Kallmann syndrome and deafness: an uncommon combination: A case report and a literature review.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
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.
Nader
N
Salama
Deafness; Hypogonadism; Kallmann syndrome
J Clin Endocrinol Metab. 2013 Jan;98(1):20-30
23118429
J Clin Endocrinol Metab. 1993 Apr;76(4):827-31
8473391
Front Horm Res. 2010;39:51-61
20389085
Am J Med Sci. 1996 Mar;311(3):135-8
8615388
J Pediatr. 2006 Mar;148(3):410-4
16615981
J Clin Invest. 2010 Oct;120(10):3668-72
20940512
Am J Hum Genet. 2013 May 2;92(5):707-24
23643381
Am J Med Genet A. 2010 Nov;152A(11):2796-801
20949504
Ann Endocrinol (Paris). 2010 May;71(3):149-57
20362962
Clin Endocrinol (Oxf). 2001 Aug;55(2):163-74
11531922
Otol Neurotol. 2013 Dec;34(9):1590-4
24232061