Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection

Fowler, Karen B.; McCollister, Faye P.; Dahle, Arthur J.; Boppana, Suresh B.; Britt, William J.; Pass, Robert F.

doi:10.1016/s0022-3476(97)70248-8

Cited by 481 publications

(374 citation statements)

References 30 publications

Supporting

Mentioning

341

Contrasting

Unclassified

Order By: Relevance

“…They could also have had undetected cytomegalovirus infection, a leading cause of deafness. 13 More recently, Valaes et al reported a significantly higher incidence of SNHL (9.2%) in infants with moderate or marked neonatal jaundice (hemolytic and nonhemolytic) than in the children with slight or no jaundice (1.1%).…”

Section: Discussionmentioning

confidence: 96%

Lack of Deafness in Crigler–Najjar Syndrome Type 1: A Patient Survey

Suresh

Lucey²

1997

Pediatrics

View full text Add to dashboard Cite

ABSTRACT. We performed a questionnaire survey about 42 patients with Crigler-Najjar syndrome type 1 who were currently alive. Information was obtained on their age, sex, birth weight, gestation, parental consanguinity, other family members affected, age of onset of jaundice, neonatal and postneonatal bilirubin values, neonatal and postneonatal therapy, problems faced with phototherapy, liver transplantation, current growth status, current neurologic status, and the status of hearing. Patients were between 2 months and 21 years of age. There were 18 males and 24 females. Thirty-nine patients had been born at full term gestation and 3 had been preterm. Jaundice was noted on postnatal day 1 in 34%, between days 2 and 4 in 55%, and after day 11 of life in 11% of patients. In the neonatal period bilirubin values (mean ؎ SD) were typically 19.8 ؎ 4.5 mg/dL. Eighty-six percent of patients had neonatal peak bilirubin values of >20 mg/dL. Parental consanguinity was present in 44% and a history of Gilbert's disease in one parent was present in 10% of patients. Causes of exacerbations of jaundice reported were respiratory infections, febrile illnesses, vaccinations, fasting, surgery, emotional stress, and noncompliance with treatment. Neonatal therapy consisted of exchange transfusion in 28%, phototherapy in 79%, phenobarbitone in 20%, and cholestyramine, albumin, infusions, and plasmapheresis in one case each. The mainstay of postneonatal therapy was home phototherapy for 10 to 16 hours, primarily at night during sleep, using blue lights or a combination of blue and fluorescent lights. Some patients used innovatively designed phototherapy units. Problems reported with phototherapy were decreased effectiveness with age, poor compliance, restriction of activity and play, inability to travel or take vacations, irritation from eye shades, difficulty keeping eye protection on, difficulties in temperature maintenance, tanning of the skin, embarrassment from the need to be nearly nude during phototherapy, and difficulty in procuring phototherapy lamps. Other therapies that had been tried included oral agar, albumin infusions, antioxidants, acupuncture, bilirubin oxidase, calcium infusions, clofibrate, cruciferous vegetables, cholestyramine, chlorpromazine, flumecinol, plasmapheresis, tin mesoporphyrin, ursodeoxycholic acid, and urinary alkalinization. Fifteen children had undergone liver transplantation (5 auxiliary and 10 orthotopic). All 42 patients are reportedly of normal height and weight. Neurodevelopmental status is said to be normal in 77% of patients. Two patients have kernicterus, 4 have cerebellar symptoms, and 1 each has developmental delay, mild intention tremor, and mild speech delay. Hearing was reported to be normal in 94% of patients. The 2 children with hearing loss have conductive loss from otitis media. With home phototherapy prolonged, survival free of neurologic deficits is possible

show abstract

Section: Discussionmentioning

confidence: 96%

Lack of Deafness in Crigler–Najjar Syndrome Type 1: A Patient Survey

Suresh

Lucey²

1997

Pediatrics

View full text Add to dashboard Cite

show abstract

“…90,91 Some authors attribute hearing loss to the cytopathic effect of the virus itself and the host immune response on inner ear structures. [92][93][94][95][96] Regarding a possible delayed onset of hearing loss, percentages in the literature range from 0% to 50%. 3,24,27,66,67 We calculated ∼18% in the symptomatic group and ∼9% in the asymptomatic group, but in both groups there was significant heterogeneity between studies.…”

Section: Discussionmentioning

confidence: 99%

Hearing Loss and Congenital CMV Infection: A Systematic Review

Goderis¹,

Leenheer²,

et al. 2014

View full text Add to dashboard Cite

BACKGROUND AND OBJECTIVE: Hearing loss caused by congenital cytomegalovirus (cCMV) infection was first observed in 1964. Today cCMV is the most common cause of nonhereditary sensorineural hearing loss in childhood. Our objective was to provide an overview of the prevalence of cCMV-related hearing loss, to better define the nature of cCMV-associated hearing loss, and to investigate the importance of cCMV infection in hearing-impaired children. METHODS:Two reviewers independently used Medline and manual searches of references from eligible studies and review articles to select cohort studies on children with cCMV infection with audiological follow-up and extracted data on population characteristics and hearing outcomes. RESULTS:Thirty-seven studies were included: 10 population-based natural history studies, 14 longitudinal cohort studies, and 13 retrospective studies. The prevalence of cCMV in developed countries is 0.58% (95% confidence interval, 0.41-0.79). Among these newborns 12.6% (95% confidence interval, 10.2-16.5) will experience hearing loss: 1 out of 3 symptomatic children and 1 out of 10 asymptomatic children. Among symptomatic children, the majority have bilateral loss; among asymptomatic children, unilateral loss predominates. In both groups the hearing loss is mainly severe to profound. Hearing loss can have a delayed onset, and it is unstable, with fluctuations and progression. Among hearing-impaired children, cCMV is the causative agent in 10% to 20%. Despite strict selection criteria, some heterogeneity was found between selected studies.CONCLUSIONS: This systematic review underscores the importance of cCMV as a cause of sensorineural hearing loss in childhood. Pediatrics 2014;134:972-982

show abstract

“…11 Other studies showed unilateral SNHL in 33% to 52% of the cases, with all degrees of severity of hearing loss. 12 The high prevalence of SNHL in children congenitally infected by CMV suggests a viral tropism for the inner ear. Autopsied pediatric CMV cases confirmed that cytomegalic cells were present in the inner ear; lesions were mainly observed in the endolymphatic compartment of the inner ear, particularly in the vestibular structures.…”

Section: What This Study Addsmentioning

confidence: 99%

“…10 Some published studies have found unilateral deafness, of all degrees of severity, in 33% to 52% of cases. 12,35 Progressive deterioration of hearing was observed in 11% to 50% of cases, hearing fluctuations in 16% to 23%, and late-onset deafness in 5% to 50%. 10,12,35 In our study, hearing loss was diagnosed at a mean age of 15.4 months (SD 20.4 months) (1 child was 7 years old).…”

Section: Audiologic Characteristicsmentioning

confidence: 99%

“…12,35 Progressive deterioration of hearing was observed in 11% to 50% of cases, hearing fluctuations in 16% to 23%, and late-onset deafness in 5% to 50%. 10,12,35 In our study, hearing loss was diagnosed at a mean age of 15.4 months (SD 20.4 months) (1 child was 7 years old). This is earlier than the mean age of 22 months reported by Fowler in children symptomatic at birth.…”

Section: Audiologic Characteristicsmentioning

confidence: 99%

See 1 more Smart Citation

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

Bernard¹,

Wiener-Vacher

Abbeele

et al. 2015

Pediatrics

View full text Add to dashboard Cite

BACKGROUND: Congenital cytomegalovirus (CMV) infection is the leading infectious cause of neurologic disabilities and sensorineural hearing loss in children. Sensorineural hearing loss prevalence in CMV suggests a viral tropism for the inner ear. Vestibular disorders induced by CMV infection are underestimated. This is the largest and most thorough study to assess the incidence of vestibular disorders and their correlation with hearing thresholds in children with CMV.

show abstract

Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection

Cited by 481 publications

References 30 publications

Lack of Deafness in Crigler–Najjar Syndrome Type 1: A Patient Survey

Lack of Deafness in Crigler–Najjar Syndrome Type 1: A Patient Survey

Hearing Loss and Congenital CMV Infection: A Systematic Review

Vestibular Disorders in Children With Congenital Cytomegalovirus Infection

Contact Info

Product

Resources

About