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"Minimized rotational vestibular testing" as a screening procedure detecting vestibular areflexy in deaf children: screening cochlear implant candidates for Usher syndrome type I.
Eur Arch Otorhinolaryngol. 2008 Jul; 265(7):759-63.EA

Abstract

Morbus Usher (USH), a combination of sensorineural hearing loss and retinal visual impairment, is classified into group I-III. USH I patients are born deaf. Within the first 10 years of life, they develop a severe vision impairment due to progressive retinal dystrophy (retinitis pigmentosa). USH I patients show vestibular deficits. The incidence of USH I among congenitally deaf children is assumed to be as high as 10%. We intend to create a simple examination procedure for screening congenitally deaf children for vestibular deficiency and subsequently USH I. The examination procedure is named "Minimized Rotation". The vestibular function of deaf children was examined by Minimized Rotation during their preoperative cochlear implant candidacy examination. A lack of postrotational nystagmus was seen as an indication for vestibular deficit. Subsequently some of these patients were examined under general anaesthesia by electroretinography (ERG) at the Department of Ophthalmology. A total of 117 children were examined by Minimized Rotation. In 19 children (16.2%) no rotational nystagmus was found. Six of these children were additionally examined at the Department of Ophthalmology using Ganzfeld ERG. Three of them (50%) showed generalized dysfunction of the retina; 8.1% of the children undergoing preoperative evaluation for cochlear implatation are assumed to show abnormalities of the retina. Rotational examination seems to be an appropriate screening method to detect vestibular deficits, which is one sign of USH I. The results always have to be verified by Ganzfeld-ERG or further genetic investigations. Children with USH I are threatened by progressive reduction of vision. We, therefore, consider USH I children always to be implanted bilaterally with a cochlear implant to maximize the benefit of auditory rehabilitation.

Authors+Show Affiliations

Teschner M
Department of Otolaryngology, Medical University of Hannover, 30625, Hannover, Germany. teschner.magnus@mh-hannover.de
Neuburger J
No affiliation info available
Gockeln R
No affiliation info available
Lenarz T
No affiliation info available
Lesinski-Schiedat A
No affiliation info available

MeSH

ChildChild, PreschoolCochlear ImplantationDeafnessHumansInfantMass ScreeningPatient SelectionPreoperative CarePrevalenceReflex, AbnormalReflex, Vestibulo-OcularRetinaRotationSeverity of Illness IndexUsher SyndromesVestibular Function Tests

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18058117

Citation

Teschner, Magnus, et al. ""Minimized Rotational Vestibular Testing" as a Screening Procedure Detecting Vestibular Areflexy in Deaf Children: Screening Cochlear Implant Candidates for Usher Syndrome Type I." European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated With the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery, vol. 265, no. 7, 2008, pp. 759-63.
Teschner M, Neuburger J, Gockeln R, et al. "Minimized rotational vestibular testing" as a screening procedure detecting vestibular areflexy in deaf children: screening cochlear implant candidates for Usher syndrome type I. Eur Arch Otorhinolaryngol. 2008;265(7):759-63.
Teschner, M., Neuburger, J., Gockeln, R., Lenarz, T., & Lesinski-Schiedat, A. (2008). "Minimized rotational vestibular testing" as a screening procedure detecting vestibular areflexy in deaf children: screening cochlear implant candidates for Usher syndrome type I. European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated With the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery, 265(7), 759-63.
Teschner M, et al. "Minimized Rotational Vestibular Testing" as a Screening Procedure Detecting Vestibular Areflexy in Deaf Children: Screening Cochlear Implant Candidates for Usher Syndrome Type I. Eur Arch Otorhinolaryngol. 2008;265(7):759-63. PubMed PMID: 18058117.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - "Minimized rotational vestibular testing" as a screening procedure detecting vestibular areflexy in deaf children: screening cochlear implant candidates for Usher syndrome type I. AU - Teschner,Magnus, AU - Neuburger,Juergen, AU - Gockeln,Roland, AU - Lenarz,Thomas, AU - Lesinski-Schiedat,Anke, Y1 - 2007/12/06/ PY - 2007/08/20/received PY - 2007/11/14/accepted PY - 2007/12/7/pubmed PY - 2008/12/31/medline PY - 2007/12/7/entrez SP - 759 EP - 63 JF - European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery JO - Eur Arch Otorhinolaryngol VL - 265 IS - 7 N2 - Morbus Usher (USH), a combination of sensorineural hearing loss and retinal visual impairment, is classified into group I-III. USH I patients are born deaf. Within the first 10 years of life, they develop a severe vision impairment due to progressive retinal dystrophy (retinitis pigmentosa). USH I patients show vestibular deficits. The incidence of USH I among congenitally deaf children is assumed to be as high as 10%. We intend to create a simple examination procedure for screening congenitally deaf children for vestibular deficiency and subsequently USH I. The examination procedure is named "Minimized Rotation". The vestibular function of deaf children was examined by Minimized Rotation during their preoperative cochlear implant candidacy examination. A lack of postrotational nystagmus was seen as an indication for vestibular deficit. Subsequently some of these patients were examined under general anaesthesia by electroretinography (ERG) at the Department of Ophthalmology. A total of 117 children were examined by Minimized Rotation. In 19 children (16.2%) no rotational nystagmus was found. Six of these children were additionally examined at the Department of Ophthalmology using Ganzfeld ERG. Three of them (50%) showed generalized dysfunction of the retina; 8.1% of the children undergoing preoperative evaluation for cochlear implatation are assumed to show abnormalities of the retina. Rotational examination seems to be an appropriate screening method to detect vestibular deficits, which is one sign of USH I. The results always have to be verified by Ganzfeld-ERG or further genetic investigations. Children with USH I are threatened by progressive reduction of vision. We, therefore, consider USH I children always to be implanted bilaterally with a cochlear implant to maximize the benefit of auditory rehabilitation. SN - 0937-4477 UR - https://www.unboundmedicine.com/medline/citation/18058117/"Minimized_rotational_vestibular_testing"_as_a_screening_procedure_detecting_vestibular_areflexy_in_deaf_children:_screening_cochlear_implant_candidates_for_Usher_syndrome_type_I_ DB - PRIME DP - Unbound Medicine ER -