A CLINICAL, GENETIC AND AUDIOLOGICAL STUDY OF PATIENTS AND FAMILIES WITH UNILATERAL VESTIBULAR SCHWANNOMAS .1. CLINICAL-FEATURES OF NEUROFIBROMATOSIS IN PATIENTS WITH UNILATERAL VESTIBULAR SCHWANNOMAS

Citation
Wj. Neary et al., A CLINICAL, GENETIC AND AUDIOLOGICAL STUDY OF PATIENTS AND FAMILIES WITH UNILATERAL VESTIBULAR SCHWANNOMAS .1. CLINICAL-FEATURES OF NEUROFIBROMATOSIS IN PATIENTS WITH UNILATERAL VESTIBULAR SCHWANNOMAS, Journal of Laryngology and Otology, 110(7), 1996, pp. 634-640
Citations number
39
Categorie Soggetti
Otorhinolaryngology
ISSN journal
00222151
Volume
110
Issue
7
Year of publication
1996
Pages
634 - 640
Database
ISI
SICI code
0022-2151(1996)110:7<634:ACGAAS>2.0.ZU;2-7
Abstract
Ninety-three patients with unilateral vestibular schwannomas were exam ined in a clinical, genetic and audiological study, to determine wheth er they had features associated with neurofibromatosis Type 1 or neuro fibromatosis Type 2, In 91 families, one patient only was found to be affected with a unilateral vestibular schwannoma. Patients did have a few cafe-au-lait macules, but fewer than six in number. None of the pa tients satisfied the cutaneous diagnostic criteria for neurofibromatos is Type 1. Neither Lisch nodules nor presenile posterior Subcapsular l enticular opacities or cortical opacities were a feature. Five patient s with unilateral vestibular schwannomas are described where the clini cal findings raised the possibility of neurofibromatosis Type 2. It is suggested that certain individuals with unilateral vestibular schwann omas are at risk of developing neurofibromatosis Type 2. Furthermore, the possibility of neurofibromatosis Type 2 should be considered if mo re than one individual in a family is found to be affected with a unil ateral vestibular schwannoma.