Krämer U
Laryngol Rhinol Otol (Stuttg). 1987 May;66(5):275-9.
Neurofibromatosis (also known as multiple neuroma and Recklinghausen's disease) is caused by a deficient proliferation of the neuroectodermal and mesodermal crest. Therefore, a wide variety of symptoms can be found including cutaneous, neurological, ophthalmological, visceral and osseous changes. Hallmark of this disease are so-called café-au-lait areas of pigmentation, or spots. The ENT specialist is confronted with bilateral acoustic tumours, peripheral nerve tumours, cutaneous tumours, tumours of the larynx and alterations of the facial bones. Eight cases of neurofibromatosis are demonstrated, and recent literature is discussed. Management of patients suffering from neurofibromatosis is mainly palliative and symptomatic. To achieve a good result in treatment there must be an effective cooperation between specialists of different disciplines.