Olfactory function in patients with hypogonadotropic hypogonadism: an all-or-none phenomenon?

Abstract

Hypogonadotropic hypogonadism (HH) refers to an endocrine defect of hypothalamic origin resulting in gonadal hypoplasia and frequently associated with anosmia or severely impaired olfactory function (Kallmann's syndrome). This apparently results from a disruption in the migration of neurons from the olfactory placode to the bulb and hypothalamus early in development, and so provides a unique opportunity to investigate olfactory function in human subjects with congenitally incomplete peripheral systems. Olfactory performance in 37 HH patients and 37 age-matched controls was compared using a modified version of the Munich Olfaction Test. This test is based on the sniff-bottle method and includes tests of (i) odor quality discrimination, (ii) intensity discrimination, (iii) detection thresholds, and (iv) recognition, hedonic evaluation and identification ability. The patients could be divided into two distinct groups differing significantly on all four subtests and showing no overlap in performance: 20 anosmics, conforming to Kallmann's syndrome, and 17 apparent normosmics whose performance was slightly poorer, but not significantly different to that of the controls. The unexpected failure to find a continuum of olfactory dysfunction now raises the question whether HH with or without anosmia represents two syndromes with distinct etiologies, or rather reflects the ability of the olfactory system to function well despite morphological impairment.