Vestibular anomalies and dysfunctions in children with inner ear malformations: A narrative review

Abstract

About 20% of children with congenital hearing loss present malformations of the inner ear. In the past few years much has been understood about the morphology and function of the anterior part of the labyrinth, since hearing loss may have a dramatic effect on the overall development of a child. Nowadays, for most of them, a chance for hearing rehabilitation is available, making hearing loss a treatable condition. The anomalies range from the lack of development of the whole inner ear to specific anomalies of isolated structures. Despite the frequent concomitant involvement of the posterior part of the labyrinth, this part of the inner ear is frequently neglected while discussing its morphology and dysfunction. Even though vestibular and balance function/dysfunction may have a significant impact on the global development of children, very little is known about these specific disorders in patients with inner ear malformations. The aim of this review is to summarize the available literature about vestibular anomalies and dysfunctions in children with inner ear malformations, discussing what is currently known about the topic.

The child who lived: Down syndrome among Neanderthals?

“…Alagille syndrome includes partial or total agenesis of the PSC ( 55 , 56 ), and, because CN-46700 has a normal PSC, we can also discard this syndrome. The presence of a normal modiolus in CN-46700 also allows us to discard Pendred syndrome ( 37 , 48 ). Last, the presence of branchio-otorenal syndrome can also be ruled out because both the dimensions of the cochlear nerve canal and the facial nerve pathway are normal ( 32 , 33 ).…”

“…LSC dysplasia is the most common congenital inner ear disorder (34). Although it can be asymptomatic (35), it most commonly is associated with sensorineural or mixed (sensorineural and conductive) hearing loss (36,37) in addition to vestibular symptoms such as loss of balance and severe vertigo (34,(36)(37)(38). A dilated VA is also congenital and can occur in isolation or in association with other disorders of the inner ear or adjacent structures (26,(38)(39)(40)(41).…”

Causas de vértigo periférico en la infancia

“…Pruebas accesorias: a) VEMPs: No se utilizan en el diagnóstico de VPB, pero sí para controlar la progresión de la enfermedad o seguimiento de respuesta al tratamiento [17,54]. b) Diagnóstico etiológico: Pruebas en función del diagnóstico etiológico [17,57,60] (Tablas 4 y 7) Estos criterios, establecidos para el adulto, deben matizarse en el niño. El cribado vestibular del recién nacido no está generalizado (ver capítulo).…”