Try it
ENT · Ear and audiology: osteopetrosis
Sample itemA 2-year-old has anaemia, hepatosplenomegaly, visual loss, facial palsy and conductive deafness. X-rays show uniformly dense bones. What is the basic defect?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Failure of osteoclast resorption causing narrowing of skull foramina
In osteopetrosis, osteoclasts fail to resorb bone, so bone is dense and brittle and marrow space is lost, giving anaemia and extramedullary haematopoiesis. Narrowed optic, facial canal and ossicular spaces cause blindness, facial palsy and deafness.
Option by option
A. Failure of osteoclast resorption causing narrowing of skull foramina (correct)
Correct. Failed osteoclast resorption leaves dense bone and narrowed foramina and canals.
B. Excessive osteoclast activity with disordered remodelling
Excessive osteoclast activity with chaotic remodelling is the process in Paget disease.
C. Unmineralised osteoid from vitamin D deficiency
Unmineralised osteoid from vitamin D deficiency produces rickets, with soft bones rather than dense ones.
D. Defective synthesis of type I collagen
Defective type I collagen is the defect in osteogenesis imperfecta, with fragile bones and blue sclerae.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
More ENT questions
- AudiologyIn a tuning fork test, Rinne is negative (bone conduction louder than air conduction) in…
- EarWhich type of chronic suppurative otitis media is considered 'unsafe'?
- LarynxIn singers, vocal nodules typically occur at:
- Vestibular disordersA patient has recurrent attacks of vertigo lasting hours, with fluctuating low-frequency…