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

This question asks which condition classically combines hereditary kidney disease with sensorineural hearing loss.

1. Fanconi's syndrome: This causes a proximal renal tubular reabsorption defect but has no classical link to deafness.
2. Berger's disease: This IgA nephropathy causes recurrent hematuria but is not associated with hearing loss.
3. Albright's syndrome: This affects bone and endocrine tissues and has no connection to hereditary nephritis with deafness.
4. Alport's syndrome: A defect in type IV collagen, present in both the kidney's glomerular basement membrane and the inner ear, causes this syndrome's classic combination of progressive hereditary nephritis and sensorineural deafness.

Because type IV collagen is shared between the kidney's filtering membrane and inner ear structures, only Alport's syndrome produces this exact combination.

Let's summarize:

• Type IV collagen is a component of both the glomerular basement membrane and the inner ear.
• A defect in this collagen in Alport's syndrome damages both organs, causing hereditary nephritis with deafness.

So this presentation is seen in Alport's syndrome.