Clinical characteristics of children with cochlear nerve dysplasias.

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.

Atypical presentation of geniculate ganglion venous malformation in a child: conductive hearing loss without facial weakness.

We describe an unusual presentation of geniculate ganglion venous malformation, a rare facial nerve lesion, emphasizing the importance of the differential diagnosis, imaging characteristics, and controversies in management. A child presented with moderate right-sided conductive hearing loss and a House-Brackmann grade I facial nerve function bilaterally. Computed tomography and magnetic resonance imaging showed a mass demonstrating features consistent with a geniculate ganglion venous malformation.