Sudden onset deafness as a presenting manifestation of chronic lymphocytic leukemia.

An unusual patient with typical Rai Stage 2 (Binet Stage A) chronic lymphocytic leukaemia (CLL), who presented with sudden onset deafness as the initial manifestation of disease is reported. This sensorineural hearing loss improved dramatically after the administration of chemotherapy. This unique observation was also associated with reduction of the circulating B-CLL cells and with the achievement of a partial response, lasting for almost three years. Recently there was another episode of sudden deafness, associated with a rising leukocyte count, and disease activity followed once again by recovery after chemotherapy. Audiograms were recorded showing positive findings on presentation and recovery after therapy. This very rare manifestation of CLL was presumed to be due to infiltration of the cochlear duct or the 8th cranial nerve although all imaging techniques were negative, because of the rapid relief and recovery achieved after specific chemotherapy. The importance of early diagnosis and therapy is stressed in the light of the rapid clinical recovery observed here.