The 18q-syndrome is associated with hearing impairment in 50-80 per cent of cases. The hearing loss may be sensorineural or conductive. A high proportion of cases are associated with narrow or stenosed external auditory canals. This may be a useful clinical pointer to the syndrome. Two cases with impaired hearing are presented in this paper including one case with complex external ear and middle ear malformations. The clinical and audiological features in each case are described.