Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes.

[pendred syndrome]

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 .

Diseases
Validation

Diseases presenting "hearing loss" symptom

22q11.2 deletion syndrome

achondroplasia

adrenomyeloneuropathy

alexander disease

benign recurrent intrahepatic cholestasis

canavan disease

cohen syndrome

congenital toxoplasmosis

dentinogenesis imperfecta

fabry disease

familial mediterranean fever

heparin-induced thrombocytopenia

hirschsprung disease

holt-oram syndrome

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

kabuki syndrome

kallmann syndrome

neonatal adrenoleukodystrophy

pendred syndrome

von hippel-lindau disease

wolf-hirschhorn syndrome

This symptom has already been validated