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A clinical, genetic and audiological study of patients and families with unilateral vestibular schwannomas. I. Clinical features of neurofibromatosis in patients with unilateral vestibular schwannomas

Published online by Cambridge University Press:  29 June 2007

W. J. Neary ,
V. E. Newton ,
S. N. Laoide-Kemp ,
R. T. Ramsden ,
G. Griffith ,
D. G. Evans ,
R. Harris  and
T. Strachan
W. J. Neary
Affiliation:
Centre for Audiology, Education of the Deaf and Speech Pathology (CAEDSP), University of Manchester, UK
V. E. Newton
Affiliation:
Centre for Audiology, Education of the Deaf and Speech Pathology (CAEDSP), University of Manchester, UK
S. N. Laoide-Kemp
Affiliation:
Centre for Audiology, Education of the Deaf and Speech Pathology (CAEDSP), University of Manchester, UK
R. T. Ramsden
Affiliation:
Department of Otolaryngology, Head and Neck Surgery, Manchester Royal Infirmary, UK
G. Griffith
Affiliation:
Manchester Royal Eye Hospital, St Mary's Hospital, Manchester, UK
D. G. Evans
Affiliation:
Department of Clinical Genetics, St Mary's Hospital, Manchester, UK
R. Harris
Affiliation:
Department of Clinical Genetics, St Mary's Hospital, Manchester, UK
T. Strachan
Affiliation:
Department of Human Geneticstt, University of Newcastle-upon-Tyne, UK
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Abstract

Ninety-three patients with unilateral vestibular schwannomas were examined in a clinical, genetic and audiological study, to determine whether they had features associated with neurofibromatosis Type 1 or neurofibromatosis Type 2. In 91 families, one patient only was found to be affected with a unilateral vestibular schwannoma. Patients did have a few café-au-lait macules, but fewer than six in number. None of the patients satisfied the cutaneous diagnostic criteria for neurofibromatosis Type 1. Neither Lisch nodules nor presenile posterior subcapsular lenticular opacities or cortical opacities were a feature. Five patients with unilateral vestibular schwannomas are described where the clinical findings raised the possibility of neurofibromatosis Type 2. It is suggested that certain individuals with unilateral vestibular schwannomas are at risk of developing neurofibromatosis Type 2. Furthermore, the possibility of neurofibromatosis Type 2 should be considered if more than one individual in a family is found to be affected with a unilateral vestibular schwannoma.

Keywords

Schwannoma, vestibularNeurofibromatosis Type 1Neurofibromatosis Type 2Genetics
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 110 , Issue 7 , July 1996 , pp. 634 - 640
Copyright
Copyright © JLO (1984) Limited 1996

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