Correlation of cochlear aperture stenosis with cochlear nerve deficiency in congenital unilateral hearing loss and prognostic relevance for cochlear implantation
Open Access
- 8 February 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Scientific Reports
- Vol. 11 (1), 1-10
- https://doi.org/10.1038/s41598-021-82818-9
Abstract
The use of neonatal hearing screening has enabled the identification of congenital unilateral sensorineural hearing loss (USNHL) immediately after birth, and today there are several intervention options available to minimize potential adverse effects of this disease, including cochlear implantation. This study aims to analyze the characteristics of the inner ear of a homogeneous group of congenital non-syndromic USNHL to highlight the features of the inner ear, which can help in clinical, surgical, and rehabilitative decision-making. A retrospective chart review was carried out at a tertiary referral center. Systematic diagnostic work-up and rigorous inclusion–exclusion criteria were applied to 126 children with unilateral hearing impairment, leading to a selection of 39 strictly congenital and non-syndromic USNHL cases, undergoing computed tomography (CT) and magnetic resonance (MR) imaging studies. The frequency and type of malformations of the inner ear in USNHL and unaffected contralateral ears were assessed, with an in-depth analysis of the deficiency of the cochlear nerve (CND), the internal auditory canal (IAC) and the cochlear aperture (CA). Inner ear anomalies were found in 18 out of 39 (46%) of the USNHL patients. In 1 subject, the anomalies were bilateral, and the CND resulted in the predominant identified defect (78% of our abnormal case series), frequently associated with CA stenosis. Only 3 out of 14 children with CND presented stenosis of the IAC. CND and CA stenosis (and to a much lesser extent IAC stenosis) are a frequent association within congenital and non-syndromic USNHL that could represent a distinct pathological entity affecting otherwise healthy infants. In the context of a diagnostic work-up, the evaluation with CT and MRI measurements should take place in a shared decision-making setting with thorough counseling. Both imaging techniques have proven useful in differentiating the cases that will most likely benefit from the cochlear implant, from those with potentially poor implant performance.This publication has 52 references indexed in Scilit:
- CINGLE-trial: cochlear implantation for siNGLE-sided deafness, a randomised controlled trial and economic evaluationBMC Ear, Nose and Throat Disorders, 2015
- Risk analysis of unilateral severe-to-profound sensorineural hearing loss in childrenInternational Journal of Pediatric Otorhinolaryngology, 2013
- Unilateral Deafness in ChildrenOtology & Neurotology, 2013
- Etiology of unilateral hearing loss in a national hereditary deafness repositoryAmerican Journal of Otolaryngology, 2012
- Longitudinal study of children with unilateral hearing lossThe Laryngoscope, 2012
- Evaluation of Pediatric Sensorineural Hearing Loss With Magnetic Resonance ImagingJAMA Otolaryngology–Head & Neck Surgery, 2008
- Cochleovestibular Anomalies in Children With CholesteatomaThe Laryngoscope, 2008
- Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention ProgramsPEDIATRICS, 2007
- Newborn Hearing Screening — A Silent RevolutionThe New England Journal of Medicine, 2006
- Amplification in the Rehabilitation of Unilateral Deafness: Speech in Noise and Directional Hearing Effects with Bone-Anchored Hearing and Contralateral Routing of Signal AmplificationOtology & Neurotology, 2006