Most of the patients who present with congenital sensorineural hearing loss (SNHL) do not demonstrate an imaging abnormality. Only 39% of patients with congenital SNHL have a documented imaging abnormality, with large vestibular aqueduct syndrome being the most common of the congenital anomalies to be identified on imaging. Congenital anomalies of the semicircular canals can result in SNHL. Dys...

BACKGROUND Congenital cytomegalovirus (CMV) infection is a cause of sensorineural hearing loss (SNHL) in children, but the magnitude of its contribution is uncertain. Quantifying the impact of congenital CMV infection requires an evidence-based assessment using a standard case definition of hearing loss. OBJECTIVES To determine the frequency of bilateral moderate to profound SNHL in children ...

OBJECTIVES To examine the incidence of congenital cytomegalovirus (CMV) infection relative to common genetic etiologies of hearing loss in a pediatric population with sensorineural hearing loss (SNHL), and to characterize intracranial radiological abnormalities in patients with CMV-associated hearing loss. DESIGN Retrospective study. SETTING Academic tertiary care center. PATIENTS A total...

BACKGROUND Cytomegalovirus (CMV) infection is the most frequent congenital infection in humans. Its prevalence and the frequency of disabling sequelae must be assessed in different populations to permit the formulation or assessment of preventive measures. OBJECTIVES To check the prevalence of congenital infection and seroprevalence in Italy; to verify the rate of sensorineural hearing loss (...

Background: Congenital hypothyroidism increases the risk of sensorineural hearing loss (SNHL). Children with hearing impairment are prone to communication disorders. The present study aimed to determine the prevalence of SNHL in patients with congenital hypothyroidism in Qazvin, Iran. Methods: This cross-sectional study was conducted on children with transient or permanent congenital hypothyroi...

OBJECTIVES To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) in children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected children. METHODS We included 92 case-patients and 51 controls assessed by using auditory brainstem response and behavioral audiometr...

BACKGROUND AND PURPOSE High-resolution T2-weighted fast spin-echo MR imaging provides excellent depiction of the cisternal and intracanalicular segments of the vestibulocochlear and facial nerves. Absence or reduction in caliber of the cochlear nerve (deficiency) has been described in association with congenital sensorineural hearing loss (SNHL). Depiction of cochlear nerve integrity may be imp...

CONCLUSIONS Congenital cytomegalovirus (CMV) infection is a major cause of bilateral and unilateral sensorineural hearing loss (SNHL) in children, accounting for 9.0% of SNHL cases. The diagnostic rate using combined genetic deafness test and CMV DNA detection test was determined to be 46.4% in bilateral profound SNHL. OBJECTIVES The present study investigated the prevalence of congenital CMV...

The association between congenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described almost 50 years ago. Studies over the intervening decades have further described the relationship between congenital CMV infection and SNHL in children. However, congenital CMV infection remains a leading cause of SNHL in children in the United States and the world today....

This investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infect...