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In Belgium the product is sold under the name "Rilatine" and in Brazil and Portugal as "Ritalina". Keywords: Neonatal hyperbilirubinaemia, Sensorineural hearing loss, Kernicterus, Exchange transfusion. Incidence of hyperbilirubinaemia varies with ethnicity and geography, with incidence being high in East Asians, native Americans and Greek islanders, and low in African Americans. The major risk factors associated with pathological jaundice are worth taking a look at (Table-1).Of all these risk factors, prematurity appears to be the most significant. There are two major complications of hyperbilirubinaemia: acute bilirubin encephalopathy, and kernicterus. We report two similar cases of infants with bilirubin levels that approached thresholds for exchange transfusion and subsequently developed sensorineural hearing loss as a consequence of neonatal hyperbilirubinaemia. The two cases being presented relate to the Aga Khan University Hospital (AKUH), Karachi, were they came separately between July 2012 and July 2013. Our first patient was a girlborn pre-term at 34 weeks of gestation after an uneventful pregnancy. Neonatal hyperbilirubinaemia, a condition characterised by an excessive concentration of bilirubin in blood, remains the most common health issue in neonatal age group. Some spontaneous improvement in auditory dysfunction as a complication of hyperbilirubinaemia in infants has been reported. Our patients developed unconjugated neonatal hyperbilirubinaemia that required exchange transfusion and subsequently developed early-onset sensorineural hearing loss as evidenced by the results of BAER. These cases emphasise the value of awareness among physicians regarding this condition that can help in timely detection of risk factors and prompt clinical monitoring and follow-up. Although a final conclusion cannot be drawn from a Case Report, but from our two cases it seems re-assuring that the hearing loss may be transient, if developed after severe hyperbilirubinaemia.
Neonatal hyperbilirubinaemia may be complicated by sensorineural hearing loss as auditory pathway is one of the most sensitive parts of the nervous system to this toxic agent, but if all possible risk factors are managed promptly with a vigilant follow-up, then these hazardous complications can be avoided.
We are grateful to the patients and their families for giving their consent for publishing this report. This journal is a member of and subscribes to the principles of the Committee on Publication Ethics. Many times hearing loss is only temporary, due to earwax or fluid build up or infections and can be restored through treatment or surgery. These graphs, created over 15 years ago in the USA, show the the incidence of hearing loss (in green) compared to other neural and sensory pathologies, representing around 10% of the American population. Sensorineural hearing loss (or perceptual hearing loss) is due to the dysfunction of the inner ear (cochlea) and generally manifest as lesions at the level of the hair cells or auditory nerve.Rare cases of central hearing loss also exist. Mouse over to see the two most common cochlear problems that cause sensorineural hearing loss. Mixed hearing lossConductive and sensorineural hearing losses can be combined in the same ear, causing a mixed hearing loss: for example, a problem in the middle ear (such as advanced otosclerosis) can have repercussions on the inner ear and cause mixed hearing loss.

Genetic and acquired hearing lossHearing loss can have a genetic cause, or occur because of pathology (acoustic trauma, infection, ototoxicity, age).
Profound deafness at birthFor every 1000 births, there are 1-1.5 children that are born with severe or profound hearing loss. Childhood deafnessThe large majority of hearing loss originates in the middle ear (em), such as otitis media.
Adult-onset hearing lossThe factors causing acquired hearing loss are always increasing, and now represent the vast majority of hearing loss. Acute bilirubin encephalopathy and kernicterus are known to be the two major complications associated with it, resulting in neurotoxic effects, including sensorineural hearing loss, hypotonia, delayed motor skills and intellectual deficits. To the best of our knowledge, these are the first reported cases from Pakistan that showed some spontaneous improvement over time in sensorineural hearing loss acquired as a complication of neonatal hyperbilirubinaemia. Written consent was obtained from the parents of the patients for sharing our experience with the two babies. No complications were noted during pregnancy or delivery with Activity-Pulse-Grimace-Appearance-Respiration (APGAR) score being 8 at 1 minute and 9 at 5 minutes. A retrospective study found spontaneous improvement in hearing loss in about 50% of infants within 15 months of diagnosis.
Prompt management with phototherapy and exchange transfusion and regular follow-up of the cases revealed spontaneous improvement in hearing loss.
Large-scale studies are required to confirm our findings and to determine the appropriate course of action which should ensure that timely intervention does take place, but dramatic interventions like cochlear implantation should only be opted for after a thorough follow-up and evidence of a stable condition without spontaneous improvement. Follow-up of all infants and of appropriate family counselling regarding all possible outcomes, including remission, is necessary. A child who is hearing impaired will experience difficulty interacting with others.Children are great imitators.
Some children are afflicted with nerve deafness, or sensorineural hearing loss, leading to permanent disability. It can also be acquired due to a genetic predisposition: some people are more affected by infection or trauma than others.
Chronic otitis of the middle ear (me) represent around 20% of these factors, fewer than Meniere's disease (M), which also affects the vesitbular system (causing dizziness).Acoustic trauma (t) is caused by overstimulation by sound and represents the largest factor in adult-onset deafness.
We report two similar cases of neonatal jaundice requiring exchange transfusion that subsequently developed sensorineural hearing loss. Maternal and neonatal risk factors present in the two cases were noted separately (Tables-2 and 3). No neonatal problems or trauma during normal vaginal delivery resulting in cephalohematoma or ecchymoses was reported. Urgent clinical need for accurate and precisebilirubin measurement the United States to prevent kernicterus. Incidence of neonatal hyperbilirubinemia: a population-based prospective study in Pakistan.

Risk factors associated with hearing loss in term neonates with hyperbilirubinemia.J Trop Pediatr. Risk factors for neonatal jaundice inbabies presenting at the University of Benin Teaching Hospital, Benin City. Follow-up studies of patients with neonatal icterus using acoustic evoked potential audiometry.
A hearing impairment that is not noticed results in developmental delays, as well as social and academic problems.Hearing impairment impacts two in every 100 children.
Other factors (in blue) complete the picture - mainly drugs that are toxic to the ear and sudden deafness.Genetic deafness (g) only account for a small percentage in this age bracket, but it is important to note that in numerous cases (acoustic trauma, ototoxicity, Meniere's), there is probably also a genetic factor accentuating or accelerating the acquisition of the hearing loss. Most all hearing impairments can be helped with hearing aids and other hearing loss treatments and devices.
A few rare central causes of hearing loss (involving the brain) exist, but the vast majority are caused by issues in the ear.
This report aims at increasing awareness of this condition among physicians to allow early detection of risk factors and prompt management and follow-up. Early diagnosis and treatment, use of hearing aids, and specialized education for hearing impaired children will help your child gain the skills and tools for successful development. Conductive hearing loss is caused at the level of the outer and middle ear, whereas sensorineural hearing loss (also known as perceptual hearing loss) occurs in the inner ear. These hearing losses may be sensorineural, conductive, or mixed; unilateral or bilateral, mild to profound, and temprary to permanent. All children can be given a complete hearing examination to determine if there is a hearing impairment. Brainstem audiometry-evoked potential (BAER) was done on 13th day of life that showed severe bilateral peripheral auditory pathway dysfunction. Glucose-6-phosphate dehydrogenase deficiency (G6PD) level was 5.0 thus showing the neonate to be G6PD-deficient. She was regularly followed up and improvement in hearing was first noted at 8 months of life when she started responding to verbal commands.
BAER done on 16th dayof life showed severe bilateral peripheral auditory pathway dysfunction.
At a follow-up visit at 1 year 3 months of age, she was able to hear fine and at 2 years, her speech was normal for age. Magnetic resonance imaging (MRI) done on 16th day of life showed changes of hyperbilirubinaemia encephalopathy.
Spontaneous improvement in hearing was first noted at the age of 1 year when he started speaking words and responding to verbal commands.

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