Auditory Brain Stem Responses of Congenital Metabolic Diseases

Kaga Kimitaka, Tanaka Yoshisato

doi:10.4295/audiology.21.127

Patients with congenital metabolic diseases and deafness were investigated by auditory brain stem responses and other audiometric methods.<br>The cases consisted of a case of osteogenesis imperfecta, a case of oculocutaneous albinism, two cases of Hunter diseases (Mucopolysaccharidosis II) and three cases of Leucodystrophy. Auditory brain stem responses were estimated by latencies of wave I, V, V-I at 85dB SL of 3kHz click, the latency intensity curve of wave V and threshold of wave V, and the results were compared with pure tone or speech audiometry.<br>The patients with osteogenesis imperfecta, oculocutaneous albinism and Hunter disease showed mixed deafness or sensory neural deafness, however, the features of their auditory brain stem responses were almost similar to those of sensory neural deafness previously reported and no specific findings due to congenital metabolic diseases were found.<br>Although the cases of Leucodystrophy showed moderate sensory neural hearing loss at the early stage of the illness, the auditory brain stem responses revealed a normal wave form and normal threshold, but the slow vertex responses were poor. Therefore, the central deafness due to cortical or subcortical lesions was suspected. Especially one of three cases showed normal auditory response at the onset, but showed wave I alone at his terminal stage, and this fact suggested that there was the advanced lesion toward the brain stem.<br>We want to emphasize that measurements of the latencies of wave I, V, V-I, the threshold and latency-intensity curve of wave V will be helpful to make differential diagnosis of deafness due to the middle ear, cochlea, brain stem and cortical or subcortical disorders.

Auditory Brain Stem Responses of Congenital Metabolic Diseases

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