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The Scheibe cochlea deformity with macrocephaly: a case for single channal implantation

Published online by Cambridge University Press:  29 June 2007

J. Ray*
Affiliation:
East of England Cochlear Implant Programme, Addenbrooke's Hospital, Cambridge, UK
R. F. Gay
Affiliation:
East of England Cochlear Implant Programme, Addenbrooke's Hospital, Cambridge, UK
Z. H. Vanat
Affiliation:
East of England Cochlear Implant Programme, Addenbrooke's Hospital, Cambridge, UK
J. A. Begg
Affiliation:
East of England Cochlear Implant Programme, Addenbrooke's Hospital, Cambridge, UK
*
Address for correspondence: Mr J. Ray, M.S., F.R.C.S., 89 Longfleet Road, Poole BH15 2HP

Abstract

An 11-year-old congenitally deaf child with bilateral primitive common cavity (Scheibe type) cochleosaccular dysplasia and benign familial macrocephaly was implanted with an extracochlear single channel device with an ear level speech processor. This paper describes the assessment, findings, dilemmas in decision making, surgical procedure and the favourable outcome after implanting. The relevant literature has been reviewed and our case is presented for the unusual combination of features.

Type
Clinical Records
Copyright
Copyright © JLO (1984) Limited 1998

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References

Aleksy, W. (1983) Comparison of benefit from UCHIRNID single-channel extracochlear implant and tactile acoustic monitor. Journal of Laryngology and Otology 18(Suppl): 5557.Google Scholar
Asch, A. J., Myers, G. J. (1976) Benign familial macrocephaly: report of a family and review of the literature. Paediatrics 57(4): 535539.CrossRefGoogle ScholarPubMed
Cole, T. R., Hughes, H. E. (1991) Autosomal dominant macrocephaly: benign familial macrocephaly or a new syndrome? American Journal of Medical Genetics 41(1): 115124.CrossRefGoogle ScholarPubMed
DiLiberti, J. H. (1992) Correlation of skeletal muscle biopsy with phenotype in the familial macrocephaly syndrome. Journal of Medical Genetics 29(1): 4649.CrossRefGoogle Scholar
Jackler, R. K., Luxford, W. M., House, W. F. (1987) Congenital malformations of the ear: a classification based on embryogenesis. Laryngoscope 97(3) (Suppl 40): 214.CrossRefGoogle Scholar
Kunst, H., Marres, H., Vancamp, G., Cremers, C. (1998) Nonsyndromic autosomal dominant hearing loss: a new field of research. Clinical Otolaryngology 23: 917.CrossRefGoogle ScholarPubMed
Okuno, H., Sando, I. (1988) Anomaly of the round window a histopathological study using a graphic reconstruction method. Auris Nasus Larynx 15(3): 147154.CrossRefGoogle ScholarPubMed
Page, E. L., Eby, T. L. (1997) Meningitis after cochlear implantation in Mondini malformation. OtolaryngologyHead and Neck Surgery 116(1): 104106.Google ScholarPubMed
Parks, T. S., Hoffman, H. J., Humphreys, R. P., Chuang, S. H. (1982) Spontaneous cerebrospinal fluid otorrhoea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia. Neurosurgery 11(3): 356362.CrossRefGoogle Scholar
Phelps, P. D. (1992) Cochlear implants for congenital deformities. Journal of Laryngology and Otology 106(11): 967970.CrossRefGoogle ScholarPubMed
Phelps, P. D., Proops, D., Sellars, S., Evans, J., Michaels, L. (1993) Congenital cerebrospinal fluid fistula through the inner ear and meningitis. Journal of Laryngology and Otology 107(6): 492495.CrossRefGoogle ScholarPubMed
Silverstein, H., Smouha, E., Morgan, N. (1988) Multichannel cochlear implantation in a patient with bilateral Mondini deformities. American Journal of Otology 9(6): 451455.Google Scholar