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PubMedDate not supplied

Deficient dietary intake of vitamin E in patients with taste and smell dysfunctions: is vitamin E a cofactor in taste bud and olfactory epithelium apoptosis and in stem cell maturation and development?

OBJECTIVES: We reviewed dietary intake of several nutrients in a large group of patients with taste and smell dysfunction, compared intake of these nutrients with standard values, and recognized that intake of vitamin E was significantly less than that of most other nutrients. Based on this observation we attempted to develop an hypothesis of the possible role vitamin E might play in these sensory disorders. METHODS: Vitamin E intake was measured in 250 patients with taste and smell dysfunctions. RESULTS: Intake of the vitamin was 3.2 +/- 0.2 mg/d (mean +/- standard error of the mean), or 36 +/- 2% of the recommended daily allowance, an intake significantly below that considered adequate. This diminished intake occurred with normal intake of total calories; protein; fat; carbohydrate; several vitamins, including thiamin, niacin, and pyridoxine; and the trace metals zinc, copper, and iron. CONCLUSIONS: Although specific relations between vitamin E intake and smell and taste dysfunctions are unclear, the non-antioxidant roles of vitamin E indicate that it is a factor in apoptosis, cellular signaling, and growth of various cell lines, suggesting that this vitamin may play a role in growth and development of stem cells in taste buds and olfactory epithelium.

Adolescent↗
PubMedDate not supplied

Clinical problems in chemosensory disturbances.

The clinical evaluation of patients with disorders of smell and taste has been hampered by difficulties in stimulus control and response measurement. These senses play a crucial role in food selection and enjoyment and in avoidance of danger, and they deserve the attention of the otolaryngologist as much as audition and vestibular function do. The definition of the degree of disability should be based on psychophysical measures rather than the patient's complaint. An anatomic diagnosis may facilitate the etiologic diagnosis, and it is important to attempt to differentiate transport abnormalities from sensorineural deficits, and receptor deficits from neural deficits. The common causes of smell and taste abnormalities are listed. Selected current work in the field with immediate clinical relevance is reviewed, and opportunities for basic studies that may provide needed data to improve the diagnostic evaluation of patients and to suggest therapeutic strategies for clinical trials are briefly discussed. These areas of study can be pursued with currently available technology.

Chemoreceptor Cells↗
PubMed1988

Assessment of patients with taste and smell disorders.

The evaluation of patients with taste and smell complaints is difficult without standardized quantitative methods of assessment. This paper summarizes recent developments in the evaluation of chemosensory patients. Evaluation must begin with a careful medical history, since certain questions can help in establishing an etiology. Several recent advances have been made in the development of standardized tests for the assessment of taste and olfactory function. Smell can be evaluated with a combination of butanol threshold and odor identification (University of Connecticut test battery) and with the University of Pennsylvania Smell Identification Test (UPSIT). Scores obtained from 91 patients show good correlation between these two testing procedures. Both tests provide normative data for comparison, with the UPSIT including both age- and sex-related norms. Traditional evaluation of taste deficits with threshold procedures has been questioned. Measures of suprathreshold taste intensity require the anchoring of taste judgements to another, presumably normal, modality. This has been approached using magnitude matching, in which taste and auditory loudness are judged on the same scale. Spatial testing of the tongue and soft palate can reveal pathology of the gustatory nerves or their central connections. Further understanding of taste and olfactory dysfunction will be facilitated by the development of standardized methods of evaluation.

Humans↗
PubMed2000

[Psychoosmology at the turn of the millennium: From "nasal reflex neurosis" to the modern psychosomatics of sudden anosmia].

This article offers a psychosomatic description of the medical disorder known as sudden anosmia, an illness which previously has been unjustly neglected. The authors present an operational definition of sudden anosmia and describe the illness's multi-facetted symptomatology. Sudden anosmia is influenced by a number of etiological factors. In this regard, it can be seen that not only classical medical factors such as neurophysiology and anatomy prove to be relevant, but also various characteristics of the fields of personality psychology, family psychology, and behavioral psychology as well. The modern diagnostics of sudden anosmia are conceived interdisciplinarily and span from olfactory measurements to rhino-psychological testing. The treatment of patients suffering from sudden anosmia is based on a multi-staged treatment concept. This concept equally considers both medical and psychological approaches, whereby behavior psychological treatment will be given particular importance in discussion of the latter. Processes for evaluation and quality assurance to monitor the methods of diagnostics and therapy provided for persons afflicted by sudden anosmia are still in their beginning stages.

Humans↗
PubMed1986

Generalized argyrosis in man: neurotological, ultrastructural and X-ray microanalytical findings.

Generalized argyrosis can produce a number of abnormalities, including skin discoloration, liver and kidney dysfunction. We describe a patient with generalized argyrosis following long-term self-treatment with oral silver intake, in whom skin discoloration, progressive taste and smell disorders, vertigo and hypesthesia were observed. These findings were confirmed by chemosensory tests and electrophysiological investigations. The development of hypogeusia was assessed by subjective tests, while the progression of hyposmia was followed by recording olfactory evoked cortical potentials. Light and electron microscopy of tissue samplings demonstrated electron-dense mineral deposits in basal membranes, in macrophages, in the perineurium of peripheral nerves, along elastic and collagenous fibers, and in necrotic cells of the oral submucosa. Silver and sulfur deposits in affected tissues could be defined by X-ray microanalysis. The quantitative ratio between silver and sulfur in involved tissues was similar to that of an inorganic silver-sulfide (Ag2S) standard. The minute increase in the sulfur content when compared to the inorganic standard suggested a sulfur containing organic matrix of the tissue precipitates. Our findings indicate that the affinity of silver for membrane and neuronal structures and the deposition of silver as an insoluble compound (Ag2S) induce the progression of clinical disease.

Ageusia↗
PubMed1990

Design issues in chemosensory trials.

Randomized clinical trials in taste and smell disorders have been infrequent, in part because of difficulties in both the design and the conduct of such trials. Hyposmia and hypogeusia, impairments of smell and taste, respectively, have a variety of causes, such as upper respiratory infection, head trauma, or laryngectomy. Once definitive diagnosis is established, eligibility and exclusion criteria may relate to etiology. Since the sense of smell is more acute at younger ages and since women can generally identify odors more accurately than men, either prestratification or poststratification should be considered. A control or comparison group is essential because, occasionally, chemosenses spontaneously return to normal. Patient recruitment may be difficult, depending to a large extent on self-referrals, with the exception of head injury cases. To enroll an adequate number of patients in a reasonable length of time usually requires participation of multiple clinical centers. Objective measurements of taste and smell must be reliably obtained before and after intervention. Design issues specific to chemosensory trials are discussed in the context of an example, a factorial design trial of surgery and steroids in patients with olfactory defects.

Humans↗
PubMed1995

Olfactory dysfunction in anorexia and bulimia nervosa.

Fifty-five eating-disordered women and 16 normal controls participated in this study to determine whether olfactory function is altered in patients with food-restricting anorexia, anorexia with bulimic features, and bulimia nervosa. Olfactory function was assessed using the University of Pennsylvania Smell Identification Test and by determining phenyl ethyl alcohol odor detection thresholds. Only the very low-weight anorexics showed impairments in their identification and detection of odors. This group's olfactory function did not improve from admission to discharge despite significant weight gain. Although, overall, smoking had only a minor influence on olfactory function, the very low-weight anorexic smokers had the lowest scores of all subjects. Since higher-weight anorexics did not show such impairments, the results suggest that the severe and prolonged starvation experienced by the very low-weight anorexics caused or contributed to intractable deficits in the olfactory system and that these deficits are compounded by smoking.

Adolescent↗
PubMedDate not supplied

[Superficial siderosis of the central nervous system: an uncommon cause of spastic paraparesia].

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS. CLINICAL CASE: We present the case of a 60 year old woman with a history of progressive worsening of a disorder of myelopathic type with signs of pyramidal liberation and sphincter incontinence associated with cerebellar ataxia, anosmia and bilateral hypoacusia. Initially she had unsteadiness, frequent falls and weakness of the legs. Her deafness was more obvious during the previous year. On lumbar puncture there was hemorrhagic CSF with increased red blood cells, iron, ferritin and protein. High field encephalic MR showed a hypointense image in T2 which surrounded the fissure of Sylvius, the brain stem, medulla oblongata, cerebellar hemispheres and sulci of the cerebellar vermis, suggestive of hemosiderosis, atrophy of the cerebellar vermis and slight cortical atrophy. Angioresonance of the intracranial vessels showed that there were no signs of aneurysms or vascular malformations. Transcranial Doppler studies were normal. CONCLUSIONS: Superficial siderosis of the CNS should be included in the differential diagnosis of the parethospastic and ataxic syndromes. The extensive study done ruled out any secondary cause such as chronic bleeding secondary to an arteriovenous malformation or bleeding aneurysm.

Ataxia↗
PubMed1992

Ultrastructural histopathology of human olfactory dysfunction.

This paper presents electron-microscopic observations on biopsies of the olfactory mucosae of several classes of patients with smell disorders: 1) patients with loss of smell function following head injury (post-traumatic anosmics or hyposmics); 2) patients with loss of smell function following severe head colds and/or sinus infections (post-viral olfactory dysfunction, or PVOD); and 3) patients that have lacked smell function since birth (congenital anosmics). Of these, the traumatic anosmics' olfactory epithelia were quite disorganized; the orderly arrangement of supporting cells, ciliated olfactory receptor neurons, microvillar cells, and basal cells was disrupted. Although many somata of ciliated olfactory receptors were present, few of their dendrites reached the epithelial surface. The few olfactory vesicles present usually lacked olfactory cilia. The post-viral anosmics, too, had a greatly reduced number of intact ciliated olfactory receptor neurons, and most of those present were aciliate. The post-viral hyposmics had a larger population of intact, ciliated olfactory receptor cells. In the seven cases of congenital anosmia studied, no biopsies of olfactory epithelium were obtained, indicating the olfactory epithelium is either absent--or greatly reduced in area--in these individuals.

Biopsy↗
PubMed2002

MR evaluation in patients with isolated anosmia since birth or early childhood.

BACKGROUND AND PURPOSE: Anosmias with chromosomal disorders has been well investigated. However, isolated anosmia (IA) has received less attention, although it occurs more frequently. We compared frontobasal structures in patients with IA since birth or early childhood with those in control subjects. METHODS: Imaging findings obtained in 16 patients with IA were compared with those obtained in eight control subjects. Imaging was performed with a standard quadrature head coil at 1.5 T. T1-weighted spin-echo (coronal plane perpendicular to frontal skull base; section thickness, 3 mm; pixels, 0.43 x 0.39 mm) and sagittal T1-weighted magnetization-prepared rapid gradient-echo (voxels, 1.0 x 1.0 x 1.0 mm) sequences were performed. We assessed the length and depth of the olfactory sulcus, olfactory bulb volume, and olfactory sulcus depth in the plane of the posterior tangent through the eyeballs (PPTE). RESULTS: Five patients with IA had bilateral hypoplastic olfactory bulbs. Three patients with IA had hypoplastic olfactory bulbs on the right and aplastic olfactory bulbs on the left. Eight patients with IA had bilaterally aplastic olfactory bulbs. The depth of the olfactory sulcus at the level of the PPTE was smaller in patients with IA than in control subjects. The depth of the olfactory sulcus was greater on the right than on the left, and there was no overlap. Among patients with IA, the depth of the olfactory sulcus differed significantly between those with and those without visible olfactory tracts. CONCLUSION: The depth of the olfactory sulcus at the level of the PPTE reflects the presence of olfactory tracts. The presence or absence of the olfactory tract may therefore have some association with cortical growth of the olfactory sulcus region. The olfactory sulcus is deeper on the right than on the left, particularly in patients with IA. We speculate that olfaction may be processed predominantly in the right hemisphere.

Adolescent↗
PubMed2000

Smell and taste disorders: a primary care approach.

Smell and taste disorders are common in the general population, with loss of smell occurring more frequently. Although these disorders can have a substantial impact on quality of life and may represent significant underlying disease, they are often overlooked by the medical community. Patients may have difficulty recognizing smell versus taste dysfunction and frequently confuse the concepts of "flavor" and "taste." While the most common causes of smell disturbance are nasal and sinus disease, upper respiratory infection and head trauma, frequent causes of taste disturbance include oral infections, oral appliances (e.g., dentures), dental procedures and Bell's palsy. Medications can interfere with smell and taste, and should be reviewed in all patients with reported dysfunction. In addition, advancing age has been associated with a natural impairment of smell and taste ability. A focused history and a physical examination of the nose and mouth are usually sufficient to screen for underlying pathology. Computed tomographic scanning or magnetic resonance imaging of affected areas, as well as commercially available standardized tests, may be useful in selected patients. The causes of olfactory dysfunction that are most amenable to treatment include obstructing polyps or other masses (treated by excision) and inflammation (treated with steroids). Enhancement of food flavor and appearance can improve quality of life in patients with irreversible dysfunction.

Age Factors↗
PubMed1991

Smell and taste disorders, a study of 750 patients from the University of Pennsylvania Smell and Taste Center.

Smell and taste disorders are common in the general population, yet little is known about their nature or cause. This article describes a study of 750 patients with complaints of abnormal smell or taste perception from the University of Pennsylvania Smell and Taste Center, Philadelphia. Major findings suggest that: chemosensory dysfunction influences quality of life; complaints of taste loss usually reflect loss of smell function; upper respiratory infection, head trauma, and chronic nasal and paranasal sinus disease are the most common causes of the diminution of the sense of smell, with head trauma having the greatest loss; depression frequently accompanies chemosensory distortion; low body weight accompanies burning mouth syndrome; estrogens protect against loss of the sense of smell in postmenopausal women; zinc therapy may provide no benefit to patients with chemosensory dysfunction; and thyroid hormone function is associated with oral sensory distortion. The findings are discussed in relation to management of patients with chemosensory disturbances.

Burning Mouth Syndrome↗
PubMedDate not supplied

[Sense of smell in patients with bilateral nasal polyposis].

INTRODUCTION: Sense of smell is susceptible to various changes, both in physiological and in numerous pathological conditions. Of quantitative disorders of smell, hyposmia and anosmia are quite common, whereas of qualitative disorders parosmia is most frequent. The aim of this paper was to examine impact of bilateral nasal polyposis on olfactory function. MATERIAL AND METHODS: The research was carried out at the Nose, Ear and Throat Clinic in Novi Sad. It included 80 examinees, 40 (20 male, 20 female) with bilateral nasal polyposis, while 40 examinees belonged to the control group (20 male, 20 female) without symptoms of nasal polyposes. Fortunato-Niccolini olfactometer was used for this examination. RESULTS AND DISCUSSION: In patients with bilateral nasal polyposis the average perception threshold values for examined odors were 15.50 ccm of odorous air, while in the control group they were 10.20 ccm of odorous air. The average identification threshold values for examined odors in patients with bilateral nasal polyposis were 18.80 ccm of odorous air, while in the control group they were 13.55 ccm of scented air. T-test showed that values of both thresholds were statistically significantly higher (p<0.01) in patients with bilateral nasal polyposis in relation to the control group. CONCLUSION: Olfactory deficit in patients with bilateral nasal polyposis is explained by difficult or impossible passage of odors into the olfactory region.

Female↗
PubMed2007

Clinical assessment and molecular analysis of GnRHR and KAL1 genes in males with idiopathic hypogonadotrophic hypogonadism.

OBJECTIVE: The pathogenesis of idiopathic hypogonadotrophic hypogonadism (IHH) is mostly unclear. We characterized the clinical findings and molecular analysis of GnRHR and KAL1 genes in 26 Brazilian males with IHH with and without hyposmia/anosmia. Design Clinical assessment was performed for endocrine status, olfactory structure and function, renal lesion, and mirror movement. The diagnosis of Kallmann syndrome (KS) included HH and the clinical complaint of hyposmia/anosmia or decreased olfactory acuity obtained by the Smell Identification Test (SIT). We analysed GnRHR and KAL1 genes using the polymerase chain reaction (PCR) direct sequencing method. RESULTS: A variable degree of HH was observed, including various clinical abnormalities, such as cryptorchidism, hearing loss, strabismus, cleft lip/palate, high-arched palate, dental agenesis, psychiatric disorders, learning dysfunction, and bimanual synkinesia. Twenty-two out of 26 patients with IHH (85%) were classified as KS. Abnormalities of olfactory bulbs/sulci were observed in 79% of KS patients. One-third of KS patients had renal defects and 45.5% had a positive family history. GnRHR gene sequence analysis showed no mutations. KAL1 sequence analysis identified two novel missense mutations: c.1061A to G in exon 7 (N304S) and c.1583C to A in exon 10 (S478X). We also observed a 14-bp deletion within exon 11 that caused a premature termination. According to the National Center for Biotechnology Information (NCBI)-Single Nucleotide Polymorphism (SNP) database, two previously described polymorphisms (rs808119 and rs809446) were detected. CONCLUSION: KAL1 mutations accounted for 12% of KS patients. This low prevalence of KAL1 mutations indicates that other genes, such as the fibroblast growth factor receptor 1 (FGFR1) gene or other as yet undiscovered genes, epigenetic events and/or environmental factors might be involved in the aetiology and phenotypic variability of KS.

Adolescent↗
PubMed2004

Reversible kallmann syndrome, delayed puberty, and isolated anosmia occurring in a single family with a mutation in the fibroblast growth factor receptor 1 gene.

Kallmann syndrome (KS) is a clinically and genetically heterogeneous disorder. Recently, loss-of-function mutations in the fibroblast growth factor receptor 1 (FGFR1) gene have been shown to cause autosomal dominant KS. To date, the detailed reproductive phenotype of KS associated with mutations in the FGFR1 has yet to be described. We report a kindred comprising a male proband with KS and spontaneous reversibility, whose mother had delayed puberty and whose maternal grandfather isolated anosmia. The proband presented at age 18 yr with KS and was subsequently treated with testosterone (T) therapy. Upon discontinuation of T therapy, he recovered from his hypogonadotropic hypogonadism, as evidenced by a normal LH secretion pattern, sustained normal serum T levels, and active spermatogenesis. The three members of this single family harbor the same FGFR1 mutation (Arg(622)X) in the tyrosine kinase domain. This report demonstrates 1) the first genetic cause of the rare variant of reversible KS, 2) the reversal of hypogonadotropic hypogonadism in a proband carrying an FGFR1 mutation suggests a role of FGFR1 beyond embryonic GnRH neuron migration, and 3) a loss of function mutation in the FGFR1 gene causing delayed puberty.

Adult↗
PubMed2002

Characteristics of olfactory disorders in relation to major causes of olfactory loss.

OBJECTIVE: To investigate the consequences of olfactory loss and explore specific questions related to the effect of duration of olfactory loss, degree of olfactory sensitivity, and cause of the olfactory loss. PATIENTS: A total of 278 consecutive patients with hyposmia or anosmia were examined. RESULTS: Causes of olfactory loss were categorized as follows: trauma (17%), upper respiratory tract infection (URI) (39%), sinonasal disease (21%), congenital anosmia (3%), idiopathic causes (18%), or other causes (3%). Our data suggest that (1) recovery rate was higher in URI olfactory loss than in olfactory loss from other causes; (2) likelihood of recovery seemed to decrease with increased duration of olfactory loss; and (3) the elderly are more prone to URI olfactory loss than younger patients. Regarding changes in quality of life (QoL), we found that (1) in most patients olfactory loss caused food-related problems; (2) loss in QoL did not change with duration of olfactory loss; (3) younger patients had more complaints than older ones, and women had more complaints than men; (4) complaint scores were higher in hyposmic patients than in anosmic patients; and (5) self-rated depression did not relate to measured olfactory function. CONCLUSIONS: Among many complaints of olfactory loss, the predominant ones were food related. This loss in QoL seemed to be of greater importance in younger than in older people, and women seem to be affected more strongly than men.

Activities of Daily Living↗
PubMed2002

[Objective test of smell with cognitive potentials].

BACKGROUND: An objective smelling test is indicated for a reliable assessment of olfactory disorders. Usually olfactory evoked potentials (OEP) are registered. But the technique of this measurement is complicated and the generation of the OEP depends on the respiration of the subject. Alternatively, the contingent negative variation (CNV) can be used in the diagnosis of anosmia and parosmia, requiring only a simple olfactory stimulator. SUBJECTS AND METHOD: OEP and CNV were derived from 25 adults with normal smelling and from 16 patients with anosmia after head injury. First, the "direct" CNV was registered when the subjects expected a tone following a smell stimulus after 1.5 s. Using two different odors in a random order, the tone only followed one of them, so the "selective" CNV was scored. RESULTS: In both tests a distinct CNV was found in 21 and 23 normal smelling subjects, respectively. OEPs were absent in 4.3 % of this control group. No patient with anosmia showed an OEP or a CNV. The amplitudes of the "selective" CNV are significantly higher than those of the "direct" CNV. No gender dependency was found. CONCLUSION: The results show that an objective olfactometry can be realized by registration of CNV. Contrary to the measurement of OEP which depend on the physical parameters of olfactory stimuli, CNV correlates well with the cognitive identification of odor.

Adult↗
PubMed2003

Identifying the pattern of olfactory deficits in Parkinson disease using the brief smell identification test.

BACKGROUND: Selective olfactory deficits occur in 70% to 90% of patients with Parkinson disease, independent of disease severity and duration. Olfactory testing may be a useful diagnostic aid for Parkinson disease, but the types of odors most commonly affected need to be identified. OBJECTIVE: To determine the pattern and types of odors affected in Parkinson disease by means of the University of Pennsylvania 12-item Brief Smell Identification Test (B-SIT; Sensonics, Inc, Haddon Heights, NJ). DESIGN: Testing patients with Parkinson disease and control subjects in 5 movement disorder clinics. PARTICIPANTS: Forty-nine nondemented patients with Parkinson disease and 52 age- and sex-matched controls. MAIN OUTCOME MEASURES: Normal or abnormal olfactory function was determined in each subject according to predetermined age and sex norms. Predictive statistics and discriminant function analyses were performed to determine the pattern and types of odors best discriminating patients from controls. RESULTS: Abnormal olfactory function was present in 40 (82%) of patients compared with 12 (23%) of controls. The B-SIT score was unaffected by smoking behavior, disease duration, or severity. The sensitivity of the B-SIT for Parkinson disease was 0.82, with a specificity and predictive value of 0.82 and 0.77, respectively. Only 5 of the 12 B-SIT odors (gasoline, banana, pineapple, smoke, and cinnamon) were required to adequately discriminate patients with Parkinson disease from controls. CONCLUSIONS: With the use of the B-SIT, 5 specific odors appear primarily affected in patients with Parkinson disease. Significantly, the ability of patients to detect some odors was unimpaired compared with that of controls. Better diagnostic aids could be developed on the basis of the selective pattern of hyposmia observed in Parkinson disease.

Adult↗