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Aims (1) To identify the yield of aetiological medical investigations in neonates confirmed to have permanent unilateral hearing loss (UHL) following detection by the newborn hearing screening, (2) To identify the association between aetiological medical investigations, risk factors for deafness, audiological profile and stability of hearing loss. Method This was a 14-year retrospective case-note study of 59 newborns with permanent UHL confirmed by Auditory Brainstem Response test performed at a tertiary Paediatric Audiovestibular Medicine Department following referral by newborn hearing screening. The associations between the aetiological medical investigations, risk factors for deafness and audiological profile were analysed using χ 2 statistics. Results High yields were obtained with MRI of internal auditory meati and with CT of the petrous temporal bones (75% and 80%, respectively). Investigation of congenital Cytomegalovirus infection gave a yield of 22%. Significant associations were found between the presence of physical examination signs and a positive CT finding (p=0.005) and the type of UHL (p=0.004). Degree of UHL had a strong association with a positive MRI result (p Conclusion We recommend prioritising imaging studies (MRI and/or CT) and investigation of congenital Cytomegalovirus infection in newborns confirmed with UHL. The results highlight the importance of a comprehensive physical examination as it will guide the medical investigation protocol, especially imaging studies. Those newborns confirmed with congenital Cytomegalovirus infection will benefit from longitudinal follow-up to detect progression of hearing loss. Future research with a larger sample is required for further recommendations.

Fourteen children with salt losing and five children with non-salt losing congenital adrenal hyperplasia were studied. Venous samples were collected for measurement of plasma renin activity, serum 17 alpha-hydroxyprogesterone, testosterone, sodium, and creatinine. Overnight urinary sodium and creatinine excretions were measured after collection on an outpatient basis. Eight 'salt losers' had a raised plasma renin activity despite mineralocorticoid treatment, as did one 'non-salt loser'. Six of the children in whom clinical and biochemical control was inadequate, including the 'non-salt loser', had an increase in the dose of fludrocortisone. When the investigations were repeated one month later, a fall in plasma renin activity accompanied by a fall in 17 alpha-hydroxyprogesterone in all but one patient were found. The dose of mineralocorticoid may be as critical as the dose of glucocorticoid in the management of congenital adrenal hyperplasia, and regular determination of plasma renin activity should be made, particularly if clinical control is difficult.