Unilateral Total Deafness in Children Clinical Problems with a Special View to Vestibular Function

A case of unilateral sensorineural hearing loss caused by a venous malformation of the internal auditory canal

“…The prevalence of a unilateral sensorineural hearing loss (SNHL) in children has been estimated to be around 0.1-0.2% [1][2][3]. It has been attributed to postnatal viral infections, sudden deafness, prenatal and perinatal problems including maternal rubella and viral infections, congenital inner ear anomalies, and other factors.…”

Normal and Pathological Maturation of Vestibular Function in the Human Child11This research was supported in part by National Eye Institute Grant No. EY-02612.

“…For this very large series, 59% of the children had "normal" vestibular responses, whereas 19% had absent responses and 22% had abnormal responses. Considering the results of Arnvig (1955), Everberg (1960), and Rosenblüt et al (1960), this would seem to be an underestimate. Everberg (1960) studied 122 adults and children with unilateral total deafness by rotation and calories, recording nystagmus by visual observation through Bartel's glasses.…”

Temporal Bone High-Resolution Computed Tomography in Non-Syndromic Unilateral Hearing Loss in Children

“…Elementary school-age children with UHL have recently been shown to perform significantly worse in oral language tests than do their normalhearing siblings [17] . The potential diagnostic role of imaging techniques in disclosing middle and inner ear malformations as the underlying cause of UHL was hinted at by Everberg [6] in the 1960s. Alongside clinical and genetic data, the author described radiological abnormalities in 18% of school-age children with unilateral deafness using multiple X-ray projections of their temporal bones.…”

“…However, considering an age range of 6-19 years, as reported by North American authors, the prevalence reaches 12.3/1,000 [5] . Several studies conducted in Scandinavian countries estimate that unilateral total deafness only accounts for a prevalence of 0.6-1/1,000 school-age children [6][7][8] . According to many authors, this condition is more prevalent among males [6,[9][10][11] .…”

“…Among acquired causes may be listed premature birth, low birth weight, ototoxic drug administration, postnatal systemic infections (i.e. cytomegalovirus infection, mumps) and labyrinthitis ossificans due to meningitis or to cranial trauma [3,4,6] . UHL, possibly due to intracellular accumulation of glycosphingolipids, especially in the vascular system, is reported in a young girl in a recent study of 20 patients affected by Fabry disease [12] .…”