Subcortical deafness as a subtype of auditory agnosia after injury of bilateral auditory radiations caused by two cerebrovascular accidents - normal auditory brainstem responses with I-VII waves and abolished consciousness of hearing.

BACKGROUND: In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness. AIMS/OBJECTIVES: The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all. MATERIALS AND METHODS: Three patients diagnosed as having total loss of hearing participated in this study. We conducted pure-tone audiometry, speech audiometry, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and brain magnetic resonance imaging (MRI) to diagnose cortical deafness with aphasia tests of these patients. RESULTS: Our studies showed that waves VI and VII as well as waves I to V have normal peak latencies in ABRs in all three patients. In brain MRI, we found complete damage of proximal parts of bilateral auditory radiations in the three patients. CONCLUSIONS: We propose 'subcortical deafness' as a subtype of auditory agnosia.

A case of cortical deafness and loss of vestibular and somatosensory sensations caused by cerebrovascular lesions in bilateral primary auditory cortices, auditory radiations, and postcentral gyruses - complete loss of hearing despite normal DPOAE and ABR.

CONCLUSION: A right-handed 38-year-old man's complete loss of hearing could be diagnosed as cortical deafness caused by cerebral vascular lesions in bilateral auditory cortices. OBJECTIVE: The aim of this case report was to study the pathophysiology of a particular patient who manifested deafness without residual hearing and lost somatosensory sensation, vestibular sensation, and articulation ability after a right internal carotid-posterior communicating artery (IC-PC) aneurysm and subarachnoidal hemorrhage. METHODS: MRI, aphasia and neurological tests, subjective and objective audiometry, and vestibular function tests were performed. RESULTS: The neurological test revealed system loss of somatosensory sensation with normal motor function and articulation ability. Brain imaging revealed extensive infarction in the bilateral primary auditory cortices, postcentral gyruses, and the bilateral partial third frontal gyruses. Pure-tone audiometry of both ears revealed off-the-scale results and speech audiometry demonstrated 0% maximum speech discrimination. However, objective audiometry showed normal distortion product otoacoustic emissions (DPOAE) and normal auditory brainstem response (ABR). The patient showed 0% perception of environmental, speech, and music sounds in both ears. He was unable to feel vestibular sensation despite normal caloric nystagmus. He showed no damage to his larynx or articulation organs.

Color picture drawings without form and eye movements: a case report of visual form agnosia in a girl.

The case of an 8-year-old girl who manifested cortical blindness and whose color drawings of faces and objects were without outlines is reported. Her birth was uneventful. When she was 10 months old, she fell down to the floor from a chair, resulting in a subarachnoidal hemorrhage. A repeat brain MRI revealed localized lesions in the visual cortices in the right and left hemispheres. As she grew older she was found to have visual imperceptions. She was found to have difficulties in learning visually the names of objects with form and letters, and in recognizing faces of her family. However, she was able to discriminate well the colors of faces and objects and learn easily the names of the objects with form by touching. She seemed to utilize subcortical vision for seeing colors of faces and objects.

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. The 5 hearing-impaired infants with no other problems underwent hearing aid fitting at approximately 1 year of age and the 28 hearing-impaired children with multiple handicaps underwent hearing aid fitting at various times from 1 to 5 years of age. The effects of their hearing aids were compared on the basis of auditory behavioral changes. The developmental scale of auditory behaviors in infancy that we proposed was introduced to evaluate the development of auditory behaviors. RESULTS: The auditory behaviors of the hearing-impaired children with no other problems showed constant changes with age after hearing aid fitting. However, among the 28 hearing-impaired children with multiple handicaps, 17 showed improvement in auditory behaviors, 5 showed no improvement in auditory behaviors because of the associated severe motor and mental retardation, and epilepsy, and 6 were unable to adapt to wearing hearing aids.

Magnetoencephalography and positron emission tomography studies of a patient with auditory agnosia caused by bilateral lesions confined to the auditory radiations.

The aim of this study was to investigate auditory cortex function in the context of auditory stimuli in a patient with auditory agnosia due to bilateral lesions confined to the auditory radiations. A male patient experienced mild left temporal hemiplegia because of right putaminal hemorrhage at the age of 43 years. Thereafter he recovered completely but hypertension persisted. When he was 53 years old, he suffered left putaminal hemorrhage and went into a coma. After recovering from the coma and right hemiplegia he could hear but could not discriminate speech sounds. Brain CT and MRI demonstrated small bilateral lesions confined to the auditory radiations. Magnetoencephalography demonstrated the disappearance of middle latency responses and auditory-evoked potential studies showed a very small Pa peak. In contrast, a positron emission tomography study demonstrated a marked increase in blood flow in the bilateral auditory cortex in response to both click and monosyllable stimuli. It is speculated that the auditory cortex receives functional projections from the cochlea via non-specific pathways in the cerebral hemispheres.

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. All four patients showed mild or moderate hearing loss in the behavioral hearing test and normal auditory brainstem responses but did not manifest total deafness. Moreover, perception tests involving speech, environmental sounds and music demonstrated that most auditory perception ability had been lost in all patients. On reaching school age, the patients were enrolled in schools for the deaf or special schools for handicapped children.