Cochlear implantation in children with cochlear nerve absence or deficiency.
ABSTRACT To evaluate speech perception after cochlear implantation in children with cochlear nerve absence or deficiency.
A retrospective case review was performed to identify children who underwent cochlear implantation with cochlear nerve absence or deficiency. The cochlear nerve was evaluated by high-resolution three-dimensional T2-weighted fast spin echo MR in the oblique sagittal and axial planes. A deficient cochlear nerve was defined as a cochlear nerve that is smaller in diameter when compared with the adjacent facial nerve in the midportion of the internal auditory canal. The cochlear nerve was considered absent if there was no imaging evidence of a cochlear nerve. Speech awareness threshold and the speech perception category score were used to measure speech perception after cochlear implantation.
Seven children who underwent cochlear implantation in an ear without imaging evidence of a cochlear nerve were identified. One child developed early closed-set speech recognition. The other 6 children developed only speech detection or pattern perception. Two children underwent cochlear implantation with a deficient cochlear nerve. One developed consistent closed-set word recognition and the other developed early closed-set word recognition. The mean follow-up time for all patients was 3.8 years (range, 1.1-7.1 yr).
Cochlear nerve deficiency is not an uncommon cause for profound sensorineural hearing loss and presents a challenge in the decision-making process regarding whether to proceed with a cochlear implant. Children with a deficient but visible cochlear nerve on magnetic resonance image can expect to show some speech understanding after cochlear implantation; however, these children do not develop speech understanding to the level of implanted children with normal cochlear nerves. Children with an absent cochlear nerve determined by magnetic resonance imaging can be expected to have limited postimplantation sound and speech awareness.
Full-textDOI: · Available from: Timothy N Booth, Mar 13, 2014
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ABSTRACT: In current terminology, auditory neuropathy spectrum disorder (ANSD) is a disease involving the disruption of the temporal coding of acoustic signals in auditory nerve fibres, resulting in the impairment of auditory perceptions that rely on temporal cues. There is debate about almost every aspect of the disorder, including aetiology, lesion sites, and the terminology used to describe it. ANSD is a heterogeneous disease despite similar audiological findings. The absence of an auditory brainstem response (ABR) and the presence of otoacoustic emissions (OAE) suggest an ANSD profile. However, to determine the exact anatomical site of the disorder, more in-depth audiological and electrophysiological tests must be combined with imaging, genetics and neurological examinations. Greater diagnostic specificity is therefore needed to provide these patients with more adequate treatment.B-ENT 01/2013; Suppl 21:65-79. · 0.08 Impact Factor
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ABSTRACT: The incidence of sensorineural hearing loss (SNHL) increased gradually in the past decades. High-resolution computed tomography (HRCT) and magnetic resonance (MR) imaging, as an important part of preimplantation evaluation for children with SNHL, could provide the detailed information about the inner ear, the vestibulocochlear nerve, and the brain, so as to select suitable candidate for cochlear implantation (CI). Brain abnormalities were not rare in the brain MR imaging of SNHL children; however, its influence on the effect of CI has not been clarified. After retrospectively analyzing the CT and MR imaging of 157 children with SNHL that accepted preoperative evaluation from June 2011 to February 2013 in our hospital and following them during a period of 14.09 ± 5.08 months, we found that the white matter change, which might be associated with the history of medical condition, was the most common brain abnormality. Usually CI was still beneficial to the children with brain abnormalities, and the short-term hearing improvement could be achieved. Further study with more patients and longer follow-up time was needed to confirm our results.International Journal of Biomedical Imaging 01/2015; 2015:275786. DOI:10.1155/2015/275786
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ABSTRACT: 1) To review the radiologic and audiologic data of 656 children with sensorineural hearing impairment who underwent 3-dimensional magnetic resonance imaging (MRI) in our cochlear implant (CI) center to understand the incidence of cochlear nerve deficiency (CND); and 2) to compare postimplant auditory and speech intelligibility outcomes in implantees with cochlear nerve (CN) aplasia, CN hypoplasia, and normal CN.Ontology & Neurotology 09/2014; DOI:10.1097/MAO.0000000000000568 · 1.60 Impact Factor