OBJECTIVE The advent of technologic improvements in assessing the hearing of newborn infants has made possible the implementation of universal newborn hearing screening. Furthermore, selective screening based on high-risk criteria fails to detect half of all infants with congenital hearing loss. Although universal screening has been recommended by the National Institutes of Health and the Joint...

OBJECTIVE Permanent congenital hearing loss, a common congenital anomaly, may affect speech and language acquisition, academic achievement and social development. Current standards emphasize early recognition of congenital hearing loss. This study was conducted to find the prevalence of hearing impairment in term newborns in Yazd, Iran. METHODS This was a descriptive-analytic study conducted ...

Of 111 children born in 1978-82 with congenital rubella infection, confirmed by the detection of rubella specific IgM shortly after birth, 68 were reported to be hearing impaired when notified to the National Congenital Rubella Surveillance Programme (NCRSP). The average age at which the diagnosis of hearing impairment was confirmed was 11.6 months. Recent audiograms showed a hearing loss that ...

Hearing loss due to mutations in the connexin gene family, which encodes gap junctional proteins, is a common form of hereditary deafness. In particular, connexin 26 (Cx26, GJB2) mutations are responsible for ~50% of non-syndromic hearing loss, which is the highest incidence of genetic disease. In the clinic, Cx26 mutations cause various auditory phenotypes ranging from profound congenital deaf...

The recent article on ‘‘Hearing loss in congenital Zika virus’’ is very interesting.1 Leal et al. reported an interesting case of sensorineural hearing loss in a case of congenital Zika virus.1 In fact, it is no doubt that Zika virus can induce teratogenic effect and the main pathology is on the fetal neurological system. However, the myth is the exact mechanism underlying the hearing loss in t...

Toxoplasmosis is a systemic infection caused by the protozoan parasite Toxoplasma gondii, which is transmitted through the ingestionofcontaminated food. Less frequently, toxoplasmosis is acquired by blood transfusion or an organ transplant. Congenital toxoplasmosis, which is caused by vertical transmission from the mother to the fetus, has an estimated prevalence of 20% of infected mothers tran...

Duplication of the internal auditory canal is an uncommon, congenital malformation that can be associated with sensorineural hearing loss owing to aplasia/hypoplasia of the vestibulocochlear nerve. Only 14 such cases have been reported to date. We report the case of a 13-month-old girl with bilateral, congenital, sensorineural hearing loss caused by narrow, duplicated internal auditory canals a...

Universal newborn hearing screening (UNHS) was begun in the United States in several geographic locations, the states of Rhode Island, Hawaii, and Colorado in the early 1990s. Since that time, evidence-based research indicates that universal newborn hearing screening results in earlier identification of congenital hearing loss leading to the provision of earlier intervention. Outcome data of ch...

X-linked mixed hearing loss with stapes fixation is a congenital, recessive anomaly. The condition is characterized by progressive mixed hearing loss, reduced vestibular responses, and congenital fixation of the stapes with perilymph gusher encountered during surgical intervention. This paper describes the audiologic history of a family with two affected male children and one carrier female chi...

As many as 40% of patients with congenital sensorineural hearing loss will have a structural inner ear malformation identified on a temporal bone imaging study (1). Anomalies of the internal auditory canal (IAC) are rare and have been reported to account for only 12% of all congenital temporal bone abnormalities. Internal auditory canal abnormalities may be associated with hypoplasia or aplasia...