Salama Nader
Department of Surgery (Section of Urology), Taibah Faculty of Medicine, Taibah University, Al-Madinah, Saudi Arabia.; Department of Urology, Alexandria Faculty of Medicine, Alexandria, Egypt.
Int J Reprod Biomed. 2016 Aug;14(8):541-4.
Kallmann syndrome (Kal S) is an isolated form of hypogonadotrophic hypogonadism in combination with a defect in smell sensation. Depending on the genetic form of the disease, a number of non-reproductive, non-olfactory abnormalities may also be existent. In the present report, we describe a male with Kal S associated with hearing loss, and the successful treatment of his sexual and reproductive defects.
A 23-year-old Caucasian man presented with a lifelong lack of erection and ejaculation. The patient reported also anosmia combined with loss of hearing ability. A diagnostic work-up identified the presence of Kal S associated with sensorineural hearing loss. Administration of gonadotrophins regained the erection and a viable-sperm containing ejaculation.
Lack of erection and ejaculation are important components of delayed puberty which could lead to diagnosis of Kal S. The existence of a hearing impairment in the reported patient makes the recommendation to screen the hearing ability in Kal S of utmost importance.
卡尔曼综合征(Kal S)是一种孤立性促性腺激素缺乏性性腺功能减退症,伴有嗅觉缺陷。根据疾病的遗传形式,还可能存在一些非生殖、非嗅觉异常。在本报告中,我们描述了一名患有卡尔曼综合征并伴有听力损失的男性,以及其性和生殖缺陷的成功治疗。
一名23岁的白种男性,自幼缺乏勃起和射精功能。患者还自述嗅觉缺失并伴有听力丧失。诊断检查确定其患有卡尔曼综合征并伴有感音神经性听力损失。给予促性腺激素后恢复了勃起功能,并出现了含有活精子射精。
勃起和射精功能缺失是青春期延迟的重要组成部分,可能导致卡尔曼综合征的诊断。报告患者存在听力障碍,这使得在卡尔曼综合征患者中筛查听力能力的建议至关重要。