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
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
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.