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[Olfactory cortical evoked potentials].

The objective evaluation of smell is the least used functional ear, nose and throat exploration. Evoked potentials can be recorded after chemical olfactory stimulation using a method similar to recording cortical auditory evoked potentials. Our results with this technique and peppermint oil as the stimulant were fully satisfactory. In 10 patients with normal smell, a characteristic wave with a long latency was recorded in the absence of auditory and visual stimuli. In contrast, in 10 anosmic patients this characteristic recording was absent. The easy recording technique proposed here can be useful for olfactory assessment of objective disorders, detection of malingers, and medical-legal reports.

Adult↗

Scent of a disorder: olfactory functioning in schizophrenia.

The use of olfactory probes to assess frontal and temporal-limbic system functioning in patients with schizophrenia has garnered increasing interest among basic and clinical investigators. Deficits in odor identification, detection threshold sensitivity, discrimination, and memory have been reported and are thought to represent a centrally mediated deficit in the processing of this information. These impairments are seen in affected probands, first-degree family members, and those at risk for developing the illness, suggesting a genetic vulnerability or predisposition to chemosensory abnormalities. The observed deficits are not explained by gender, medication use, cognitive impairment, or smoking status, and support the hypothesis of primary dysfunction in the olfactory system. Along this same line, structural abnormalities in the peripheral and central olfactory brain regions, as well as disruptions of the basic physiology of this system, have been described. The study of olfactory processing in schizophrenia has already advanced the knowledge of the neural substrate for this disorder. Because the olfactory system continuously regenerates throughout life, it allows for a unique view of an ongoing neurodevelopmental process.

Humans↗

University of Pennsylvania Smell Identification Test: a rapid quantitative olfactory function test for the clinic.

Despite the fact that clinical otolaryngologists are often presented with complaints of olfactory dysfunction, they have limited means to diagnose these problems. A major reason has been the lack of a clinically-useful and reliable quantitative test of olfactory function. Recent work at our Clinical Research Center has resulted in the development of such a test. This test--the University of Pennsylvania Smell Identification Test (UPSIT)--can be self-administered and uses microencapsulated odorants which are released by scratching standardized odor-impregnated test booklets. As indicated in this paper, studies have demonstrated that the UPSIT can identify most malingerers and is sensitive to age, gender, smoking habits, and a wide variety of olfactory disorders. A description of this new test, along with some of its applications, is presented.

Humans↗

[Presymptomatic detection of Parkinson's disease].

Parkinson's disease (PD) is characterized by a progressive degeneration of mesencephalic dopaminergic neurons. More than half of these neurons are lost in a presymptomatic phase of an estimated 4-6 years duration. It is obvious that any type of treatment aimed at slowing down the disease process should preferably be applied in this presymptomatic phase. Presymptomatic detection of PD has therefore become an important goal. In a recent study in a population of 361 asymptomatic first degree relatives of PD patients, we were able to demonstrate that presymptomatic detection is possible by means of a combination of three olfactory processing tasks and [123l] beta-CIT single photon emission computed tomography (SPECT) scanning of the nigrostriatal dopaminergic system. These results are a first step towards the development of a screening strategy that may be applied in the general population. Impairments of olfactory function, however, are not specific to PD but are also associated with other neurodegenerative disorders (e.g. Alzheimer's disease) and certain lifestyle characteristics (e.g. smoking). In the next few years our research efforts will focus on two different approaches to develop a more specific screening strategy. First, olfactory processing tasks will be combined with tasks aimed at detecting subtle (visuo)motor disturbances and early cognitive impairments. In parallel, an effort will be made to define disease-specific patterns of olfactory dysfunction in neurodegenerative disorders.

Aged↗

Olfactory function in people with genetic risk of dementia.

BACKGROUND: Screening for sensorial impairment is a secondary objective in the context of neurodegenerative diseases, including dementias. For example, olfactory dysfunction is among the first signs of Alzheimer's disease. There has been no study of olfactory function in Irish subjects at risk of dementia. AIM: To investigate olfactory function in non-demented Irish persons, who carry genetic risk factors for dementia. METHODS: Thirty-eight Irish adult subjects, who are at risk of dementia, were recruited. Cognitive performance and olfactory function were assessed and apolipoprotein E (APOE) genotype determined. RESULTS: Three and six subjects had a Mini Mental State Examination (MMSE) and Brief Smell Identification Test (B-SIT) score, respectively, outside the normal range. While five out of the fifteen epsilon-4 allele positive subjects had B-SIT scores outside the normal range, only one out of the twenty-three epsilon-4 allele negative subjects had; the difference in this frequency was significant (P=0.025). There was no significant difference (P=0.266) in the frequency of abnormal MMSE scores between epsilon-4 allele groups. CONCLUSION: Further investigation is required to explore the reasons for the higher prevalence of olfactory dysfunction in epsilon-4 allele positive subjects.

Age Distribution↗

[Olfactory disorders].

Smell and taste problems are of major importance to those who suffer from olfactory disorders. The inability to determine the presence of odors in the home and the markedly reduced capacity or incapacity to appreciate food flavors are key reasons given for limited social interaction. Patients experiencing distorted smells and tastes may avoid food, which results in weight loss and possible malnutrition. We present an overview of smell disorders, based on physiological considerations, with specific attention to clinical characteristics of conditions most commonly causing smell disorders.

Adult↗

Olfactory deficits in patients with schizophrenia and severe polydipsia.

BACKGROUND: The present study was designed to assess olfactory function in severely polydipsic/hyponatremic patients with schizophrenia who also had intermittent water intoxication. METHODS: The University of Pennsylvania Smell Identification Test and an olfactory acuity battery were administered to three groups of male subjects: 9 patients with schizophrenia and severe polydipsia/hyponatremia, 9 control nonpolydipsic/normonatremic patients with schizophrenia, and 9 normal controls. RESULTS: Male patients with severe polydipsia/hyponatremia and intermittent water intoxication had marked olfactory acuity and identification deficits when compared to the patient control group of similar age and age at illness onset, and to normal controls. CONCLUSIONS: The finding of deficient acuity (detection threshold) in the polydipsic/hyponatremic group but not the nonpolydipsic, normonatremic group suggests that for this subgroup, abnormalities of olfactory sensory function may occur in a pattern previously reported for other brain disorders such as Alzheimer's disease.

1-Butanol↗

Sniffin'Sticks: a new olfactory test battery.

The olfactory test battery "Sniffin'Sticks" comprises a perception threshold test, an odour discrimination test and an odour identification test. The purpose of this study was to examine the suitability of the Sniffin'Sticks for use in everyday practice and to obtain (at least provisional) normal values. Thirty normosmic and 15 anosmic volunteers were examined with the Sniffin'Sticks and the "University of Pennsylvania Smell Identification Test" (UPSIT). All three Sniffin'Sticks tests distinguish between normosmics and anosmics in a highly significant manner. The good correlation of the individual tests with each other and with the results of the UPSIT documents the reliability of the test results. Critical mention must be made of the overly complex determination of the olfactory threshold. In conclusion, the Sniffin'Sticks test battery provides a validated instrument adapted to European conditions for the examination of olfactory disorders. It has proven successful in everyday clinical practice and constitutes a major aid for compiling medical certificates.

Humans↗

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.

Adolescent↗

[Exploration of olfactory difficulties].

Sense-of-smell disorders are a frequent occurrence at ORL clinics. The clinical exploration must include a thorough and comprehensive interview to discover the etiology. The clinical examination is easily performed using cold-light endoscopes, which can reveal anatomical or mucous membrane pathologies, even in far- situated anatomical parts. The access to olfactometric examinations is limited by the small number of health centers which have the proper equipment and trained staff. Guided by the initial interview and clinical exploration, the diagnosis of smell disorder can benefit from the conclusive and factual contribution of the CAT scan and MRI.

Diagnostic Imaging↗

[Parosmias--definition and clinical picture].

The paper proposes the definition and technical terms with greater precision for the description of qualitative smelling disturbances. Thereafter, parosmias are relevant only for the ENT specialist. Their further classification seems to be unnecessary or even false. These parosmias are always described to be troublesome for the patient since he feels the abnormality of the olfactory sensation. Thus, a clear distinction against phantosmias (olfactory hallucinations) and pseudoosmias (olfactory illusions) is given. The cause of parosmias is frequently speculative. Most frequently there occur traumas and influenza. Their treatment is equally the same as in postinfluenza hyposmias. Additionally, the dripping of 4% novocaine or 10% cocaine on the olfactory mucosa is performed. The parosmia is defined to be an independent entity of disorder, not only accompanying hyposmia. The parosmia may occur separately, too, and it does not necessarily recede together with hyposmia and anosmia.

Hallucinations↗

Hypogandotropic hypogonadism with anosmia: the Kallmann syndrome.

Five cases are presented and the literature reviewed concerning the association of anosmia or hyposmia and hypogonadotropic hypogonadism (Kallmann syndrome). Two of the five cases are sporadic and without associated anomalies, the third is associated with ichthyosis and epilepsy, the fourth has a sister with hypogonadism and cleft lip and palate while the fifth case also has Albright's hereditary osteodystrophy (pseudopseudohypoparathyroidism). It is concluded that "Kallmann syndrome" is present in a wide spectrum of disorders with different genetic etiologies.

Adult↗

Urinary steroid pattern in male hypogonadotrophic hypogonadism.

During clinical studies on a 34-year-old man with marked hypogonadism secondary to pituitary gonadotrophin insufficiency, an attempt was made to determine whether the testes were capable of androgen production when stimulated by exogenous chorionic gonadotrophin (A.P.L.).Androgen excretion before and after chorionic gonadotrophin administration was studied. Total and fractionated 17-ketosteroid patterns suggested a severe Leydig cell deficiency which was confirmed by testicular biopsy.Panhypopituitarism was considered excluded by a normal response to metyrapone ditartrate, by a normal serum PBI and a normal radioactive iodine uptake. Genetic disorders were likewise excluded to the extent possible by determining that the chromosome pattern was normal and by determining that no similar condition existed in another member of the same family. The association of anosmia with gonadotrophin insufficiency in this case suggests a primary disorder in the hypothalamo-mamillary-tuber cinereum area.

17-Ketosteroids↗

Olfactory loss as a result of toxic exposure.

Olfactory loss can occur through accidental exposure, poor industrial hygiene, or exposure to low levels of toxins in the ambient air over long periods. This loss can lead to transient olfactory disorders, irreversible anosmia, temporary olfactory fatigue, or industrial anosmia. Inevitably, a practicing otolaryngologist will encounter a patient with complaints of decreased smell and taste that initially may be difficult to diagnose and treat. Much of the challenge in evaluating a patient with disturbances of olfaction is in obtaining adequate quantitative measurements of sensory dysfunction and identifying a source for the olfactory loss. Although there is no particular test for environmental toxins as a source of olfactory loss, an accurate cause can be determined by obtaining a careful, detailed history. A significant exposure history and lack of more common causes of olfactory loss strengthens an argument for environmental toxins as an etiology. Unfortunately, no available treatments can reverse permanent damage caused by toxic exposure, but removal from the source of toxins may allow for repair of the olfactory system and return of normal function, especially in acute exposures. Despite the increasing number of studies investigating toxic exposure on olfactory function, these effects are understood poorly. With continued study of human exposure to these substances and the use of animal models, the mechanisms by which damage occurs will be understood better and new approaches for diagnosis and treatment will be developed. Furthermore, with increasing regulations of occupational environments and stricter policies on industrial air pollution, olfactory dysfunction secondary to toxicity should become less prevalent.

Drug-Related Side Effects and Adverse Reactions↗

Ear, nose, and throat manifestations of Sjögren's syndrome: retrospective review of a multidisciplinary clinic.

OBJECTIVE: To assess the otolaryngologic manifestations of Sögren's syndrome (SS). DESIGN: A retrospective case study. SETTING: The Toronto Hospital. METHOD: Case note review of 196 patients evaluated in a multidisciplinary clinic for this disease. Patients were retrospectively classified according to the revised international classification. MAIN OUTCOME MEASURES: The prevalence of subjective and objective audiologic and otorhinolaryngologic abnormalities. RESULTS: One hundred eleven patients were diagnosed with primary and 26 with secondary SS, leaving 60 with unclassified sicca syndrome. There was minimal otologic pathology. There was a mildly increased prevalence of sensorineural deafness in secondary SS (41-60 years, 36%; 61-80 years, 70%). Approximately 50% of patients in each group complained of nasal symptoms, but only 20% had abnormal findings on rhinoscopy. Similarly, 60 to 70% in each group complained of throat symptoms, but only 20% had abnormal findings on indirect laryngoscopy. Thirty-eight percent of primary patients had parotid gland symptoms, and 25% had abnormally swollen glands, with eight subsequently diagnosed with lymphoma. No patients in the other two groups had abnormal parotid glands. CONCLUSIONS: SS does not appear to be associated with increased otologic or audiologic disease, except perhaps in conjunction with systemic autoimmunity. Nose and throat symptoms are common in SS, but the complications of mucosal dryness on examination are unusual (approximately 20%). Primary SS can cause serious parotid morbidity secondary to inflammation and infection. There is also a significant risk of lymphomas that often present as parotid masses, necessitating long-term follow-up.

Aged↗

[Drug therapy for disturbances of smelling].

Disorders of the sense of smell are frequent. This severely can affect the quality of live. Appropriate testing is needed to determine the nature of the patient's complaints. Following the pathogenesis, two main groups of smell disorders are discernable: The conductive-respiratory smell disorders, where the odour can not reach the receptor and the senso-neuronal disorders where perception or transduction is affected. Therefore two different therapeutical approaches are described.

Adrenal Cortex Hormones↗

[Meningioma with pseudo-vascular disclosure].

In a retrospective study of 125 case reports of intracranial supratentorial meningiomas, the authors have isolated 10 patients whose meningioma had been revealed by pseudo-vascular syndromes. Mean age and sex ratio were the same in this group of patients as in those patients whose meningioma had been more classically disclosed by an epileptic seizure, a motor or a progressive sensorimotor deficit. The site of the tumour exerted an influence on the nature of clinical manifestations. There was no correlation between the size of the tumour and the transient or prolonged course of the pseudo-vascular syndrome. The pathogenesis of these disorders is discussed.

Adult↗