Association of deafness & nephritis is seen in –
**Core Concept**
The association of deafness and nephritis is a clinical feature of a specific disease entity that involves both the kidneys and the auditory system. This condition is characterized by the involvement of the inner ear and the kidneys, leading to symptoms of hearing loss and renal dysfunction.
**Why the Correct Answer is Right**
The correct answer is associated with Alport syndrome, a genetic disorder that affects the type IV collagen in the kidneys, ears, and eyes. Alport syndrome leads to progressive kidney disease and hearing loss due to the disruption of the glomerular basement membrane and the inner ear structures. The disease is caused by mutations in the COL4A3, COL4A4, and COL4A5 genes, which code for the alpha chains of type IV collagen. The hearing loss in Alport syndrome is typically progressive and can lead to complete deafness in affected individuals.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because it does not specify a condition that is associated with both deafness and nephritis.
**Option B:** This option is incorrect because it does not refer to a well-known disease entity that involves both the kidneys and the auditory system.
**Option C:** This option is incorrect because it does not accurately describe the condition associated with deafness and nephritis.
**Clinical Pearl / High-Yield Fact**
Alport syndrome is an X-linked dominant condition that affects approximately 1 in 50,000 people worldwide. It is characterized by the triad of hematuria, hearing loss, and eye abnormalities.
**Correct Answer:** C. Alport syndrome