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Clinical characteristics of children with cochlear nerve dysplasias.
MedLine Citation:
PMID:  23086614     Owner:  NLM     Status:  Publisher    
OBJECTIVES/HYPOTHESIS: To describe the clinical and audiometric characteristics of children with cochlear nerve dysplasia (CND). STUDY DESIGN: Retrospective chart review of clinical database of children with inner ear anomalies treated at a tertiary care children's hospital. METHODS: Institutional review board-approved retrospective review from June 30, 2006, to July 1, 2011; 18 children were identified with magnetic resonance imaging (MRI) evidence of CND defined as a cochlear nerve 50% smaller than the adjacent facial nerve. RESULTS: Of the 18 patients, nine were girls and nine were boys. Average age at time of MRI diagnosis of CND was 4.6 years. Twelve children had cochlear nerve aplasia, and six had hypoplasia. Three were affected bilaterally: two with aplasia and one with hypoplasia. Unilateral dysplasia was found in 15 children; of these, 60% occurred on the left side. Other inner ear anomalies were found in 50%, including all patients with bilateral CND. Severe-to-profound hearing loss was found in the involved ear(s) in 14 of 18 patients, including all bilateral patients. Of the 18 patients tested, 13 (72%) had an audiometric profile of auditory neuropathy/dys-synchrony syndrome (auditory neuropathy spectrum disorder [ANSD]). Comorbid conditions were present in 56% of patients. Two patients were syndromic. Family history of hearing loss was present in 11% of patients. CONCLUSIONS: Many patients with CND have ANSD, and more than half have comorbidities. Approximately half of affected patients have other inner ear anomalies in the involved ears. Unilateral CND may be more common on the left side. Laryngoscope, 2012.
Jessica Levi; Julie Ames; Katie Bacik; Colin Drake; Thierry Morlet; Robert C O'Reilly
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-19
Journal Detail:
Title:  The Laryngoscope     Volume:  -     ISSN:  1531-4995     ISO Abbreviation:  Laryngoscope     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8607378     Medline TA:  Laryngoscope     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.
Department of Otolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, U.S.A.
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