Otosclerose infantil: relato de caso e revisão da literatura

A otospongiose e uma osteodistrofia do osso temporal caracterizada pela reabsorcao e neoformacao ossea desordenada. Clinicamente, caracteriza-se por disacusia condutiva, neurossensorial, e/ou mista, progressiva e zumbidos. O inicio dos sintomas ocorre entre 30 e 40 anos de idade sendo rara sua manifestacao na infância. Descrevemos o caso de um paciente de 11 anos de idade, com quadro de hipoacusia unilateral progressiva ha 5 anos. O exame otorrinolaringologico revelou mancha rubra de Schwartze em orelha esquerda. A audiometria, imitanciometria e a tomografia computadorizada demonstraram caracteristicas sugestivas de otospongiose. Realizamos uma revisao dos aspectos clinicos, diagnosticos e da conduta terapeutica da otospongiose na infância. Otospongiosis is an osteodystrophy of the temporal bone, characterized by disordered neoformation and deposition of bone, characterized by the presence of a progressive conductive, sensorineural or mixed hearing loss and tinnitus. Typically, otospongiosis presents as a slowly progressive conductive hearing loss in the third to fourth decade of life. Uncommonly children and adolescents may also have conductive or sensorineural hearing loss caused by otosclerosis. We describe a case of an 11-year-old patient, with progressive unilateral conductive hearing loss for 5 years. The otoscopic examination revealed a positive Schwartz's sign in the left ear. Audiometry, impedanciometry and CT scan showed characteristics that suggested otospongiosis. We reviewed clinical aspects, diagnosis and the therapeutic approach for otospongiosis in children.