The objective of this report is to emphasize the clinical features of delayed endolymphatic hydrops in comparison with Ménière's disease. Four cases were selected from a series of 16 examined between the years 1993 and 2005, due to their unusual characteristics. A short clinical history and significant tests are presented for each of the four cases. The cases of delayed endolymphatic hydrops discussed in this article suggest that hydrops is the most important underlying pathology that causes the hearing loss and the vestibular symptoms both in the better ear and in the ear with severe hearing loss. They also suggest that this condition probably occurs in patients with congenitally sensitive ears that make them prone to the development of the late hydrops.