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1/3. A case of pure word deafness associated with landau-kleffner syndrome: a long-term study of auditory disturbance.

    A long-term study of auditory disturbance of a female case who had suffered from pure word deafness associated with landau-kleffner syndrome was reported. The patient developed this syndrome at age 4, and we continued the follow-up until she reached 20. The following became clear after the investigation: (1) even by the age of 20 her auditory defect had not improved significantly; (2) from an early stage she could not identify either vowels or consonant-vowel syllables; (3) later she had no difficulty identifying vowels, but her consonant-discrimination score hardly improved; and (4) her problem in consonant identification was unique in that she could discriminate between the voiced and voiceless group but had great difficulty identifying the consonants within each group. These findings led to the conclusion that the patient is unable to recognize short time duration consonants due to an insensibility to loudness and a defect in temporal resolution.
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2/3. Autophony and the patulous eustachian tube.

    Autophony and the patulous eustachian tube is a real but rare clinical entity often misdiagnosed because the symptoms so mimic those of middle ear effusion. The diagnosis is made by the history of fullness or blockage and hearing ones own voice and breath sounds in the ear. The drum is usually atrophic and moves with respiration but this may not be visible. The usual past history is of weight loss. The diagnostic test is to observe a regular increase or decrease in middle ear pressure when increasing or decreasing the ambient pressure while measuring the impedance in a pressure chamber. The usual variations in pressure with opening and closing of the tube are not seen because the tube remains open all the time. Few patients need operative intervention and explanation is all that is usually required. When surgical treatment is necessary the application of 20% silver nitrate to the lumen of the tube on a wire covered by cotton has given results with few complications. Injection of Teflon paste anterior to the mouth of the eustachian tube has been stopped by the manufacturer of the paste because of serious complications caused by the Teflon paste being accidentally injected into the internal carotid artery. Why some patients with a patulous tube and movement of the drug with respiration have no symptoms and other patients with very bothersome symptoms have so few objective signs and no movement of the drum, that can be observed with respiration, remains a mystery that needs further study.
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3/3. Bihippocampal damage with emotional dysfunction: impaired auditory recognition of fear.

    A right-handed man developed a sudden transient, amnestic syndrome associated with bilateral hemorrhage of the hippocampi, probably due to Urbach-Wiethe disease. In the 3rd month, despite significant hippocampal structural damage on imaging, only a milder degree of retrograde and anterograde amnesia persisted on detailed neuropsychological examination. On systematic testing of recognition of facial and vocal expression of emotion, we found an impairment of the vocal perception of fear, but not that of other emotions, such as joy, sadness and anger. Such selective impairment of fear perception was not present in the recognition of facial expression of emotion. Thus emotional perception varies according to the different aspects of emotions and the different modality of presentation (faces versus voices). This is consistent with the idea that there may be multiple emotion systems. The study of emotional perception in this unique case of bilateral involvement of hippocampus suggests that this structure may play a critical role in the recognition of fear in vocal expression, possibly dissociated from that of other emotions and from that of fear in facial expression. In regard of recent data suggesting that the amygdala is playing a role in the recognition of fear in the auditory as well as in the visual modality this could suggest that the hippocampus may be part of the auditory pathway of fear recognition.
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