Article Rating (internal/ external validity; study design) Sample (N with postnatal HL/N with HL; selection period )
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1 Table A1. Studies on children with a hearing loss that included postnatally developed hearing loss (Trend 1 publications) [where 35delG = 35 deleted Guanine; ABR = Auditory Brainstem Response; ASSR = Auditory Steady State Response; BERA = Brain stem electric response audiometry; BOA = Behavioural Observation Audiometry; COR = Conditioned Orientation Response; (c)cmv = (Congenital) Cytomegalovirus; DHS = Distraction Hearing Screening; ECMO = Extracorporeal Membrane Oxygenation; GJB2 = gap junction protein β2; GA = gestational age; HFO = High Frequency Oscillation; LBW = Low Birth Weight; MAS = Meconium Aspiration Syndrome; NICU = Neonatal Intensive Care Unit; OME = Otitis Media with Effusion; PPHN = Persistent Pulmonary Hypertension of the Newborn; PTA = Puretone Audiometry; RDS = Respiratory Distress Syndrome; RFs = Risk Factors; (SN)HL = (Sensorineural) Hearing Loss; (TE)OAEs = (Transient Evoked) Otoacoustic Emissions; tymps = tympanometry; UNHS = Universal Newborn Hearing Screening; VDT = Visitors Distraction Test; VROA = Visual Reinforcement Observation Audiometry]. Article Article Rating (internal/ external validity; study design) Aim of research Sample (N with postnatal HL/N with HL; selection period ) Testing (at birth; at follow-up) RFs present in cases with postnatal HL (N of cases) Hutchin et al. (2000) 8 / Good; Case study To present a case of delayed-onset, progressive SNHL Single case study ABR; audiologic assessment at 2 yrs, 11 mths due to parental concern PPHN (1) Treated with HFO and inhaled nitric oxide Kawashiro et al. (1996) 6 / Good; Case Study To report 10 children who developed HL after discharge from the NICU 10/10 case; 4-year period ABR; ABR, COR if 10 mths to 3 yrs, 3 mths PPHN (8), MAS (5), fetal hydrops (2), pneumonia (1), sepsis (1) > 1 RF in some children Konkle & Knightly (1993) 8 / Good; Case Study To present the pre-, peri-, and postnatal histories for 2 infants who suffered RDS 2/2 ABR; PTA 2 yrs, 5 mths due to parental concern High-frequency jet ventilation (2)
2 Korver et al. (2009) 6 / Fair; Cohort To investigate the contribution of ccmv infection to HL 14 HL with ccmv/165 HL without ccmv OAEs, ABR at 2 wks or DHS at 9 mths; referral due to parental concern Symptomatic CMV (2) Norris et al. (2006) 8 / Good; Case Study To document 9 children with 2 pathogenic GJB2 mutations who had non penetrance of HL at birth 9/9 ABR,OAEs; ABR, ASSR, behavioural testing 2 pathogenic mutations in the GJB2 gene (9) Pagarkar et al. (2006) 9 / Good; Case Study To present siblings with homozygous 35delG mutations, who passed hearing tests in early infancy and developed progressive SNHL 2/2 Sibling 1- VDT; sound field screen, audiometric sweep test, ABR, PTA, OAEs Sibling 2- ABR at 6 months; ABR, DHS Homozygous 35delG mutations (2) Ramsebner et al. (2007) 9 / Good; Cohort To evaluate GJB2 genetic testing within a UNHS program in a European population 3/24 children with nonsyndromic HL; 3- year period OAEs; BERA, age appropriate audiometry, OAEs, tymps Homozygous for 35delG and 35delG/R184P (2) Wild-type GJB2 (1) Thiringer et al. (1984) 8 / Fair; Cohort To analyse the impact of perinatal RFs on the development of HL 8/146; 4-year period Respiratory audiometry 6mths (for children with RFs); BOA >5 mths, VROA > 10 Case1- twin, asphyxia, mechanical ventilation, sepsis, LBW; Case2- twin, hyperbilirubinemia, family
3 in children up to 6- yrs old mths, play audiometry > 2 yrs, 6 mths, and screening at 4 yrs with play audiometry history, LBW; Case3- meningitis; Case4- asphyxia, hypoglycaemia,?cerebral haemorrhage, family history; Case5- LBW, family history; Case6- sepsis, meningitis, family history; Case7- asphyxia, family history; Case8- asphyxia, apnoea, cardiac arrest, mechanical ventilation, hypoglycaemia,?infection, family history Weichbold et al. (2006) 9 / Good; Cohort To determine the percentage of children with postnatal HL and the percentage thereof who have RFs for postnatal HL 23/105; 4-year period TEOAEs; TEOAEs, ABR, PTA RFs in 17/23: family history (3), meningitis (2), craniofacial malformation (2), PPHN (1), CMV (1), ECMO (1), recurrent OME (1), ototoxic therapy (5), GA <33 weeks (2) > 1 RF in 1 child
4 Table 4. Studies that follow a group of children with one risk factor or a series of risk factors (Trend 2 publications) [where ABR = Auditory Brainstem Response; BOA = Behavioural Observation Audiometry; CDH = Congenital Diaphragmatic Hernia; COR = Conditioned Orientation Response; CPA = Conditioned Play Audiometry; (c)cmv = (congenital) Cytomegalovirus; (DP)OAEs = (Distortion Product) Otoacoustic Emissions; ECMO = Extracorporeal Membrane Oxygenation; GA = gestational age; HL = hearing loss; HFO = High Frequency Oscillation; LBW = Low Birth Weight; MAS = Meconium Aspiration Syndrome; PPHN = Persistent Pulmonary Hypertension of the Newborn; PSEP = Preauricular Skin Tags and Ear Pits; PTA = Puretone Audiometry; RDS = Respiratory Distress Syndrome; RFs = Risk Factors; TEOAEs = Transient Evoked Otoacoustic Emissions; tymps = tympanometry; VROA = Visual Reinforcement Observation Audiometry]. Article Article Rating Sample (N; Testing N with postnatal HL (internal/external selection period) (at birth; at follow-up) validity; study design) >1 RF Borradori et al. (1997) 7 / Good; 547; 5-year period ABR (for children with RFs); 4 Case-control ABR at 6, 12, 18, and 24 mths RFs in sample: severe RDS (4),
5 prolonged ventilation (4), LBW of 1400g (1), pneumothoraces (4) Robertson et al. (2009) 10 / Good; Cohort 1279; 30-year period ABR (TEOAEs during mid s); BOA, VROA, CPA 5 RFs in sample: GA 28 weeks (5), LBW <1250g (5), prolonged neonatal oxygen use (5), family history (2), myringotomies (3) CMV Dahle et al. (2000) 8 / Good; Cohort 860 (651 asymptomatic; 209 symptomatic); 24- year period ABR; ABR at 3, 6, 12 mths, VROA at > 9 mths to 2 yrs, 6 mths to 3 yrs (every 6 mths), and annually thereafter 18/651 asymptomatic 23/209 symptomatic Foulon et al. (2008) 10 / Good; Cohort 60 (3 symptomatic; 57 asymptomatic); ABR/DPOAEs; ABR, DPOAEs, PTA at 6 and 12 mths and 3
6 10-year period annually thereafter Fowler et al. (1999) 10 / Fair; Cohort 388 (53 symptomatic; 335 asymptomatic); 16- year period ABR; age appropriate behavioural and speech testing, tymps, and reflexes at 6 and 12 mths, and annually thereafter Cohort with hearing loss increased from 5.2% at birth to 15.4% by 72 mths old Fowler et al. (1997) 10 / Fair; Cohort 307 (asymptomatic ccmv) ABR; age appropriate behavioural and speech testing, tymps, and reflexes at 6, 12 mths, and annually thereafter 4 Hicks et al. (1993) 10 / Good; Cohort 23; 6.5-year period ABR; ABR, behavioural 2 measures Iwasaki et al. (2007) 9 / Good; Cohort 18 (16 asymptomatic; 2 symptomatic); 7.5- ABR; ABR or behavioural measures every 6 to 12 mths until 4 yrs 1/16 asymptomatic
7 year period Johnson et al. (1986) 6 / Good; Cohort 40 Behavioural testing or ABR; 2 VROA, CPA performed annually 3 yrs, ABR if HL suspected Rivera et al. (2002) 10 / Good; Cohort 180 (symptomatic); 31-year period ABR; ABR, behavioural audiometry every 6 to 24 mths, annually thereafter 26 Rosenthal et al. (2009) 10 / Good; Cohort 580 (160 symptomatic; 420 asymptomatic); 17- year period ABR; ABR at 6 and 12 mths, behavioural measures > 9 mths to 2 yrs, 6 mths to 3 years (every 6 mths), and annually thereafter 23/160 symptomatic 15/420 asymptomatic Ross et al. (2006) 9 / Good; Cohort 300 (124 nonprimary infection; Serial audiologic testing occurred at 3 to 8 weeks, 6, 12, 4/124 non-primary infection 10/176 primary infection
8 176 primary infection); 20-year 18, 24, and 36 months of age, then annually thereafter period Williamson et al. (1992) 9 / Good; 59 (asymptomatic); ABR; ABR, behavioural 1 case-control 6-year period audiometry if <3 yrs, behavioural audiometry for children > 3 yrs, tymps CDH Javidnia & Vaccani (2009) 9 / Good; Cohort 17; 8-year period ABR; audiologic testing 5 Masumoto et al. (2007) 8 / Good; Cohort 16; 8-year period ABR; ABR, behavioural audiometry, PTA 4 RFs in sample: ototoxic drugs, neuromuscular blocking agents,
9 HFO, inhaled nitric oxide, duration of hypocapnia, hypoxia, severe acidosis, severe alkalosis, and mechanical ventilation Robertson et al. (1998) 9 / Good; Cohort 37; 6-year period ABR or behavioural audiometry; 8 ABR, VROA, CPA, PTA ECMO Cheung et al. (1996) 9 / Good; Cohort 63; 5-year period ABR; BOA, VROA, CPA at 6, 6 12, 18, 24 mths and 4 yrs Fligor et al. (2005) 10 / Good; Cohort 111; 8-year period ABR; VROA, CPA, PTA at 12, 14 18, 30, and 42 mths Lasky et al. (1998) 7 / Good; Cohort 66 ECMO, 6 survivors of HFO ABR, TEOAE, or DPOAE; tymps, BOA, VROA, CPA, 7/66 ECMO 5/6 HFO
10 therapy PTA. RFs in sample: MAS, CMV, hydrocephalus, PPHN, RDS and intraventricular haemorrhage, Bronchopulmonary dysplasia Mann & Adams (1998) 6 / Good; Cohort 123; 9-year period ABR; otoscopy, ABR, VROA, 10 CPA, PTA, tymps, OAEs Sweitzer et al. (1997) 8 / Good; Cohort 133; 7-year period ABR; BOA, COR, CPA 1 (ECMO secondary to sepsis) PPHN Hendricks-Munoz & Walton (1988) 9 / Good; Cohort 40: 2-year period ABR; ABR, CPA, VROA at 4, 6, 12 mths, 2 to 3 yrs 4 PSEP Roth et al. (2008) 10 / Good; Cross- 637 (587 infants TEOAEs; ABR within 1 mth, 1/11 (high risk group) sectional with PSEP and no assessment up to 3 yrs RFs in sample: Branchio-oto-renal
11 syndrome) other RFs (lowrisk), 39 with PSEP and other RFs (high-risk), 11 with PSEP, including major auricular deformities and/or associated syndromes (very high-risk); 7.5-year period Severe Neonatal Respiratory Failure Robertson et al. (2002) 10 / Good; Cohort 81 (66 non-cdh; 15 CDH); 2-year period ABR; BOA, VROA, CPA, PTA 4 yrs 13
12 Robertson et al. (2006) (Duplicate) 10 / Good; Cohort 81; 2-year period ABR (55/81 infants); BOA, VROA, CPA, PTA 4 yrs 13 RFs in sample: received loop diuretics, aminoglycosides and neuromuscular blockers and 50/81 also received vancomycin Neonatal Toxoplasmosis Brown et al. (2009) 10 / Good; 5 studies Long-term serial audiometric 2/ 5 studies performed serial Systematic literature evaluations audiometry and neither of these review reported any cases of progressive or delayed-onset hearing loss LBW ( 1500g) Salamy et al. (1989) 10 / Good; Cohort 224; 5-year period ABR; ABR at 3 to 6 mths, 9 to 15 mths, 2 to 4 yrs, if HL suspected 3 yrs behavioural 6 RFs in sample: greater amounts of furosemide administration with
13 measures also performed longer duration, administration of aminoglycosides, more episodes of low ph, hypoxemia, and higher bilirubin levels
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