):  
Patricia L. Purcell ◽  
Justin R. Shinn ◽  
Scott S. Coggeshall ◽  
Grace Phillips ◽  
Angelisa Paladin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Nerve ◽  
Unilateral Hearing Loss ◽  
Canal Stenosis

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

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.

Unilateral Hearing Loss Due to Cochlear Nerve Involvement as Isolated Symptom of a Primary Medulloblastoma

2019 ◽  
Vol 51 (02) ◽  
pp. 170-172
Author(s):  
Janine Magg ◽  
Thomas Nägele ◽  
Michael Alber ◽  
Annette Weichselbaum ◽  
Martin Ebinger ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Pediatric Population ◽  
Wnt Signaling Pathway ◽  
Neurofibromatosis Type ◽  
Cochlear Nerve ◽  
Vestibular Schwannomas ◽  
Clinical Feature ◽  
Unilateral Hearing Loss ◽  
Common Symptom ◽  
The Central Nervous System

AbstractUnilateral sensorineural hearing loss is a common symptom of vestibular schwannomas in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas and is often the initial clinical feature. While rare cases of sensorineural impairment presenting as vision or hearing loss due to metastatic medulloblastoma are known, hearing loss as an isolated presenting symptom of primary malignant neuroepithelial tumors of the central nervous system has not been reported in the pediatric population so far. We present two adolescents with unilateral hearing loss due to cochlear nerve dysfunction as the only symptom of a primary nonmetastatic medulloblastoma of the WNT signaling pathway family members subgroup.

Bony cochlear nerve canal stenosis in pediatric unilateral sensorineural hearing loss

2018 ◽  
Vol 106 ◽  
pp. 72-74 ◽  
Author(s):  
Chae-Hyun Lim ◽  
Ji Hyung Lim ◽  
Doyoun Kim ◽  
Ho sung Choi ◽  
Dong-Hee Lee ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Canal Stenosis

scholarly journals Frequent Association of Cochlear Nerve Canal Stenosis With Pediatric Sensorineural Hearing Loss

2012 ◽  
Vol 138 (4) ◽  
pp. 383 ◽  
Author(s):  
Abigail Wilkins ◽  
Sanjay P. Prabhu ◽  
Lin Huang ◽  
Patricia B. Ogando ◽  
Margaret A. Kenna
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Canal Stenosis

Cochlear nerve canal stenosis and associated semicircular canal abnormalities in paediatric sensorineural hearing loss: a single centre study

2020 ◽  
Vol 134 (7) ◽  
pp. 603-609
Author(s):  
Z Zainol Abidin ◽  
F Mohd Zaki ◽  
T Y Kew ◽  
B S Goh ◽  
A Abdullah
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Semicircular Canal ◽  
Semicircular Canals ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Bony Labyrinth ◽  
Canal Stenosis ◽  
Computed Tomography Images ◽  
Canal Diameter

AbstractObjectiveThis study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis.MethodThis was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated.ResultsCochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent).ConclusionCochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

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