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

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 unil...

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Bibliographic Details
Published in:Journal of laryngology and otology 1996-07, Vol.110 (7), p.634-640
Main Authors: Neary, W. J., Newton, V. E., Laoide-Kemp, S. N., Ramsden, R. T., Griffith, G., Evans, D. G., Harris, R., Strachan, T.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Factors
Aged
Biological and medical sciences
Cafe-au-Lait Spots - complications
Cranial Nerve Neoplasms - complications
Cranial Nerve Neoplasms - genetics
Cranial Nerve Neoplasms - pathology
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Evoked Potentials, Auditory
Eye Diseases - complications
Female
Genetics
Humans
Main Articles
Male
Medical sciences
Middle Aged
Neurilemmoma - complications
Neurilemmoma - genetics
Neurilemmoma - pathology
Neurofibromatoses - complications
Neurofibromatosis Type 1
Neurofibromatosis Type 2
Otorhinolaryngology. Stomatology
Pedigree
Schwannoma
Tumors
vestibular
Vestibular Nerve
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Summary: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.
ISSN:0022-2151
1748-5460
DOI:10.1017/S0022215100134486