No eye for ears

A 50-year-old man was seen for chronic renal failure of unknown origin. He had been diagnosed in 1981 and monitored continuously while his renal functions had deteriorated slowly; his current glomerular filtration rate was 17 ml/min. On this occasion, he was seen by a new doctor who observed that he was hard of hearing. The day was unusually bright and sunny; hence lateral cervical scars, 3 cm in length, were noted bilaterally (Figure 1). The patient recalled that these were from surgery in childhood. On even closer examination, bilateral pre-auricular pits were also found, and the patient could express some whitish material from one of these (Figure 2). Further history taking revealed that his father and grandmother had similar pits, and that his father had died with renal failure of unknown cause. The patient had four children: three of whom had pre-auricular pits and branchial sinuses but no known renal problems. One of his daughters had died in a car accident, and her kidneys had been ‘unsuitable’ for transplantation although details were unavailable (Figure 3).