Waardenburg syndrome.

Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

Evaluation of audiovestibular status in leprosy.

One hundred twenty five cases of lepromatous leprosy and 25 cases of tuberculoid leprosy were investigated for audiovestibular status. Impaired hearing was detected in 52 percent and vestibular hypofuction in 7.2 per cent of lepromatous cases. Conductive deafness was due to eustachian tube catarrh secondary to atrophic rhinitis associated with the disease. The perceptive deafness and vestibular hypofuction were due to end organ lesion probably due to E.N.L. reaction. The vestibulococlear nerve involvement was considered to be unlikely. In tuberculoid leprosy derangement in hearing was not observed in any cases.