Congenital ear anomalies and microtia — formative SAQs

SAQ 1 (10)

A two-day-old is noted at the postnatal check to have an unusual right ear. It is smaller than the left, with a vertical peanut-shaped remnant of cartilage and a lobule but no recognisable auricular architecture, and there is no external canal opening. The left ear is normal. The newborn hearing screen refers on the right and passes on the left. The baby is otherwise well. [1]

a) State the Marx grade of the right ear, and name the associated canal problem and the type of hearing loss expected on that side. (3 marks) [1]

b) Explain, with the embryology, why the inner ear and sensorineural hearing are usually preserved despite the malformed outer and middle ear. (3 marks) [1]

c) Outline the hearing pathway for this child, naming the confirmatory test and the expected finding, and explain why the management is less urgent than in bilateral disease. (2 marks) [11]

d) Describe the staged reconstructive pathway over childhood, naming the typical age of autologous reconstruction and the role of the Jahrsdoerfer CT score. (2 marks) [3]

SAQ 2 (10)

Two children are discussed in the craniofacial clinic. The first is a newborn with bilateral microtia, bilateral absent canals and a bilateral refer on the newborn hearing screen, who also has a small recessed chin. The second is a four-year-old with a preauricular pit, a previously excised branchial neck sinus, a family history of maternal hearing loss and a paternal uncle on dialysis. [11]

a) For the newborn, name the likely syndrome, the two most immediate clinical priorities (hearing and airway), and the specific early hearing intervention and its rationale. (3 marks) [12] [11]

b) For the four-year-old, name the likely syndrome, the single most important additional investigation, and the reason it is urgent. (3 marks) [12]

c) Contrast the management of a soft, structurally complete folded ear detected at birth with that of a true grade III microtia, naming the intervention and the time window for each. (2 marks) [6]

d) Explain how the Jahrsdoerfer CT score guides the choice between atresiaplasty and a bone-conduction hearing device, stating the threshold score. (2 marks) [3]