Congenital cytomegalovirus (CMV) infection is an important public health problem with approximately 7 in 1,000 newborns infected and consequently at risk for hearing impairment. Newborn hearing screening will fail to detect this hearing impairment in approximately half of the cases because late onset hearing loss is frequent. Hearing impairment has profound impact on cognitive and social develo...

The advent of hearing screening in newborns in many states has led to an increase in the use of genetic testing and related genetic services in the follow-up of infants with hearing loss. A significant proportion of those with congenital hearing loss have genetic etiologies underlying their hearing loss. To ensure that those identified with congenital hearing loss receive the genetic services a...

Hearing loss is one of the common congenital problems among neonates [1]. The prevalence of significant hearing loss ranges from 1.2 per 1,000 healthy newborn infants and 2 to 5% in high-risk newborns [2,3]. Nearly 50% of congenital hearing loss is due to genetic defects [1]. About 50% of hearing defects can be detected in a selective screening based exclusively on hearing risk criteria [1]. Ea...

BACKGROUND AND OBJECTIVES Congenital cytomegalovirus (CMV) is the leading cause of nongenetic deafness in children in the United States and can cause neurodevelopmental impairment in term infants. Limited data exist regarding congenital CMV infections in preterm infants. We aimed to determine the incidence and association with outcomes of congenital CMV in very low birth weight (VLBW) preterm i...

Objective. Bilateral congenital cholesteatoma is a very rare condition. We describe the first case of an adult patient presenting with bilateral congenital cholesteatoma with labyrinthine fistula. Case report. We present a 34-year-old woman of bilateral congenital cholesteatoma with labyrinthine fistula, inducing leftsided mixed conductive-sensorineural hearing loss and vertigo, subsequently ac...

Molecular diagnostic testing of individuals with congenital sensorineural hearing loss typically begins with DNA sequencing of the GJB2 gene. If the cause of the hearing loss is not identified in GJB2, additional testing can be ordered. However, the step-wise analysis of several genes often results in a protracted diagnostic process. The more comprehensive Hereditary Hearing Loss Arrayed Primer...

1 Recent advances in basic science, technology and treatments have increased our abilities to manage hearing and balance diseases more effectively. Advances in molecular genetics have allowed better understanding of congenital and hereditary hearing loss. The advent of regenerative medicine led to auditory hair cell regeneration in laboratory animals. Advances in hearing aids, cochlear implants...

A case report is presented of a 62-year-old software product manager who had normal hearing in one ear and a congenital profound hearing loss in the other ear and then sustained a sudden sensorineural hearing loss in the only hearing ear. The approach to amplification decisions, cochlear implant evaluation, and rehabilitation options are discussed. Providing aural rehabilitation and continually...

Introduction: Cochlear implantation (CI) improves language skills among children with hearing loss. However, children with CIs still fall short of fulfilling some other needs, including musical perception. This is often attributed to the biological, technological, and acoustic limitations of CIs. Emotions play a key role in the understanding and enjoyment of music. The present study aimed to in...