Auditory Brainstem Response (ABR) in Infantile Gaucher's Disease

Abstract

The auditory brainstem response (ABR) was studied in an infant with Gaucher's disease. The infant was normal until the age of three months. His illness began with stridor, strabismus, inguinal hernia and failure to thrive. Thereafter, muscular rigidity with opisthotonus, ocular palsies, difficulty in swallowing and respiratory failure due to central origin developed. He died of respiratory failure at the age of one year and four months. The ABR was abnormal at six and eight months of age. Initially, at the age of six months, there was a lengthening of the peak latencies of wave I, II and III and disappearance of the waves after IV. But at eight months, more marked lengthening of the peak latencies of the waves and the disappearance of waves after III were noted as his general condition deteriorated. The autopsy revealed relative preservation of the nuclei and tracts of the auditory pathways in the brainstem. The ABR was useful for monitoring the progress of the disease in this patient because it made detection of brainstem lesions possible.

Key words

Infantile Gaucher's disease - Auditory brainstem response (ABR) - Slow vertex response (SVR)