2019
DOI: 10.1259/bjro.20180050
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Radiological diagnosis of the inner ear malformations in children with sensorineural hearing loss

Abstract: Malformations in either the inner ear, vestibulocochlear nerve (VIIIth) or auditory cortex of the brain can lead to congenital sensorineural hearing loss (SNHL). In most cases the underlying disorders involve the membranous labyrinth at a microscopic level and therefore radiological examinations are entirely normal. In a significant proportion however (up to 20%), there are abnormalities visualized in the inner ear and/or the VIIIth nerve; the type of abnormality is relevant for the surgical planning of a coch… Show more

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Cited by 17 publications
(17 citation statements)
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“…In this malformation, there was a complete labyrinthine aplasia to the point, where cochlear and vestibular elements are not formed. Due to the failure of the development of the otic vesicles to the absence of the formation of inner ear, this anomaly is described as an otocytic deformity and described as the extremely rare and the most severe inner ear abnormality (Quirk et al 2019). This deformity occurs after the formation of the otic vesicles is complete, because the semicircular canal is the first structure to be formed from the otic vesicles.…”
Section: Michel Deformity or Labyrinthine Aplasiamentioning
confidence: 99%
“…In this malformation, there was a complete labyrinthine aplasia to the point, where cochlear and vestibular elements are not formed. Due to the failure of the development of the otic vesicles to the absence of the formation of inner ear, this anomaly is described as an otocytic deformity and described as the extremely rare and the most severe inner ear abnormality (Quirk et al 2019). This deformity occurs after the formation of the otic vesicles is complete, because the semicircular canal is the first structure to be formed from the otic vesicles.…”
Section: Michel Deformity or Labyrinthine Aplasiamentioning
confidence: 99%
“…Otic capsule and inner ear structures are completely formed at birth and remain stable in size over time, except for endolymphatic duct and sac which continues to grow until the age of four years old and the vestibular aqueduct which continues to develop until puberty [5,8]. An arrest at any one of these stages corresponds to a particular IEM (Table 2).…”
Section: Normal Anatomy and Development Of The Inner Earmentioning
confidence: 99%
“…Since the characterisation of different CH's subtypes can be challenging, the use of normative values in identifying and classifying CH, rather than rely on visual inspection alone, has been proposed and seems to be promising [8,25].…”
Section: Ch-iv (Cochlea With Hypoplastic Middle and Apical Turns)mentioning
confidence: 99%
“…5 In cases with severe or profound hearing loss (HL), quite a high prevalence of inner ear malformations (IEMs) of around 15 to 20% has been reported in a study. 6 Though cochlear implantation in cases with IEMs has favorable surgical and speech results 7 and is a safe procedure in experienced hands, 8 however difficulties and intraoperative complications do occur. 9 Complications ranging from pain, minor bleed, infection, breakdown of skin, facial palsy, taste changes, vertigo, cerebrospinal fluid leak, device failure as well as mal-placement, skull base and brain damage to complete deafness and death may occur.…”
Section: Introductionmentioning
confidence: 99%