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Verbal auditory agnosia with focal EEG abnormality: an unusual case of a child presenting to an ENT surgeon with “deafness”

Published online by Cambridge University Press:  29 June 2007

Uday Kale
Affiliation:
Department of Otolaryngology and Head and Neck Surgery, North Riding Infirmary, Middlesbrough, Cleveland.
Mohamed El-Naggar
Affiliation:
Department of Otolaryngology and Head and Neck Surgery, North Riding Infirmary, Middlesbrough, Cleveland.
Maurice Hawthorne*
Affiliation:
Department of Otolaryngology and Head and Neck Surgery, North Riding Infirmary, Middlesbrough, Cleveland.
*
Address for correspondence: Mr Maurice Hawthorne, F.R.C.S., Department of Otolaryngology, North Riding Infirmary, Newport Road, Middlesbrough, Cleveland TSI 5JE.

Abstract

Verbal auditory agnosia implies the failure to recognize sounds in a patient who is nevertheless not deaf. We present a child referred to the ENT outpatients for suspected hearing loss investigation. At one point she had grommets inserted on the basis of a flat tympanogram but with no effect. All hearing tests, including electrocochleography and distraction tests, revealed normal hearing thresholds. At the age of three years and three months, and on direct questioning of the parents, we discovered that the child responded remarkably well to music. Paediatric neurologists made the diagnosis of verbal auditory agnosia. The condition is very similar to another acquired language disorder called Landau-Kleffner syndrome. It is unusual for such a case to present primarily to an ENT surgeon.

Type
Clinical Records
Copyright
Copyright © JLO (1984) Limited 1995

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Footnotes

This paper was presented at the North of England Neurology Association (N.E.N.A.) meeting held at Middlesbrough, April 1994.

References

Hirsch, E., Marescaux, C., Maquet, P., Metz-Lutz, M. N., Kiesmann, M., Salmon, F., Frank, G., Kurtz, D. (1990) Landau-Kleffner syndrome: a clinical and EEG study of five cases. Epilepsia 31(6): 756767.CrossRefGoogle Scholar
Koeda, T., Kohno, A. (1992) Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome. No to Hattatsu 24(3): 262267 (Abstract).Google ScholarPubMed
Marescaux, C., Hirsch, F., Fink, S., Maquet, P., Schumberger, E., Sellal, F, Metz-Lutz, M. N., Alembik, Y., Salmon, E., Frank, O. (1990) Landau-Kleffner syndrome: a pharmacologic study of five cases. Epilepsia 31(6): 768777.CrossRefGoogle ScholarPubMed
Paeto, R., Kajola, M., Korkman, M., Hamalainen, M.. Granstrom, M. L., Han, R. (1991) Landau-Kleffner syndrome: epileptic activity in the auditory cortex. Neuroreport 2(4): 201204.CrossRefGoogle Scholar
Von Suchodoletz, A. (1992) Landau–Kleffner syndrome. Kinderarztliche Praxis 60 (9–10): 294296 (Abstract).Google ScholarPubMed