Question

Sensory Neural Deafness associated with hereditary nephritis is seen in

• A. Fanconi's syndrome
• B. Berger's disease
• C. Albright's syndrome
• D. Alport's syndrome

Hint:

Think of the type IV collagen defect that damages both the kidney's filtering membrane and the inner ear.

Answer 1

The Correct Option is D

Step 1: Understanding the Question:
We need to identify which condition classically combines hereditary nephritis with sensorineural hearing loss.

Step 2: Key Formula or Approach:
A specific genetic disease affects type IV collagen, a protein found both in the filtering membrane of the kidney and in the structures of the inner ear, so a defect in this collagen damages both organs together.

Step 3: Detailed Explanation:
Fanconi's syndrome is a defect of proximal renal tubular reabsorption, causing loss of glucose, amino acids, phosphate, and bicarbonate in the urine, but it is not classically linked with sensorineural deafness.
Berger's disease, or IgA nephropathy, is an immune-mediated glomerular disease causing recurrent hematuria, but it does not have a classical association with hearing loss.
Albright's syndrome refers to conditions affecting bone and endocrine function, such as McCune-Albright syndrome, and is unrelated to hereditary nephritis with deafness.
Alport's syndrome is caused by a genetic defect in type IV collagen, which is a key structural component of both the glomerular basement membrane in the kidney and the structures of the inner ear. This shared defect explains why Alport's syndrome classically presents with progressive hereditary nephritis together with sensorineural deafness, and often ocular abnormalities as well.

Step 4: Final Answer:
So sensorineural deafness associated with hereditary nephritis is seen in Alport's syndrome.