ABSTRACT. Objective. To find an efficient tool for testing olfactory function in children and use it to investigate a group of children with CHARGE (coloboma, congenital heart disease, choanal atresia, mental and growth retardation, genital anomalies, and ear malformations and hearing loss) syndrome.Methods. We adapted for children an olfaction test that had just been validated in an adult French population and investigated a control group of 25 healthy children aged 6 to 13 years. We then tested the olfactory capacity of a group of 14 children with CHARGE syndrome, aged 6 to 18 years. A questionnaire was completed with the parents about their children's feeding difficulties and their ability to recognize odors in everyday life. We recorded and scored the histories of feeding behavior anomalies, the visual and auditory status, and current intellectual levels. MRI of the olfactory tracts and bulbs was analyzed for 9 of 14 children.Results. We showed that healthy children have similar olfactory function to that of the adult control group, which was representative of the general population, without any difference for either gender or age. We also showed that all children with CHARGE syndrome had olfactory deficiency. Half of them were anosmic, and the others had olfactory residual function (hyposmic). We found no association between olfactory deficiency and feeding behavior, visual or auditory impairment, or intellectual level. Parental subjective evaluations were accurate for only half of the group. All of the MRIs showed anomalies of the olfactory tracts and bulbs varying from moderate hypoplasia to complete aplasia, without any relation between the radiologic and the functional results.Conclusions. Olfaction can be assessed in children, even the young and disabled. Our results support the proposition that rhinencephalon anomalies should be included as a major criterion for the diagnosis of CHARGE syndrome. ABBREVIATIONS. CHARGE, coloboma, congenital heart disease, choanal atresia, mental and growth retardation, genital anomalies, and ear malformations and hearing loss; PEA, phenylethylalcohol. O lfaction has always been and remains the most neglected sense in studies of child development and behavior, the main reasons being the poor knowledge concerning the role of olfaction in human development and behavior and the lack of available tools for investigating olfaction, in particular in children. Development of the olfactory system begins very early in the human embryo; olfactory bulbs have their definitive structure at day 56. Then, cells of the olfactory placode differentiate to gonadotropic cells and migrate to the hypothalamic region along the terminal nerve (cranial nerve 0), the olfactory nerve (cranial nerve 1), and the vomeronasal nerve. One part of the olfactory system projects to the anterior part of the hypothalamus for odor perception and discrimination; the other projects to the limbic system and the hippocampus for the behavioral impact of olfaction and olfactory emotional memory. 1 The Jacobson vome...

To characterize the self-reported olfactory and gustatory symptoms, olfactory function, and causes in parosmia. Design: Assessment of olfactory symptoms and function in patients with a chief complaint of parosmia. Setting: A university hospital clinic and research facility. Patients: Fifty-six consecutive patients presented to the ORL Clinic, European Hospital Georges Pompidou, with a chief complaint of parosmia between October 2001 and November 2003. Main Outcome Measures: Subjective olfactory symptom analysis and olfactory function test results. Results: The mean duration of parosmia was 63.0 months. Forty patients (71.4%) reported associated hyposmia and 16 (28.6%) reported anosmia. Olfactory testing revealed moderate to severe olfactory loss in all patients. Quantitative and qualitative alterations occurred simultaneously in 32 patients (57.1%); parosmia onset occurred within 3 months after quantitative dysosmia in 19 patients (33.9%) and after 3 months in 5 patients (8.9%). The sensation of parosmia was always unpleasant. The main odorant triggers eliciting parosmia are described. The mean severity of flavor dysfunction of the population, evaluated using a 10-cm visual analog scale, was 6.4. Thirty-one patients (55.4%) viewed their olfactory alteration as severely affecting their quality of life. The main clinical association of parosmia was upper respiratory tract infection, found in 42.8% of the patients. Others clinical associations are described. Conclusion: The series of patients with parosmia presented herein, the largest in the literature, permits a clinical description of this rare olfactory abnormality.

Olfactory cleft disease involves various pathophysiologic processes. Computed tomography scanning provides the most information for diagnosis. Indications of functional endoscopic surgery remain to be defined after failure of medical therapy.