Changes in auditory behaviors of multiply handicapped children with deafness after hearing aid fitting.

Conclusion: The early diagnosis of deafness and the early fitting of hearing aids in multiply handicapped children are recommended for language development in these children even when their neurological or mental status is poor.

Subjects And Methods: The subjects consisted of 5 hearing-impaired infants with no other problems and 28 hearing-impaired children with multiple handicaps. Behavioral audiometry and auditory brainstem responses were used for evaluating hearing impairment.

Three young adult patients with Pelizaeus-Merzbacher disease who showed only waves I and II in auditory brainstem responses but had good auditory perception.

Three young adult males with Pelizaeus-Merzbacher disease have been followed up since childhood. This disease is thought to be a dysmyelinating disorder of the brain during the prenatal period caused by gene mutations. The patients manifested horizontal nystagmus and severe rigidity of the extremities.

Auditory agnosia in children after herpes encephalitis.

Four pediatric patients whose bilateral auditory cortices were damaged by herpes encephalitis at an early age were studied. Their brain CT and MRI scans demonstrated common bilateral lesions of the auditory cortices. Their auditory perception was investigated by means of behavioral and objective hearing tests and auditory perception tests.