Cochlear Implantation in Children With CHARGE Syndrome: Therapeutic Decisions and Outcomes

Lanson, Biana G.; Green, Janet E.; Roland, J. Thomas; Lalwani, Anil K.; Waltzman, Susan B.

doi:10.1097/mlg.0b013e31806009c9

Cited by 65 publications

(47 citation statements)

References 14 publications

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“…The underdeveloped vestibulum found by Satar et al [6] and Morimoto et al [7] further underlines the fact that the pars superior is affected. Reports in the literature [8] of SCC aplasia or CHARGE syndrome (58% co-incidence in our collection, 60% in that of Satar et al [6]) normally recommend cochlear implantation for these patients (CHARGE is a syndrome involving coloboma, heart defects, choanal atresia, mental retardation, genitourinary and ear anomalies). Three patients with cochlear nerve aplasia and coexisting SCC aplasia responded to auditory stimuli, despite the non-visibility of the cochlear nerve (17).…”

Section: Discussionmentioning

confidence: 82%

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The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations

Giesemann

Kontorinis²,

Zajaczek³

et al. 2011

Eur Radiol

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Section: Discussionmentioning

confidence: 82%

“…Three patients with cochlear nerve aplasia and coexisting SCC aplasia responded to auditory stimuli, despite the non-visibility of the cochlear nerve (17). Lanson et al [8] reported on 10 implanted patients with CHARGE syndrome, all of whom showed varying but limited degrees of auditory benefit.…”

Section: Discussionmentioning

confidence: 96%

The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations

Giesemann

Kontorinis²,

Zajaczek³

et al. 2011

Eur Radiol

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“…The challenges with CI described in our case study for a subject with KS may be applicable to individuals with CHARGE syndrome who are eligible for CI. In other words, both groups have handicaps in addition to hearing loss, and both groups have intellectual disabilities and anomalies of the petrosal bone [10] . Given the similarity between the groups and the lack of research on CI in KS, studies investigating CI in individuals with CHARGE syndrome could aid in clinical decision making when it comes to implantation in subjects with KS.…”

Section: Discussionmentioning

confidence: 99%

Cochlear Implantation in a Patient with Kabuki Syndrome

Vesseur

Cillessen²,

Mylanus³

2016

Int Adv Otol

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Criteria for cochlear implants are expanding and now include children with disabilities in addition to hearing loss, such as those with Kabuki syndrome (KS). This case report describes language outcomes and changes in the quality of life of a female child with KS after cochlear implantation. The subject had a profound progressive sensorineural hearing loss, cognitive impairments, and other disabilities and communicated using vocalized sounds and the Dutch Sign Language. After cochlear implantation at an age of nine years and three months, the patient displayed no progress in speech production and minimal progress in receptive language development, but she had an increased awareness of the world and an increase in the quality of life.

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“…Therefore, a larger number of criteria will usually influence the decision as to whether these patients should undergo cochlear implantation (CI). Evaluation of children with a CI who also have CHARGE syndrome demonstrates varying but limited degrees of auditory benefit [10]. There is a consensus in the literature that these children benefit; however, realistic expectations have to be discussed with parents beforehand.…”

Section: Hypoplasia and Aplasia Of The Vestibulocochlear Nerve (Vcn)mentioning

confidence: 99%

Some Remarks on Imaging of the Inner Ear: Options and Limitations

Giesemann

Hofmann

2015

Clin Neuroradiol

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The temporal bone has a highly complex anatomical structure, in which the sensory organs of the cochlea and the vestibular system are contained within a small space together with the sound-conducting system of the middle ear. Detailed imaging is thus required in this anatomical area. There are a great many clinical aims for which the highest-possible spatial resolution is required. These include the localization of cerebrospinal fluid fistulas, the detection of malformations of the middle and inner ear and the vestibulocochlear nerve, an aberrant course of the facial nerve and anomalies of the arterial and venous structures, the confirmation of dehiscence of the semicircular canals and finally, the verification of endolymphatic hydrops in cases of Ménière's disease. However, the term 'high resolution' is very time dependent. Two milestones in this respect have been (in 1991) the 3D visualization of the inner ear by means of maximum-intensity projection (MIP) of a T2-weighted constructive interference in steady state (CISS) sequence of a 1.5-tesla magnetic resonance imaging (MRI) scanner (Tanioka et al., Radiology 178:141-144, 1991) and (in 1997) imaging of the vestibulocochlear nerve for the diagnosis of hypoplasia inside the internal auditory canal using the same sequence (Casselman et al., Radiology 202:773-781, 1997).The objective of this article is to highlight the options for, and the challenges of, contemporary imaging with regard to some clinical issues relating to the inner ear.

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Cochlear Implantation in Children With CHARGE Syndrome: Therapeutic Decisions and Outcomes

Cited by 65 publications

References 14 publications

The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations

The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations

Cochlear Implantation in a Patient with Kabuki Syndrome

Some Remarks on Imaging of the Inner Ear: Options and Limitations

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