Pediatric Sensorineural Hearing Loss, Part 1: Practical Aspects for Neuroradiologists

Abstract

SUMMARY: SNHL is a major cause of childhood disability worldwide, affecting 6 in 1000 children. For children with prelingual hearing loss, early diagnosis and treatment is critical to optimizing speech and language development, academic achievement, and social and emotional development. Cross-sectional imaging has come to play an important role in the evaluation of children with SNHL because otolaryngologists routinely order either CT or MR imaging to assess the anatomy of the inner ears, to identify causes of hearing loss, and to provide prognostic information related to potential treatments. In this article, which is the first in a 2-part series, we describe the basic clinical approach to imaging of children with SNHL, including the utility of CT and MR imaging of the temporal bones; we review the most recent proposed classification of inner ear malformations; and we discuss nonsyndromic congenital causes of childhood SNHL.ABBREVIATIONS: ABR ϭ auditory brain stem response; BCNC ϭ bony cochlear nerve canal; CISS ϭ constructive interference in steady state; CMV ϭ cytomegalovirus; CN ϭ cochlear nerve; CND ϭ cochlear nerve deficiency; FN ϭ facial nerve; FSE ϭ fast spin-echo; IAC ϭ internal auditory canal; IP-I ϭ incomplete partition type I; IP-II ϭ incomplete partition type II; SCC ϭ semicircular canal; SNHL ϭ sensorineural hearing loss; VA ϭ vertebral artery

Mri and High Resolution Ct in Congenital Hearing Loss

“…In our study, (18) patients diagnosed as having middle ear anomalies, their age ranged from 1 year to 19 years and the main complaints was hearing loss; (6) patients with abnormal tympanic cavity (4 males and 2 females), (13) patients with anomalies of the ossicular chains (9 males and 4 females) and (3) patients with congenital cholestatoma (2 males and one female). According to HRCT diagnosis, as seen in Table (2) and Chart (2), the tympanic cavity was within normal limits in (10) patients (7 males and 3 females), hypo-plasia of the tympanic cavity in (6) patients (4 males and 2 females) and one case with oval window bony atresia.…”

“…al, 2012 (13) SNHL presenting later in life is often related to inner ear malformations any of which may be suggested by findings at cross-sectional imaging. Neuroradiologists who routinely interpret temporal bone studies performed for the evaluation of childhood SNHL should be familiar with these potential causes and their respective imaging appearances; furthermore, they should be familiar with findings such as severe labyrinthine ossification, which might preclude or complicate cochlear implantation in a child with hearing loss.…”

Etiología de la hipoacusia infantil.

“…En cualquier fase de la embriogénesis pueden producirse anomalías de oído medio e interno, manifestándose con hipoacusia progresiva, fluctuante, de tipo conductivo, neurosensorial o mixta. En un 40% de pacientes se demuestra alguna alteración en la tomografía computarizada (TC) o en la resonancia magnética (RM) [16]. En resumen, las dificultades diagnósticas de la hipoacusia neonatal provienen de la gran variedad de causas existentes, muchas de las cuales no tienen una prueba definitiva para su diagnóstico.…”

Moderate-Severe Hearing Loss in Children: A Diagnostic and Rehabilitative Challenge

“…Also, the genetician may provide information to parents among inheritance modalities and the pre-natal diagnosis if available [81,82] . Neuroradiologic examination is another necessary diagnostic stage: temporal bone CT (computed tomography) scans and brain, brainstem, pontocerebellar angle, inner ear and central auditory system MRI (magnetic resonance imaging) are recommended in all permanent hearing loss in children and, frequently, they are complementary examinations [83] . CT scans are essential in case of cranial trauma, but also in case of pre-surgical evaluation before cochlear implant surgery, in case of cholesteatoma, or in case of suspected middle ear malformation [84][85][86] .…”

“…CT scans are essential in case of cranial trauma, but also in case of pre-surgical evaluation before cochlear implant surgery, in case of cholesteatoma, or in case of suspected middle ear malformation [84][85][86] . MRI has a high degree of definition of labyrinth fluid contents; it is suitable in the inner ear and central auditory system evaluation [83] .…”