AURAL SYMPTOMS AS PRIMARY PRESENTATION OF LANGERHANS CELL HISTIOCYTOSIS

Langerhan's cell histiocytosis is an uncommon granulomatous disease, c haracterized by the idiopathic proliferation of Langerhan's cells or t heir marrow precursors. It encompasses the diseases previously associa ted with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christi an syndrome and Letterer-Siwe syndrome. A series of 54 patients were d iagnosed with this condition in Dublin over a 33-year-period (1959-199 2). Twenty-seven patients had aural symptoms, of whom 15 had no other lesions at the time of presentation. Otorrhoea was the most frequent o tological symptom, followed by lesions in the temporal bone. LCH may m imic common aural conditions such as otitis externa, otitis media or a cute mastoiditis and a high index of suspicion is required to recogniz e it on clinical presentation. The clinical presentation of multi-foca l disease may be so dramatic that the otologic findings may be initial ly overlooked. The mortality rate was 14.8%. Therapeutic regimes inclu ded no treatment, curettage, chemotherapy, radiotherapy or multi-modal ity treatment.