Audiologic impairment associated with bilirubin-induced neurologic damage

Olds, Cristen; Oghalai, John S.

doi:10.1016/j.siny.2014.12.006

Cited by 36 publications

(21 citation statements)

References 78 publications

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“…Preterm infants are much more likely to have sensori neural hearing loss (1 in 50) than are normalterm neo nates (1 in 1000) 100 . Risk factors for hearing loss include the need for intensive neonatal care 100 , and hyperbili rubinaemia 99,[101][102][103] . Nearly all individuals who did not sur vive in neonatal intensive care units failed ABR screening, and postmortem analysis revealed bilateral hair cell loss in several infants (selectively in IHCs in three of 15), whereas the number of SGNs was not reduced 104 .…”

Section: Acquired Auditory Synaptopathiesmentioning

confidence: 99%

Auditory neuropathy — neural and synaptic mechanisms

2016

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Section: Acquired Auditory Synaptopathiesmentioning

confidence: 99%

Auditory neuropathy — neural and synaptic mechanisms

2016

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“…Towards the end of the 1970s, electrophysiological tests such as ABR and OAE have been used to better evaluate cochlear hair cells, 8 th nerve and brainstem pathway which allowed a better understanding of the effects of hyperbilirubinemia on hearing function 12 . Although some previous studies argued that the pathology in the auditory tract affects cochlea and/or the 8 th nerve without brainstem involvement; more recently, an auditory disorder characterized by normal cochlear functions (as evidenced by the presence of cochlear microphonics in ABR and the normal OAE response) but unresponsive to treatment or severely impaired ABR has been found related to bilirubin toxicity [13][14][15][16] . This condition which develops at the retrocochlear (neuronal) level, is now described as auditory neuropathy spectrum disorder (ANSD) 4,15,16 .…”

Section: Discussionmentioning

confidence: 99%

Long-term Effects of Indirect Hyperbilirubinemia on Auditory and Neurological Functions in Term Newborns

Besli¹,

Metin²,

Aksit³

et al. 2020

MMJ

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Objective:The aim of this study was to investigate the long-term effects of hyperbilirubinemia on neurological and hearing function in otherwise healthy term newborns with neonatal indirect hyperbilirubinemia. Method: This study was performed prospectively in 41 term newborns hospitalized for indirect hyperbilirubinemia. Patients with no signs of hemolysis were categorized in 3 groups based on stabil levels as sTB <20 mg/dl, 20-24.9 mg/dl, and =>25 mg/dl. Patients with total bilirubin level =>20 mg/dl and hemolytic disease were classified as the fourth group. The relationship between maximum sTB level, duration of exposure to sTB levels >20 mg/dl and etiology of jaundice with neurological and auditory functions was investigated. Detailed neurological examination, Denver II developmental screening test and hearing tests (otoacoustic emissions, OAE and auditory brainstem responses, ABR) were performed to all patients between 18-24 months of age. Results: Neurodevelopmental disorder was found in 5 (12.2%) patients. Hemolytic disease was detected in two of these patients. Hearing loss was found in 4 (9.8%) of the patients. Two of these patients had auditory neuropathy spectrum disorder and the other two had cochlear hearing loss. The sTB levels of all these patients were above 25 mg/dl. No neurological disorder or hearing loss was found in the patients who had stabil of <25 mg/dl. Exposure time to sTB levels above 20 mg/dl was significantly longer in patients with neurological dysfunction and pathologic ABR results (p:0.007, p:0.007; p<0.05). Conclusion:This study demonstrates that kernicterus may develop in term newborns with severe hyperbilirubinemia (sTB>25 mg/dl) without any finding of significant hemolysis. Not only the bilirubin level but also the duration of exposure to high bilirubin levels may be effective in the development of bilirubin neurotoxicity. The high rate of hearing loss in our patients emphasizes the importance of screening for infants with severe hyperbilirubinemia using comprehensive auditory evaluation for early diagnosis of possible hearing loss. ÖZAmaç: Bu çalışmada, neonatal indirekt hiperbilirubinemi dışında başka sorunu olmayan term yenidoğanlarda, hiperbilirubineminin nörolojik gelişim ve işitme fonksiyonu üzerinde uzun dönem etkilerinin araştırılması amaçlanmıştır. Yöntem: Bu çalışma, indirekt hiperbilirubinemi nedeniyle yatırılarak tedavi edilen 41 term yenidoğanda ileriye dönük olarak gerçekleştirilmiştir. Hemoliz bulgusu saptanmayan hastalar sTB <20 mg/dl, 20-24.9 mg/dl, =>25 mg/dl olarak 3 gruba ayrılmıştır. Hemolitik hastalık saptanan ve sTB =>20 mg/dl olan hastalar 4. grup olarak belirlenmiştir. Maksimum sTB düzeyi, sTB >20 mg/dl üzerinde kalma süresi ve sarılık etyolojisi ile nörolojik ve işitsel fonksiyonlar arasındaki ilişki incelenmiştir. Tüm olgulara 18-24. aylar arasında ayrıntılı nörolojik muayene, Denver II gelişimsel tarama testi ve işitme testleri (otoakustik emisyon, OAE ve işitsel beyin sapı cevapları, ABR) incelemesi yapılmıştır. Bulgular: Hastaların 5'inde (%12,2) nö...

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“…NICU infants are more likely to have low birth weight, history of birth hypoxia, hyperbilirubinemia, history of extracorporeal membrane oxygenation, history of sepsis, and ototoxic medication use, which are all known risk factors for hearing loss [10, 18]. Hyperbilirubinemia can be associated with neurologic damage in infants and damage to the auditory system primarily involves the brainstem and cranial nerve VIII, which clinically presents as ASND [19]. Farhat et al compared 2063 NICU infants with 8724 healthy controls in the well-baby nursery and determined that 1.9 % of the NICU infants had confirmed hearing loss compared to 0.3 % of controls [20].…”

Section: Medical Evaluationmentioning

confidence: 99%

Diagnosis and Management of Congenital Sensorineural Hearing Loss

Chen

Oghalai

2016

Curr Treat Options Peds

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Opinion statement Hearing loss is the most common sensory disorder in the USA. The diagnosis of congenital hearing loss starts with newborn hearing screening, which is best performed with auditory brainstem evoked responses in order to avoid the risk of missing auditory neuropathy spectrum disorder. A careful history and physical exam can occasionally help reveal the etiology for congenital hearing loss. Imaging studies, either CT temporal bones or MRI of the internal auditory canals without gadolinium, and genetic testing, in particular for connexin 26, connexin 30, and Pendred syndrome, are the most useful diagnostic tests. Management of congenital hearing loss involves early fitting of amplification. Early cochlear implantation, preferably before 2 years of age, should be strongly considered for children with bilateral severe hearing loss.

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Audiologic impairment associated with bilirubin-induced neurologic damage

Cited by 36 publications

References 78 publications

Auditory neuropathy — neural and synaptic mechanisms

Auditory neuropathy — neural and synaptic mechanisms

Long-term Effects of Indirect Hyperbilirubinemia on Auditory and Neurological Functions in Term Newborns

Diagnosis and Management of Congenital Sensorineural Hearing Loss

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