The bony cochlear nerve canal in children with absent or hypoplastic cochlear nerves

Pagarkar, Waheeda; Gunny, Roxana; Saunders, Dawn; Yu, Wai‐Yung; Rajput, Kaukab

doi:10.1016/j.ijporl.2011.02.017

Cited by 32 publications

(35 citation statements)

References 25 publications

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“…An abnormal CVN has been reported in patients in whom the cochlear aperture size is normal or in patients with normal size IAC, or in patients with no associated cochleovestibular malformation [Glastonbury et al, 2002;Adunka et al, 2006Adunka et al, , 2007Buchman et al, 2006;Bradley et al, 2008;McClay et al, 2008;Carner et al, 2009;Warren et al, 2010;Kutz et al, 2011;Pagarkar et al, 2011;Colletti et al, 2014]. Our data highlight the association of normal cochleae, normal cochlear apertures, and normal caliber IAC with severe CVN abnormalities.…”

Section: Imaging Recommendationssupporting

confidence: 67%

“…Clearly, some nerve fibers were available to transmit auditory signals to the brainstem [Casselman et al, 1997;Ozdoğmuş et al, 2004;Warren et al, 2010]. These encouraging results have led to inclusion of ABR testing to augment the imaging data [Kim et al, 2006;Oker et al, 2009;Buchman et al, 2011;Kutz et al, 2011;Pagarkar et al, 2011;Roland et al, 2012;Zhang et al, 2012;Vincenti et al, 2014;Wu et al, 2014]. At times, ABR testing may indicate the presence of a viable nerve in response to electrical stimulation; however, the absence of a response inconsistently predicts the aided auditory benefit [Nikolopoulos et al, 2000;Kim et al, 2008;Roland et al, 2012].…”

Section: Current Imaging Data Does Not Predict Auditorymentioning

confidence: 99%

“…Some children, thought to have absent CVN, nonetheless seemed to benefit from a CI and/or exhibit measurable unaided hearing thresholds [Casselman et al, 1997;Ito et al, 1999;Acker et al, 2001;Govaerts et al, 2003;Zanetti et al, 2006;Bradley et al, 2008;Kang et al, 2010;Warren et al, 2010;Buchman et al, 2011;Kutz et al, 2011;Pagarkar et al, 2011;Zhang et al, 2012;Vincenti et al, 2014;Wu et al, 2014]. The Birman classifications have the benefit of directly cataloging what is seen on imaging and providing an association with outcome, lending it diagnostic usefulness.…”

Section: Introductionmentioning

confidence: 99%

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Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids

Kari

Loggins

et al. 2018

Audiol Neurotol

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Objective: Imaging characteristics and hearing outcomes in children with cochleovestibular or cochleovestibular nerve (CVN) abnormalities. Study Design: Retrospective, critical review. Setting: Tertiary referral academic center. Patients: Twenty-seven children with CVN abnormalities with magnetic resonance (MRI) and/or computed tomography (CT). Study Intervention(s): None. Main Outcome Measure(s): Determine the likely presence or absence of a CNV and auditory stimulation responses. Results: Two of 27 cases had unilateral hearing loss, and all others had bilateral loss. Eleven (46%) were identified with a disability or additional condition. Twenty-two (42%) ears received a cochlear implant (CI) and 9 ears (17%) experienced no apparent benefit from the device. MRI acquisition protocols were suboptimal for identification of the nerve in 22 (42%) ears. A likely CVN absence was associated with a narrow cochlear aperture and internal auditory canal and cochlear malformation. Thirteen (48%) children with an abnormal nerve exhibited normal cochleae on the same side. Hearing data were available for 30 ears, and 25 ears (83%) exhibited hearing with or without an assistive device. One child achieved closed set speech recognition with a hearing aid, another with a CI. One child achieved open set speech recognition with a CI. Conclusions: Current imaging cannot accurately characterize the functional status of the CVN or predict an assistive device benefit. Children who would have otherwise been denied a CI exhibited auditory responses after implantation. A CI should be considered in children with abnormal CVN. Furthermore, imaging acquisition protocols need standardization for clear temporal bone imaging.

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Section: Imaging Recommendationssupporting

confidence: 67%

Section: Current Imaging Data Does Not Predict Auditorymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids

Kari

Loggins

et al. 2018

Audiol Neurotol

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“…Both MRI and HRCT are indispensable tools in the preexamination assessing the patient's suitability for CI surgery, especially to exclude children with hypoplasia/aplasia of the hearing nerve, as an intact cochlear nerve is required for successful hearing rehabilitation after CI surgery. A small inner ear canal may be indicative of CND in HRCT, but might also be of average width [Pagarkar et al, 2011]. In order to be able to differentiate the beneficial information obtained from MRI and HRCT, Pagarkar et al [2011] suggested a combination of the diagnostic imaging tools with audiological tests for the evaluation of the integrity of the hearing nerve.…”

Section: Discussionmentioning

confidence: 99%

“…A small inner ear canal may be indicative of CND in HRCT, but might also be of average width [Pagarkar et al, 2011]. In order to be able to differentiate the beneficial information obtained from MRI and HRCT, Pagarkar et al [2011] suggested a combination of the diagnostic imaging tools with audiological tests for the evaluation of the integrity of the hearing nerve. Bilaterally deaf children with uni-or bilateral cochlear nerve aplasia/hypoplasia showed overall poorer results in comparison to their peers having other malformations, such as an enlarged vestibular aqueduct (EVA) [Buchman et al, 2011;Valero et al, 2012].…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

et al. 2015

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For adult patients with single-sided deafness (SSD), treatment with a cochlear implant (CI) is well established as an acceptable and beneficial hearing rehabilitation method administered routinely in clinical practice. In contrast, for children with SSD, CI has been applied less often to date, with the rationale to decide either on a case-by-case basis or under the realm of clinical research. The aim of our clinical study was to evaluate the longitudinal benefits of CI for a group of children diagnosed with SSD and to compare their outcomes with respect to patient characteristics. Evaluating a pool of paediatric SSD patients presenting for possible CI surgery revealed that the primary aetiology of deafness was congenital cochlear nerve deficiency. A subgroup of children meeting the CI candidacy criteria for the affected ear (the majority with acquired hearing loss) were enrolled in the study. Preliminary group results suggest substantial improvements in speech comprehension in noise and in the ability to localise sound, which was demonstrated through objective and subjective assessments after CI treatment for the group, with results varying from patient to patient. Our study shows a trend towards superior outcomes for children with acquired hearing loss and a shorter duration of hearing loss compared to congenitally deafened children who had a longer duration of SSD. This indicates an interactive influence of the age at onset, aetiology and duration of deafness upon the restoration of binaural integration and the overall benefits of sound stimulation to two ears after CI treatment. Continued longitudinal investigation of these children and further studies in larger groups may provide more guidance on the optimal timing of treatment for paediatric patients with acquired and congenital SSD.

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

Wilkins¹,

Prabhu²,

Huang³

et al. 2012

Arch Otolaryngol Head Neck Surg

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The bony cochlear nerve canal in children with absent or hypoplastic cochlear nerves

Cited by 32 publications

References 25 publications

Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids

Abnormal Cochleovestibular Anatomy and Hearing Outcomes: Pediatric Patients with a Questionable Cochleovestibular Nerve Status May Benefit from Cochlear Implantation and/or Hearing Aids

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

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

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