Syndrome associated with anosmia is:
**Core Concept**
Kallmann syndrome is a rare genetic disorder characterized by delayed or absent puberty and anosmia (loss of smell). The primary cause of anosmia in Kallmann syndrome is hypoplasia or aplasia of the olfactory bulbs and tracts, resulting from mutations in genes involved in the development of the olfactory system.
**Why the Correct Answer is Right**
Kallmann syndrome is specifically associated with anosmia due to the underdevelopment of the olfactory system. This is caused by mutations in genes such as KAL1, FGFR1, and FGF8, which play crucial roles in the migration and differentiation of olfactory bulb neurons. The anosmia in Kallmann syndrome is often described as congenital or acquired during childhood, and it is typically bilateral and symmetric.
**Why Each Wrong Option is Incorrect**
**Option A:** Goldenhar syndrome is a rare congenital disorder characterized by epibulbar dermoids, hemifacial microsomia, and other ocular and auricular abnormalities. While it may involve some degree of craniofacial deformity, it is not specifically associated with anosmia.
**Option C:** Prader-Willi syndrome is a genetic disorder characterized by short stature, obesity, intellectual disability, and other physical and behavioral abnormalities. While it may involve some degree of sensory impairment, anosmia is not a defining feature of the syndrome.
**Option D:** This option is incorrect because only one of the above options (Kallmann syndrome) is specifically associated with anosmia.
**Clinical Pearl / High-Yield Fact**
Kallmann syndrome is a rare genetic disorder that highlights the complex interplay between genetic and environmental factors in the development of the olfactory system. It is essential to recognize the characteristic features of Kallmann syndrome, including delayed or absent puberty and anosmia, to make an accurate diagnosis.
**β Correct Answer: B. Kallmann syndrome**