Rapid Increase in Neural Conduction Time in the Adult Human Auditory Brainstem Following Sudden Unilateral Deafness

Individuals with sudden unilateral deafness offer a unique opportunity to study plasticity of the binaural auditory system in adult humans. Stimulation of the intact ear results in increased activity in the auditory cortex. However, there are no reports of changes at sub-cortical levels in humans. Therefore, the aim of the present study was to investigate changes in sub-cortical activity immediately before and after the onset of surgically induced unilateral deafness in adult humans. Click-evoked auditory brainstem responses (ABRs) to stimulation of the healthy ear were recorded from ten adults during the course of translabyrinthine surgery for the removal of a unilateral acoustic neuroma. This surgical technique always results in abrupt deafferentation of the affected ear. The results revealed a rapid (within minutes) reduction in latency of wave V (mean pre = 6.55 ms; mean post = 6.15 ms; p < 0.001). A latency reduction was also observed for wave III (mean pre = 4.40 ms; mean post = 4.13 ms; p < 0.001). These reductions in response latency are consistent with functional changes including disinhibition or/and more rapid intra-cellular signalling affecting binaurally sensitive neurons in the central auditory system. The results are highly relevant for improved understanding of putative physiological mechanisms underlying perceptual disorders such as tinnitus and hyperacusis.     

Unusual presentation and clinical variability in Belgian pedigrees with progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA

We studied 14 patients from three unrelated Belgian pedigrees with a familial mitochondrial disorder and multiple deletions of mitochondrial DNA (mtDNA). In one family with an oculopharyngeal presentation there is a clear autosomal dominant inheritance. Progressive external ophthalmoplegia (PEO), “ragged red fibres” (RRF) and multiple deletions of mtDNA are common to all three families. Therefore a diagnosis of autosomal dominant progressive ophthalmoplegia with multiple deletions of mtDNA (adPEO) was made in one family at least. Our data confirm the previous observations that adPEO is a systemic disorder rather than a pure myopathy. In our pedigrees frequently associated features include axonal peripheral neuropathy, dysphagia, psychiatric illness, and sudden death. Mild ataxia, pes cavus and mitral valve prolapse with associated mitral insufficiency also occur. In some cases onset is atypical with neuropathy, adolescent onset myopathy or psychiatric illness. In such cases the common features of PEO and muscle weakness always complete the clinical phenotype later during the course of the disease. Biochemical studies on mitochondrial fractions prepared from one patient's muscle, revealed no abnormalities of respiratory chain enzyme activities.     

外伤性截瘫合并耳聋11例临床分析

初步分析了11例外伤性截瘫合并耳聋患者,指出外伤性截瘫多有慢性肾功能不全、肾性贫血等并发症。这些并发症对耳聋的发生有一定的影响,但长时间或反复使用耳毒性药物是一个不可忽视的重要因素。作者对此提出了预防耳聋的一些具体措施,可供临床参考或借鉴。     

豚鼠爆震性聋耳蜗结构与功能的动态变化

目的 探讨爆震性聋与耳蜗损伤之间的关系。方法 通过畸变产物耳声发射及扫描电镜进行研究。结果 豚鼠爆震后即刻出现听阈的提高，与爆震前相比，DPOAEs幅值于1kHz处开始出现非常显著的减低（P〈0．05），在8kHz处两者的差值更大（P〈0．05），DPOAEs幅值随频率升高而逐渐下降，以高频段更为严重。爆震后20天DPOAEs幅值在0．5、0．7、1kHz处基本恢复至爆震前水平，在1．5—8kHz处较爆震后即刻明显提高，但仍低于爆震前水平（各频率均P〈0.05）。爆震后40天DPOAEs幅值与爆震后20天无明显改变（各频率均P〉0．05）。扫描电镜下见豚鼠爆震后即刻出现IHC纤毛排列紊乱，第一排OHC形态基本正常，第二排OHC部分纤毛扭曲或倒伏，尚可看到鸟翼状结构，第三排OHC倒伏、分散，部分折断；20天组IHC纤毛排列仍然紊乱，第一排OHC纤毛基本正常，第二、第三排OHC纤毛排列极度扭曲，以第三排更为严重，少数OHC溶解变性，空位由支持细胞取代；40天组与20天组无明显差别。结论 爆震性聋出现耳蜗HC结构改变及功能减退，提示耳蜗损伤与爆震性聋紧密相关。     

Pure word deafness and pure anarthria in a patient with frontotemporal dementia

A 66-year-old right-handed man developed pure anarthria following pure word deafness. In addition to language disorders, his behavior gradually changed and finally included violence against his wife. Brain magnetic resonance imagings revealed atrophy of the left perisylvian area, which included the inferior half of the precentral gyrus and the upper portion of the superior temporal gyrus, consistent with frontotemporal dementia (FTD). It has been documented as either a disorder of expressive language or as an impaired understanding of word meaning. Unlike with pure anarthria, pure word deafness is not included in the clinical diagnostic current criteria for FTD. However, a large variety of language symptoms can appear in FTD according to the distribution of pathological changes in the frontotemporal cortices. This case suggests that pure word deafness could be a prodomal symptom of FTD.     

中药蝶脉灵注射液在心肺复苏中脑保护作用的实验研究

目的 探讨中药蝶脉灵注射在心脏骤停时对脑复苏的作用。方法 用电刺激导致室颤制备家兔心脏骤停模型，在光镜和电镜下观察大剂量肾上腺素治疗时，蝶脉灵注射液对脑组织的影响。结果 蝶脉灵注射液对脑细胞有明显的保护作用。结论 蝶脉灵注射液能明显改善心脏骤停时脑缺血性损害，因而有利于脑复苏。     

Chameleon voices: Interpreting for deaf parents

As interpreters for their deaf parents, hearing children are a cultural link between two often separate worlds: the Deaf and the Hearing. Data from a 4 year study of adult hearing children throughout the United States indicate significant differences between hearing daughters and hearing sons. Not only were daughters more likely than sons (regardless of birth order or age differences) to interpret for their parents, but daughters were also far more likely to be bilingual: fluent in both spoken English and American Sign Language. A similar gender bias has been observed among the general hearing public: women are far more likely to attend sign language classes and to work as interpreters for the deaf. This paper explores the social mechanisms and cultural values which determine the gender of the way we communicate with one another. Informants' narratives suggest that sign language and the practice of interpreting often touched upon a larger pattern of socialization and status differences between women and men. The discussion then turns to consider how these differences affect the cultural identity of hearing sons versus hearing daughters.     

Correlation between the Ki-67 antigen in the brainstem and physiological data on sleep apnea in SIDS victims

Background: The Ki-67 antigen appears in all human proliferating cells during late G1, S, M and G2 phases of the cell cycle, but is consistently absent in the G_o phase (noncycling) cells. The correlation between Ki-67 in the brainstem and sleep apnea in victims of the sudden infant death syndrome (SIDS) was investigated to elucidate cell kinetics in the brainstem of this condition, which is still the main cause of postneonatal infant death. Materials and methods: Twenty-six cases of SIDS occurred among 38 infants dying under 6 months of age in a cohort of 27,000 infants studied prospectively to characterize their sleep–wake behavior. All the infants had been recorded during one night in a pediatric sleep laboratory some 3–12 weeks before death. The frequency and duration of sleep apnea were analyzed. At autopsy, brainstem material was collected and immunohistochemistry for Ki-67 was carried out. The density of Ki-67-positive neurons was measured semiquantitatively. Correlation analyses were carried out between the density of Ki-67-positive neurons and the data on sleep apnea. Results: Except in two cases in SIDS victims and in one control, the detection of Ki-67 was negative. No correlation analysis between the Ki-67 and of sleep apnea was found. Conclusions: There were no abnormal cell kinetics detected by the demonstration of Ki-67 antigen in the brainstems of SIDS victims.