先天性失嗅患者的临床研究

目的总结先天性失嗅的分类、临床表现以及影像学特点。方法回顾性分析2004年8月-2006年9月收治的8例先天性失嗅病例的临床资料。4例为伴有其他异常的先天性失嗅患者，其中卡尔曼综合征（Kallmann syndrome）3例，鼻腔鼻窦发育异常1例；另外4例为孤立性失嗅。8例患者均行详细的病史采集、全面体检、T＆T嗅觉检查、嗅觉事件相关电位测试、鼻内镜检查和鼻窦CT检查。7例行嗅觉通路MRI和性激素检测，2例行嗅黏膜活组织检查。结果8例患者自幼均未闻到过任何气味。除1例鼻腔鼻窦发育异常外，其余7例耳鼻咽喉科常规检查以及鼻内镜检查未见异常。主观嗅觉测试均为完全失嗅。嗅觉事件相关电位测试显示最大嗅刺激引不出嗅觉事件相关电位。CT检查显示1例鼻腔鼻窦未发育，其余7例未见异常。MRI检查：6例均为嗅球、嗅束缺失，嗅沟缺失或部分变浅；1例双侧嗅球发育差。内分泌检查：3例青春期后外生殖器以及男性第二性征发育不良，血清睾酮、雌二醇、促黄体生成素以及促卵泡生成素水平低于正常。其余4例性激素检测正常。嗅黏膜活检：1例未见嗅上皮典型结构；另1例为鳞状上皮组织伴慢性炎性病变。结论先天性失嗅诊断应依据病史、专科体检、嗅觉测试、鼻窦cT以及嗅路MRI检查结果。嗅路MRI对诊断有重要价值。     

嗅裂疾病三例

目的报道一种新的嗅觉障碍疾病——嗅裂疾病，总结其临床特征、影像学特点以及治疗尝试。方法本组3例，均以完全失嗅为主诉。详细采集病史，行耳鼻咽喉科常规体检、鼻内镜检查。主观嗅功能测试使用T＆T标准嗅觉计行嗅觉察觉阈和识别阈测试。以醋酸异戊酯为刺激剂行嗅觉事件相关电位测试，刺激量为2ml。行鼻窦冠状位和轴位CT扫描。治疗方案：①抗生素治疗半个月；②糖皮质激素治疗半个月。结果鼻内镜检查患者双侧嗅裂处黏膜肿胀，嗅裂消失。T＆T主观嗅觉测试均为完全失嗅，最大嗅刺激未引出嗅觉事件相关电位。鼻窦CT提示局限于双侧嗅裂的软组织影。抗生素治疗嗅觉无改善。局部和全身糖皮质激素治疗，1例嗅觉改善，但停药后嗅觉又消失，再次使用糖皮质激素治疗无效。另2例嗅觉无改善。结论嗅裂疾病是一种以嗅觉障碍为主诉，病变局限于嗅裂的疾病，鼻窦CT有助于确诊，抗生素和糖皮质激素治疗效果不理想。     

年龄和性别对嗅觉相关电位的影响

嗅觉相关电位（olfactory event—related potentials,OERP）系由嗅刺激剂（odrants）刺激嗅黏膜,应用计算机叠加技术,在头皮特定部位记录到的特异性脑电位。OERP作为一项客观而灵敏的电生理指标,对嗅觉系统及其相关疾病的诊断具有重要的理论和临床应用价值。本研究旨在阐明年龄和性别对OERP各波潜伏期及振幅的影响,以正确分析OERP的测试结果。     

慢性鼻及鼻窦炎患者主客观嗅功能检查的相关性分析

目的 探讨慢性鼻及鼻窦炎(CRS)致嗅觉障碍患者主观嗅功能(T&T嗅觉检查法)和嗅觉诱发电位(olfactory event related potential,OERP)的相关性.方法 89例CRS患者按有无鼻息肉分成两组,记录年龄、性别、症状、鼻窦CT、鼻内镜检查、主观嗅觉功能和OERP.结果 非鼻息肉组T&T得分...     

正常年轻人嗅觉事件相关电位的特点

目的:研究正常年轻人嗅觉事件相关电位(OERP)的特点.方法:嗅觉正常的健康年轻志愿者55例,使用OEP-98C型嗅觉诱发电位仪,以醋酸异戊酯(刺激浓度＜1 648×10-6)为气味刺激剂行OERP测试.在刺激间隔优化测试时,分别采用间隔5次(15 s)、10次(30 s)、20次(60 s)呼吸的刺激间隔.结果:OERP波形分类:①N1P2型;②P1N1P2型;③P1N1P2P3型;④P1N1P2N2P3型.引出率:正常年轻人OERP的引出率为100%.其中P1引出率为60.4%,P2引出率为100.O%,P3引出率为32.1%.男女引出率差异无统汁学意义(P＞0.05).潜伏期:P1、N1、P2、N2、P3潜伏期分别为(233±32)ms、(365±52)ms、(511±90)ms、(625±56)ms、(717±88)ms;男女各波潜伏期差异无统计学意义(P＞0.05).幅值:P1、N1、P2、N2、P3幅值分别为(7.22±5.93)μV、(-6.00±2.56)μV、(16.65±8.19)μV、(6.35±3.55)μV、(13.23±6.93)μV;以上各波男女间幅值差异无统计学意义(P＞0.05).N1P2幅值为(22.58±8.25)μV,男性为(19.53±6.89)μV,女性为(25.24±8.44)μV,女性N1P2幅值大于男性(P＜0.05).随刺激间隔增加,P2和N1P2幅值增加;潜伏期无显著改变.结论:正常年轻人OERP P2的引出率为100%,P1引出率较P3高,性别、刺激间隔足影响N1P2幅值的因素.     

用氦氖(He-Ne)激光治疗嗅觉紊乱的可能性

嗅觉紊乱可影响内脏、情绪和防御功能，患者多，故诊治问题亟待研究。对310例18～70岁的不同原因嗅觉紊乱者进行观察，病因中最常见为患流感或上感后（153例，占49．4％）；次为颅脑外伤（50例，16．l％）；精神情绪创伤（32例，10．3％）。依病程可分为急性（1月以内），亚急性（1～6月）和慢性（6月以上）嗅觉紊乱，以慢性者居多。266例（85．7％）属嗅功能低下，25例为嗅觉过度敏感，17例为恶臭嗅味（其中12例有客观恶臭味，转胃肠科及口腔科治疗）。全部患者均经临床、嗅觉计、鼻窦X线片、断层片，必要时CT和MRI检查及脑电图、鼻分泌…     

嗅觉障碍患者鼻内镜术后嗅黏膜形态学观察

目的 观察嗅觉丧失患者鼻内镜术后嗅觉不同恢复程度、不同时间嗅黏膜的组织学情况.方法 按孙安纳法配制试嗅液,选取慢性鼻窦炎鼻息肉嗅觉丧失患者33例(60侧)功能性鼻内镜术后第4、12、24周测试嗅觉并取嗅区黏膜.术后24周随机选取嗅觉恢复正常、部分恢复和无恢复每组各5例和2例正常者的嗅区黏膜行透射电镜观察.结果 术前嗅上皮均表现为不同程度的损伤.术后4周细胞损伤程度加重.术后12周嗅觉完全或部分恢复组,细胞形态趋于正常,组间无明显差别.嗅觉无恢复组,细胞形态改善.术后24周,嗅觉完全或部分恢复组,细胞形态恢复正常,较术后12周时好,比对照组更佳.嗅觉无恢复组,细胞形态趋于正常.结论 慢性鼻窦炎鼻息肉嗅觉障碍与嗅上皮形态学异常有关.鼻腔通气引流和细胞形态学恢复是嗅觉恢复的必要条件.     

分鼻侧评估嗅觉功能及单侧嗅觉减退的临床分析

目的 了解嗅觉减退患者左右两侧嗅觉功能损伤程度是否相同.方法 对104例以嗅觉减退为主诉的患者,通过病史采集了解嗅觉减退的相关病史及其现况,耳鼻咽喉科常规检查及鼻窦CT检查排除鼻腔结构异常或仅发生于单侧鼻腔-鼻窦的病变,并分别对左、右鼻侧进行T&T嗅觉计嗅觉识别阈测试,评估患者每侧嗅觉功能.结果 本组104例嗅觉减退患者,90例(86.5％)患者双侧嗅觉功能水平相同,14例(13.5％)患者双侧嗅觉功能水平不同,其中6例患者表现为单侧(左侧或右侧)嗅觉功能正常,而另一侧嗅觉功能减退.14例左右鼻侧嗅觉功能不同的患者,其常规查体及影像学检查均未见明显鼻腔结构异常或发生于单侧鼻腔鼻窦的病变.结论 人类的左、右两个鼻腔分别拥有一个完整的嗅觉传导系统,尽管双侧受损概率相同,但受损程度可不同.单侧嗅觉减退作为嗅觉障碍的一种特殊表现形式,只有通过分鼻侧进行嗅觉功能评估,才能更全面地了解患者的嗅觉功能.     

上呼吸道感染和鼻部炎性疾病嗅觉障碍的治疗

目的:观察鼻内气动喷射雾化吸入布地奈德混悬液对上呼吸道感染及鼻-鼻窦炎性疾病相关嗅觉障碍的临床治疗效果.方法:20例嗅觉障碍患者,相关病因包括鼻-鼻窦炎、变应性鼻炎、上呼吸道感染、嗅裂病.在进行治疗前,采用T&T嗅觉检查法和记录嗅觉事件相关电位(OERPs)对嗅觉功能进行评估.然后行鼻腔雾化吸入布地奈德混悬液(1 mg,2 ml),每天1次,共15次.每5次雾化吸入结束后,采用T&T嗅觉检查法复查嗅觉功能.15次雾化吸入全部结束后,同时复查T&T嗅觉识别阈和OERPs.结果:20例上呼吸道感染及鼻-鼻窦炎性疾病相关的嗅觉障碍患者经过鼻腔雾化吸入治疗,5例(25%)患者嗅觉功能恢复正常,10例(50%)患者嗅觉功能明显改善,3例(15%)患者嗅觉功能稍有改善,2例无效;总有效率为90%.结论:鼻腔气动喷射雾化吸入布地奈德混悬液对上呼吸道感染及鼻-鼻窦炎性疾病相关的嗅觉障碍患者具有较好治疗效果,该治疗方法简便、无创、有效,患者依从性好,是较好的治疗方法.     

慢性鼻窦炎嗅觉障碍的嗅粘膜病理学观察

目的观察慢性鼻窦炎嗅觉障碍患者的嗅粘膜病理变化及其与嗅觉障碍的治疗和预后的关系。方法对２３例住院行鼻内窥镜手术治疗的鼻窦炎患者的嗅粘膜进行了组织学观察。术前嗅觉检查均为嗅觉缺失，失嗅时间５～３０年，平均１１．８年。男１５例，女８例，年龄２６～７１岁，平均４８岁。结果２３例（２３侧）嗅粘膜均发现有病理组织学的改变，其中程度不等的嗅上皮萎缩１０侧次（４３．５％），程度不等的嗅细胞减少１６侧次（６９．６％），呼吸上皮化生６侧次（２６．１％），嗅腺粘液腺化生１０侧次（４３．５％）。术后随访２～６个月，嗅觉恢复或改善者１６例（６９．６％），为嗅上皮正常或轻中度病变者。嗅觉无变化７例（３０．４％），为嗅上皮中重度或重度病变者。结论嗅上皮病变程度与术后嗅觉恢复程度成正相关，嗅细胞的数量减少越明显，嗅觉障碍治疗的预后越差。     

Normal sense of smell in Kallmann syndrome. A case report

BACKGROUND: Kallmann's syndrome (KS) was first mentioned in 1944 as an association of anosmia and hypogonadotropic hypogonadism. Causes are multiple genetic defects the most common of which is the x-linked KS appearing mostly in men. However, autosomal dominant and autosomal recessive forms have also been described. PATIENT: We present a case of KS with normosmia (male, 39 years of age). All symptoms of hypogonadotropic hypogonadism were present. RESULTS: Psychophysical olfactory testing revealed left-sided anosmia with right-sided normosmia which was confirmed by electrophysiological measures of olfactory function. Magnetic resonance imaging indicated aplasia of the left olfactory tract and bulb, whereas the right-sided structures appeared to be normal. CONCLUSIONS: As indicated in this case with lateralized anosmia and contralateral normosmia, overall olfactory function strongly depends on the "best" nostril. Therefore, in many clinical situations, lateralized olfactory testing appears to be extremely important.     

Olfactory function evaluated by SPECT

Few articles on neuroimaging techniques in the study of central and peripheral olfactory pathways are present in the literature. By Single Photon Emission Computed Tomography (SPECT), cortical perfusion increment after sensorial stimulation can be evaluated objectively. In the present research, 10 healthy adults underwent SPECT by CER.TO.96 cerebral tomograph, before and after olfactory stimulation with lavender-water. A variable degree of cortical activation was detected in all patients. Gyrus rectus (+24.5%), orbito-frontal cortex (right +26.6%, left +25.6%), and superior temporal (right +9.9%, left +5.5%) cortical areas were always activated. A slight perfusion increase was present in middle temporal (right +3.2%, left +2.1%) and parieto-occipital (right +0.4%, left +2%) regions. Five patients affected by posttraumatic anosmia were also investigated: they showed a perfusion increment markedly inferior to 0.5% in every olfactory area. SPECT is a rather diffused, easily performed technique which yields objective semi-quantitative information on brain perfusion. Hence, it can be regarded as a promising contribution in the fields of smell neurophysiology, clinical olfactometry, and medicolegal queries.     

Steroid treatment of posttraumatic anosmia

The objective of this study was to treat posttraumatic anosmia with oral steroid and evaluate its effect. One-hundred sixteen posttraumatic patients whose olfactory thresholds were –1.0 by the phenyl ethyl alcohol threshold test assembled in our department. They were treated with a course of high-dose steroid, and followed up for at least 3 months. During the latter period of this study, magnetic resonance imaging was performed to measure the volumes of olfactory bulbs and to detect subfrontal lobe damage. Among them, 19 (16.4%) patients’ olfactory thresholds improved after steroid treatment, but the other 97 patients’ thresholds did not change. The incidences of loss of consciousness and intracranial hemorrhage after head injury, the ratios of admission and craniotomy, the intervals between head injury and steroid treatment, the volumes of olfactory bulbs, and the incidences of subfrontal lobe damage were not significantly different between patients whose thresholds improved and those whose thresholds did not improve. However, patients with olfactory improvement were significantly younger than those who remained unchanged. Our study showed that oral steroid treatment might improve olfactory acuity in some patients with posttraumatic anosmia, but the possibility of spontaneous recovery cannot be ruled out.     

嗅觉适应恢复后早期大脑皮层活化的功能性磁共振成像研究

目的 应用功能性磁共振成像技术对嗅觉适应前以及恢复后早期大脑功能活化区进行研究,探讨嗅觉适应机制。方法 给予10名右利手、无嗅觉障碍的受试者2次间隔20 min的相同的嗅觉刺激任务（分别称为任务1和任务2）,刺激剂为异戊酸,功能性磁共振扫描获得图像数据,采用SPM5软件进行数据处理。利用视觉模拟量表对2次任务进行气味强度和愉悦度评分,并将结果进行统计学分析。结果 2次任务的强度评分分别为(6.50±1.11)、(5.40±2.11)分,差异无统计学意义(t＝1.976,P=0.080);愉悦度评分分别为(4.70±1.93)、(4.55±1.50)分,差异无统计学意义(t =0.225,P=0.804)。任务1中大脑活化区域位于双侧小脑、额叶（包括眶额回）、岛叶、丘脑、扣带回、壳、杏仁体、梨状皮层、左侧顶下小叶、中央前回、右侧海马、苍白球、颞中回、缘上回。任务2中,仅见右侧额中回活化,且活化像素显著减少。任务1 -任务2的活化区域位于左中央前回、额叶（包括眶额回）、岛叶、右侧颞上回、小脑;任务2 -任务1的活化区域位于左顶下小叶和右舌回。结论 大脑的嗅觉适应较主观感觉有明显的“延时现象”,主观嗅觉适应已经恢复时,而大脑活化敏感性仍处于较低水平。接受重复嗅觉刺激时,高级嗅觉中枢在气味的感知及嗅觉高级处理过程中所起的作用有限。     

Magnetic resonance imaging for diagnosis of congenital anosmia

Magnetic Resonance Imaging (MRI) was performed on 9 patients who lacked a sense of smell since birth. Seven of them, including two patients with Kallmann syndrome, exhibited abnormality of the olfactory bulb, olfactory tract, olfactory sulcus, or rectus gyrus, with some variation among patients in type and degree of abnormality. The other two patients exhibited normal olfactory pathway morphology, and for them the possibility of acquired sensorineural anosmia could not be ruled out. MRI is useful for determining whether patients with congenital anosmia have olfactory pathway anomalies. Many patients with congenital anosmia and hypoplasty or aplasty of the olfactory pathway nevertheless had no gonadal or endocrinological disorders.     

Brain perfusion single photon emission computed tomography findings in patients with posttraumatic anosmia and comparison with radiological imaging

BACKGROUND: Different techniques in neuroimaging have been proposed for assessment of olfactory dysfunction but they are not without limitations. Recently, some studies showed the usefulness of single photon emission-computed tomography (SPECT) in evaluation of patients with posttraumatic anosmia. This study was designed to assess the possible diagnostic value of SPECT findings in patients with posttraumatic anosmia in comparison with magnetic resonance imaging (MRI)/CT imaging. METHODS: Sixteen patients who had head trauma and consequently anosmia, which was defined according to Cain's identification test, were included in this study. Two nonanosmic groups, traumatic patients and nontraumatic healthy individuals, were selected as control groups for this study. Qualitative and semiquantitative brain perfusion SPECT was performed by measuring the uptake ratio of the orbital frontal cortex to occipital pole in the sagittal projections (uptake index). All 16 target patients had a previous CT scan and/or MRI. Semiquantitative and qualitative brain perfusion SPECT were compared with radiological imaging. RESULTS: Semiquantitative assessment of brain perfusion SPECT revealed remarkable orbital frontal hypoperfusion as compared with two control groups. 87.5% of anosmic patients showed orbital frontal hypoperfusion (-2 SD below the lowest level in healthy controls). In addition, the semiquantitative SPECT method detected more orbitofrontal abnormality than the qualitative method or radiological imaging (MRI and/or CT). CONCLUSION: Findings suggest that by using SPECT, posttraumatic anosmia corresponds to the hypoperfusion in the orbital frontal cortex in a great number of patients. Because of its availability, rather low cost, technical ease, and possibility to obtain objective quantitative information, brain perfusion SPECT can be complementary to other diagnostic techniques in the evaluation of olfactory function, although additional neurophysiological and imaging studies are needed.     

Olfactory bulb volume and depth of olfactory sulcus in patients with idiopathic olfactory loss

Olfactory loss is considered as idiopathic (IOL) when no cause can be found, neither on the basis of a standardized history nor a thorough clinical evaluation. Olfactory bulb volume (OB) has been shown to be decreased in patients with olfactory loss due to trauma, infections, or sinonasal disease. However, OB volume has not yet been investigated in IOL patients. Twenty-two patients with IOL were compared with 22 controls in terms of olfactory performance and of OB volume assessed with magnetic resonance imaging (MRI). Psychophysical testing confirmed that patients had lower scores than controls: P < 0.001 for threshold (T), discrimination (D), identification (I) and global TDI score. Mean right, left and total OB volumes were significantly smaller for IOL patients as compared to controls. Both for IOL and controls, there was a significant correlation between odor thresholds and total OB volume, respectively. In conclusion, patients with IOL have decreased olfactory function and decreased OB volume when compared with controls. This was previously demonstrated for patients with postinfectious or posttraumatic olfactory dysfunction, but not for patients with uneventful conditions possibly damaging their olfactory apparatus.     

Lateralized differences in olfactory function

BACKGROUND: Birhinal testing of odor identification will not allow the detection of unilateral olfactory loss. The aim of the presented study was to evaluate side differences of odor identification in large groups of healthy subjects and in patients with nasal symptoms. PARTICIPANTS AND METHOD: Self-assessment of olfactory function and evaluation of olfactory function by means of a validated test were performed in 479 healthy subjects, in 765 patients with chronic rhinosinusitis (CRS), and in 53 patients with a tumor. A 12-item odor identification test ("Sniffin' Sticks") was used to evaluate olfactory function separately for each nostril. RESULTS: Fifteen percent of the healthy subjects demonstrated side differences in the identification of at least 3 out of 12 odors. Healthy elderly subjects showed larger side differences in identification of odor than younger ones; a general difference between odor identification with the right or left nostril was not found. Both CRS patients and patients with a tumor had larger side differences than healthy subjects. Only 20% of the patients with a tumor complained about impaired olfactory sensitivity, but more than 75% of them showed deficits in olfactory tests. CONCLUSION: Side differences of odor identification of 25% or greater should give reason for further investigation. Future studies are needed to investigate whether side differences in healthy subjects are a predicator of a higher risk for general olfactory loss.