Pediatric Sensorineural Hearing Loss, Part 2: Syndromic and Acquired Causes

B.Y Huang,M Castillo,C Zdanski

doi:10.3174/ajnr.a2499

Abstract

This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. Previously, we discussed the clinical work-up of children with hearing impairment, the classification of inner ear malformations, and congenital nonsyndromic causes of hearing loss. Here, we review and illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. Syndromes discussed include BOR syndrome, CHARGE syndrome, Pendred syndrome, Waardenburg syndrome, and X-linked hearing loss with stapes gusher. We conclude the article with a review of acquired causes of childhood SNHL, including infections, trauma, and neoplasms.

Highlights

REVIEW ARTICLESUMMARY: This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL
In the first part of our series, we reviewed some of the practical aspects related to imaging children with SNHL, including epidemiology, clinical work-up, and choices of imaging technique; we discussed the classification of congenital inner ear malformations and congenital nonsyndromic causes of SNHL
Conclusions many of the syndromes associated with SNHL do not usually demonstrate gross inner ear anomalies by imaging, there are several in which inner ear malformations are common

Summary

REVIEW ARTICLE

SUMMARY: This article is the second in a 2-part series reviewing neuroimaging in childhood SNHL. We review and illustrate the most common syndromic hereditary and acquired causes of childhood SNHL, with an emphasis on entities that demonstrate inner ear abnormalities on cross-sectional imaging. In the first part of our series, we reviewed some of the practical aspects related to imaging children with SNHL, including epidemiology, clinical work-up, and choices of imaging technique; we discussed the classification of congenital inner ear malformations and congenital nonsyndromic causes of SNHL. There are a number of well-characterized entities in which SNHL is a frequent and/or major component (Table 1) Many of these syndromes do not usually demonstrate gross inner ear anomalies by imaging, there are several in which inner ear malformations are a common and sometimes defining feature.

Inner Ear Malformations Not Common on Imaging

Findings

Conclusions