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

Wilkins, Abigail; Prabhu, Sanjay P.; Huang, Lin; Ogando, Patricia Barcelos; Kenna, Margaret A.

doi:10.1001/archoto.2012.237

Cited by 37 publications

(29 citation statements)

References 20 publications

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“…From an earlier study, the external dimensions of the cochlea (height and diameter) were found to be similar to the normal cochlea ( 17 ), therefore, it is appropriate to include IP-III under the incomplete partition anomalies. In addition, labyrinthine segment of the facial nerve is located almost above the cochlea ( 20 ) instead of making a gentle curve around the basal turn on axial sections. The labyrinthine segment of the facial nerve is the most superior structure in the temporal bone.…”

Section: I-inner Ear Malformationsmentioning

confidence: 99%

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Classification and Current Management of Inner Ear Malformations

2017

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Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

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Section: I-inner Ear Malformationsmentioning

confidence: 99%

“…The CA is considered hypoplastic ( Figure 9a ) if the width is less than 1.4 mm ( 20 ). The CA is considered to be aplastic when the canal is completely replaced by bone or there is no canal on mid-modiolar view ( Figure 9b ).…”

Section: I-inner Ear Malformationsmentioning

confidence: 99%

Classification and Current Management of Inner Ear Malformations

2017

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“…The BCNC encases the cochlear nerve from the fundus of the internal auditory canal (IAC) to the base of the cochlear modiolus; therefore, it has been suggested that BCNC stenosis serves as a marker of anatomic or functional deficiency of the cochlear nerve . Several studies have described an association between stenosis of the bony cochlear nerve canal and congenital SNHL …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2015

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“…In particular, an absent cochlear nerve canal impacts cochlear implant candidacy and has related prognostic implications. 78,79 In addition, once cochlear implantation has been completed, MRI can no longer provide meaningful diagnostics for that ear due to the implant-associated image distortion. Also, preparation for MRI after implantation requires addressing the internal magnet, which involves an additional procedure.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Yield of MRI for Pediatric Hearing Loss: A Systematic Review

Kachniarz

Chen

Gilani

et al. 2014

Otolaryngol.--head neck surg.

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Objective To perform a systematic review to analyze the diagnostic yield of magnetic resonance imaging (MRI) for pediatric hearing loss, including comparison to computed tomography (CT) and subgroup evaluation according to impairment severity and specific diagnostic findings (cochlear anomalies, enlarged vestibular aqueduct, cochlear nerve abnormalities, and brain findings). Data Sources Pubmed, EMBASE, and the Cochrane library were assessed from their inception through December 2013. Manual searches were also performed, and topic experts were contacted. Review Methods Data from studies describing the use of MRI with or without comparison to CT in the diagnostic evaluation of pediatric patients with hearing loss were evaluated, according to a priori inclusion/exclusion criteria. Two independent evaluators corroborated the extracted data. Heterogeneity was evaluated according to the I2 statistic. Results There were 29 studies that evaluated 2434 patients with MRIs and 1451 patients with CTs that met inclusion/exclusion criteria. There was a wide range of diagnostic yield from MRI. Heterogeneity among studies was substantial but improved with subgroup analysis. Meta-analysis of yield differences demonstrated that CT had a greater yield than MRI for enlarged vestibular aqueduct (yield difference 16.7% [95% CI, 9.1%–24.4%]) and a borderline advantage for cochlear anomalies (4.7% [95% CI, 0.1%–9.5%]). Studies were more likely to report brain findings with MRI. Conclusions These data may be utilized in concert with that from studies of risks of MRI and risk/yield of CT to inform the choice of diagnostic testing.

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

Abstract: Cochlear nerve canal stenosis is associated with SNHL, and the degree of stenosis predicted the degree of SNHL. In addition, the presence of CNC stenosis with additional inner ear abnormalities may affect the severity of SNHL.

Cited by 37 publications

References 20 publications

Classification and Current Management of Inner Ear Malformations

Classification and Current Management of Inner Ear Malformations

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

Diagnostic Yield of MRI for Pediatric Hearing Loss: A Systematic Review

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