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To assess the efficacy of the current universal neonatal hearing screening program in a tertiary medical institution in Oman, identify its limitations and drawbacks, and explore their causative factors. A retrospective review was carried out to analyse the hearing screening of 12,743 live babies born between January 2016 and December 2018. Screen coverage, drop outs, follow up rate, and age at completion of screening, diagnosis, and intervention were analysed. The results were compared with the Joint Committee on Infant Hearing (JCIH) performance quality indices. Prospective questionnaire-based telephonic interviews were then conducted with the parents or caregivers of neonates with hearing loss. Finally, the causes of loss to follow up or delays in hearing screenings, diagnosis, and/or early intervention were studied. The true prevalence of hearing loss was 4.0 in 1000. The coverage of first-stage screening was 90% whereas the compliance with the second stage was 88.04%. 22.8% of the patients eventually obtained final diagnostic confirmation. The overall compliance with amplification was 30.2%. The completion ages of primary screening and final confirmation were 7.98 and 17.3 weeks respectively. The importance of hearing screening is well received by parents, but problems related to communication, delays in the appointment system, and inefficient follow up tracking were identified as the main limitations and drawbacks of the program. The coverage of the neonatal hearing screening program had not yet reached the required goal of 95%. The performance indicators also fell below the international benchmark. There is a need to address the identified causative factors. Effective communication and well-maintained tracking systems need to be implemented. [ABSTRACT FROM AUTHOR]

Background: Sickle cell disease (SCD) is characterized by intermittent episodes of vascular occlusion and end-organ damage. Neurologic symptoms are frequent and auditory involvement is not unexpected., Aim: To study the prevalence and pattern of hearing loss in Omani patients with SCD., Methods: We conducted a prospective case control study on SCD patients attending the outpatient department. Age and sex matched normal volunteer blood donor controls were recruited after an informed consent and Medical Ethics Committee approval. Pure tone audiometry was performed in all cases studied., Results: Forty-six SCD patients (15 males, 32.6%) aged 16-45 years with a mean age of 26 years ± 6.9 and 29 controls (10 males, 34.4%) aged 16-39 years with a mean age of 25.24 ± 8.2 were enrolled in this study. The average hearing thresholds of SCD patients were consistently higher than controls in all frequencies tested in both ears. Of the 92 ears tested in SCD patients, 29.34% had SNHL. Ten patients had bilateral SNHL; whereas 3 and 4 cases had SNHL in left and right ears, respectively. All the control subjects had hearing thresholds within normal limits., Summary/conclusions: The study reveals a significant incidence of sensorineural hearing loss (SNHL) in SCD patients (36.95%), although the patients were clinically asymptomatic. The hearing loss was worse in the right ears and had a female preponderance. Also, the hearing loss was more severe at the higher frequencies, 2,000-8,000 Hz in SCD patients. HbS, HbF, or low hemoglobin levels did not discriminate SCD patients with SNHL, and the role of hemoglobin F in the cochlea is still not clear. Regular audiometric assessment should therefore be recommended in SCD patients routinely., (Copyright © 2010 The American Laryngological, Rhinological, and Otological Society, Inc.)