Etiologies & High-Risk Factors for Hearing Loss in Children PDF

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Universiti Kebangsaan Malaysia

Dr Nor Haniza Abdul Wahat

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hearing loss childhood hearing risk factors pediatrics

Summary

This presentation discusses etiologies and high-risk factors for hearing loss in children, including genetic factors, infections (like TORCH), and prematurity. It provides detailed information on prevalence, risk indicators, and potential complications.

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Etiologies & High- Risk Factors for Hearing Loss in Children NNNA2212 Associate Prof Dr Nor Haniza Abdul Wahat Why is it important to know about etiologies and high-risk factors for HL? 0 Loading… Projected...

Etiologies & High- Risk Factors for Hearing Loss in Children NNNA2212 Associate Prof Dr Nor Haniza Abdul Wahat Why is it important to know about etiologies and high-risk factors for HL? 0 Loading… Projected Increase in Prevalence of Hearing Loss, 2019-2050 Demographic and population trends reflect the high and rising prevalence of hearing loss globally across the life course. The number of people with hearing loss may 0 increase more than 1.5-fold during the next three decades, with over 700 million likely to experience a moderate or higher level of hearing loss. Unless action is taken, this outcome will almost certainly result in a proportionate rise in associated costs. Prevalence of hearing loss: WHO global estimates 0 Prevalence of hearing loss 35 dB or greater by age, severity, and cause (1990-2019 findings): Prevalence of hearing loss 35 dB or greater by age and severity (A) and proportion of individuals with hearing loss by age and cause for all severities (B) Loading… 0 Prevalence of Hearing Impairment 3 in 1000 babies born with HL 10x greater for infants with 1 or more risk factors, i.e., 2% to 5%. late-onset and acquired hearing loss is 6x higher than the incidence of HL in the neonatal period 0 Etiology of Childhood Hearing Impairments Unknown (50-55%) Genetic (25 - 30%) Recessive (70%) and dominant traits (25%) X-linked and mitochondrial (5%) 20% Chromosomal aberrations (5%) Teratogenic agents 10% Maternal infections Chemicals/drugs Radiation Environmental/traumatic factors Loud noise, low birth weight, prematurity, severe neonatal jaundice, medication, Injury, etc. 0 Risk Indicators for Delayed- Onset Hearing Loss 0 Risk indicators that are marked with a “§” are of greater concern for delayed-onset hearing loss 1.Caregiver concern§ regarding hearing, speech, language, or developmental delay. 2.Family history§ of permanent childhood hearing loss. 3.Neonatal intensive care of more than 5 days or any of the following regardless of length of stay: assisted ventilation, exposure to ototoxic medications (gentamycin and tobramycin) or loop diuretics (furosemide/Lasix), and hyperbilirubinemia that requires exchange transfusion. 0 4. In utero infections, such as CMV,§ herpes, rubella, §syphilis, and toxoplasmosis. 5. Craniofacial anomalies §, including those that involve the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies, craniofacial anomalies. 0 Loading… 0 6. Physical findings, such as white forelock, that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss. 7. Syndromes associated with hearing loss or progressive or late-onset hearing loss,§ such as neurofibromatosis, osteopetrosis, and Usher syndrome; other frequently identified syndromes including Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson 0 0 8.Neurodegenerative disorders,§ such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich ataxia and Charcot-Marie-Tooth syndrome. 9.Culture-positive postnatal infections associated with sensorineural hearing loss,§ including confirmed bacterial and viral (especially herpes viruses and varicella) meningitis 0 10. Head trauma, especially basal skull/temporal bone fracture§ that requires hospitalization. 11. Chemotherapy (treatment for malignancy/cancers) 0 Prematurity Drs. Greg and Joy Loy Gordon January 2005 Definitions premature: gestational age < 37 weeks moderately premature: 31-36 weeks severely premature: 24-30 weeks newborn: first day of life neonate: first month of life infant: first year of life Prematurity 5-10% of live births High morbidity and mortality due to immature organ systems Responsible for 75% of perinatal or neonatal deaths Immediate/early complications 0 Prematurity : Immediate/early complications hypoxia/ischemia intraventricular hemorrhage sensorineural injury respiratory failure necrotizing enterocolitis cholestatic liver disease nutrient deficiency social stress 0 Early complications and associated sequelae Hypoxia/ischemia mental retardation spastic diplegia microcephaly seizures Early complications and associated sequelae Intraventricular hemorrhage mental retardation spasticity seizures hydrocephalus Early complications and associated sequelae Sensorineural injury hearing impairment visual impairment retinopathy of prematurity strabismus myopia Early complications and associated sequelae Social stress child abuse/neglect failure to thrive Hyperbilirubinemia (severe neonatal jaundice) A condition marked by high levels of bilirubin in the blood. The increased bilirubin causes the infant's skin and whites of the eyes (sclera) to look yellow. 0 Different causes of jaundice Physiological jaundice Breast-feeding jaundice Usually not slow breakdown of bilirubin harmful Prematurity Immature blood, liver and enzymes 0 Increased red blood cell break down Abnormal blood cell shapes Blood type mismatch between the mother and the baby Bleeding underneath the scalp (cephalohematoma) caused by a difficult delivery Higher levels of red blood cells, which is more common in small-for-gestational-age babies and some twins Infection Lack (deficiency) of certain important enzymes 0 Inability to remove bilirubin Certain medications Congenital infections, such as rubella, syphilis, and others Diseases that affect the liver or biliary tract, such as hepatitis Hypoxia Infections (such as sepsis) Many different genetic or inherited disorders 0 Treatment No treatment Phototherapy Exchange-transfusion 0 Possible complications Cerebral palsy Deafness Kernicterus -- a severe complication of jaundice caused Loading… by too much bilirubin in a child's blood. It can lead to permanent brain damage. 0 TORCH Infections T=toxoplasmosis O=other (syphilis) R=rubella C=cytomegalovirus (CMV) H=herpes simplex (HSV) TORCH infections are a group of congenital infections that are passed from mother to child at some time during pregnancy, during delivery, or after birth When to suspect TORCH? Intra-uterine growth restriction (IUGR) (i.e. small for gestational age) Hepato-spleeno megaly (enlarged liver & spleen) Thrombocytopenia (low platelet level) Unusual rash relevant maternal history “Classic” findings of any specific infection Diagnosing TORCH Infection Maternal/prenatal history Remember most infections are mild illnesses often unrecognized Thorough examination of infant Laboratory tests Toxoplasmosis 0 Toxoplasmosis Caused by protozoan – Toxoplasma gondii Domestic cat is the definitive host with infections via: Ingestion of cysts (uncooked meats, garden products) Contact with oocysts in cat feces Much higher prevalence of infection in European countries Acute infection usually asymptomatic 1/3 risk of fetal infection with primary maternal infection in pregnancy Infection rate higher with infection in 3rd trimester Fetal death higher with infection in 1st trimester Clinical Manifestations Most (70-90%) are asymptomatic at birth Classic triad of symptoms: Chorioretinitis Hydrocephalus Intracranial calcifications Clinical Manifestations Other signs include fever, rash, enlarged liver & spleen, microcephaly, seizures, jaundice, thrombocytopenia, enlarged lymph nodes Initially asymptomatic infants are still at high risk of developing abnormalities, such as chorioretinitis and 25% have sensorineural HL Diagnosis Maternal IgG testing indicates past infection Isolation of Toxoplasma in culture from placenta, umbilical cord, infant serum Newborn serologies with IgM/IgA Prevention and Treatment Treatment for pregnant mothers diagnosed with acute toxoplasmosis can reduce the likelihood of congenital transmission by 50% Treatment of symptomatic infants Rubella 0 Rubella Single-stranded RNA virus Vaccine-preventable disease Mild, self-limiting illness Infection earlier in pregnancy has a higher probability of affected infant Clinical Manifestations Sensorineural hearing loss (50-75%) Cataracts and glaucoma (20-50%) Cardiac malformations (20-50%) Neurologic (10-20%) Others to include growth retardation, bone disease, Hepato- Splenomegaly, thrombocytopenia, “blueberry muffin” lesions “Blueberry muffin” spots representing extramedullary hematopoesis Diagnosis Maternal IgG may represent immunization or past infection – not very useful Can isolate virus from nasal secretions Less frequently from throat, blood, urine, CSF Serologic testing IgM = recent postnatal or congenital infection Rising monthly IgG titers suggest congenital infection Diagnosis after 1 year of age difficult to establish Treatment Prevention…immunize, immunize, immunize! Supportive care only with parent education Cytomegalovirus (CMV) 0 Cytomegalovirus (CMV) Most common congenital viral infection ~40,000 infants per year in the U.S. Mild, self-limiting illness Transmission can occur with primary infection or reactivation of virus 40% risk of transmission in primary infection Increased risk of transmission later in pregnancy However, more severe sequalae is associated with earlier infection Clinical Manifestations 90% are asymptomatic at birth Up to 15% develop symptoms later, notably sensorineural hearing loss Symptomatic infection SGA, HSM, petechiae, jaundice, chorioretinitis, periventricular calcifications, neurological deficits >80% develop long term complications Hearing loss, vision impairment, developmental delay Ventriculomegaly and calcifications of congenital CMV Diagnosis Maternal IgG shows only past infection Infection common – not helpful Viral isolation from urine or saliva in 1st 3weeks of life Afterwards may represent post-natal infection Treatment Treatment currently not recommended in asymptomatic infants due to side effects Herpes Simplex (HSV) 0 Herpes Simplex (HSV) HSV1 or HSV2 Primarily transmitted through infected maternal genital tract Rationale for C-section delivery prior to membrane rupture Clinical Manifestations Most are asymptomatic at birth 3 patterns of ~ equal frequency with symptoms between birth and 4wks: Skin, eyes, mouth (SEM) CNS disease Disseminated disease (present earliest) Initial manifestations very nonspecific with skin lesions NOT necessarily present Presentations of congenital HSV Diagnosis Culture of maternal lesions if present at delivery Cultures in infant: Skin lesions, oro/nasopharynx, eyes, urine, blood, rectum/stool, CSF Treatment High dose of antiviral drug Syphilis 0 Syphilis Treponema pallidum (spirochete) Transmitted via sexual contact Placental transmission as early as 6wks gestation Typically occurs during second half of pregnancy Mother with primary or secondary syphilis more likely to transmit than latent disease Congenital Syphilis 2/3 of affected live-born infants are asymptomatic at birth Clinical symptoms split into early or late (2 years is cutoff) Clinical Manifestations Early congenital (typically 1st 5 weeks): Cutaneous lesions (palms/soles) Jaundice Anemia Snuffles or rhinitis Periostitis and metaphyseal dystrophy (inflammation of the periosteum, a layer of connective tissue that surrounds bone) Funisitis (umbilical cord vasculitis) Clinical Manifestations Late congenital: Frontal bossing Short maxilla High palatal arch Hutchinson teeth Sensorineural deafness Saddle nose Can be prevented with appropriate treatment Definition of Congenital Syphilis Confirmed if T. pallidum identified in skin lesions, placenta, umbilical cord, or at autopsy Presumptive diagnosis if any of: Physical exam findings CSF findings (positive VDRL) Osteitis on long bone x-rays Funisitis (“barber shop pole” umbilical cord i.e. inflammation of the connective tissue of the umbilical cord) RPR/VDRL >4 times maternal test Positive IgM antibody Treatment Penicillin G is THE drug of choice for ALL syphilis infections Maternal treatment during pregnancy very effective (overall 98% success) Treat newborn if: They meet diagnostic criteria Mom was treated

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