Ponto-bulbar palsy with deafness (Vialetto-Van Laere syndrome).

The syndrome described by Brown(l) Vialetto(2) and Van Laere(3) is a rare, often familial, characterized by bilateral nerve deafness followed or accompanied by involvement of various motor cranial nerves, e.g., 7th and 9th to 12th, rarely 3rd, 5th and 6th. On rare occasions, there may also be involvement of spinal motor nerves and less commonly of upper motor neurons. The onset of the disease is usually in childhood and the course irregularly progressive. There is paucity of reports of this entity in the World literature. Although Fazio-londe disease has been reported(4), there is yet no report of this rare entity from India to the best of our knowledge. We report a case of Vialetto-Van Laere Syndrome.