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Published on: February 21, 2016
Clinical characteristics of children with cochlear nerve dysplasias
Jessica Levi1, Julie Ames, Katie Bacik
1Department of Otolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware 19803, USA.
Insights
Cochlear nerve dysplasia (CND) in children often presents with auditory neuropathy spectrum disorder (ANSD) and comorbidities. Many also have other inner ear anomalies, with unilateral CND more frequent on the left.
Area of Science:
- Pediatric Otolaryngology
- Neurotology
- Medical Imaging
Background:
- Cochlear nerve dysplasia (CND) is a congenital inner ear anomaly.
- Understanding its clinical and audiometric features is crucial for diagnosis and management.
Purpose of the Study:
- To characterize the clinical presentation and audiometric findings in children diagnosed with cochlear nerve dysplasia (CND).
Main Methods:
- Retrospective review of a clinical database of children with inner ear anomalies.
- Magnetic resonance imaging (MRI) was used to diagnose CND, defined as a cochlear nerve 50% smaller than the facial nerve.
- Data collected included patient demographics, CND type (aplasia/hypoplasia), laterality, associated anomalies, hearing loss severity, and audiometric profiles.
Main Results:
- 18 children with CND were identified, with an average age of 4.6 years at diagnosis.
- CND included aplasia (n=12) and hypoplasia (n=6), with 3 bilateral cases.
- Severe-to-profound hearing loss was common (14/18), and 72% (13/18) exhibited auditory neuropathy spectrum disorder (ANSD).
- 50% had other inner ear anomalies, and 56% had comorbidities. Unilateral CND was more common on the left.
Conclusions:
- CND frequently co-occurs with auditory neuropathy spectrum disorder (ANSD) in children.
- A significant proportion of children with CND present with comorbid conditions and other inner ear anomalies.
- Left-sided unilateral CND appears to be more prevalent.
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.
