A rare neurological disorder of unknown et … A rare neurological disorder of unknown etiology characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in the last 100 years, with a female to male ratio of approximately 3:1. Age of onset varies from infancy to the third decade. BVVL most frequently presents with sensorineural deafness, usually progressive and severe. Lower cranial nerve involvement and lower and upper motor neuron limb signs are common. Other features include respiratory compromise, limb weakness, slurring of speech, facial weakness, and neck and shoulder weakness. Optic atrophy, retinitis pigmentosa, macular hyperpigmentation, autonomic dysfunction and epilepsy may also occur. Approximately 50% of cases are sporaide and 50% familial, of which autosomal recessive is suggested. of which autosomal recessive is suggested.