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The indications for cochlear implantation have gradually expanded as advancements in technology have evolved, resulting in improved audiologic outcomes for both adult and children. There remains a significant underutilization of cochlear implant technology in the United States, and recognition of the potential benefits of cochlear implantation for non-traditional indications is critical for encouraging the evolution of candidacy criteria. Adult cochlear implantation candidacy has progressed from patients with bilateral profound sensorineural hearing loss (SNHL) to include patients with greater degrees of residual hearing, single-sided deafness and asymmetric hearing, and atypical etiologies of hearing loss (eg, vestibular schwannoma, Ménière's disease, and otosclerosis). Indications for pediatric cochlear implantation have similarly evolved from children with bilateral severe to profound SNHL to implanting children at a younger age, including those with residual hearing, asymmetric hearing loss, inner ear malformations, as well as cochlear nerve deficiency. In this editorial, the literature investigating cochlear implantation for nontraditional indications is reviewed with an aim to use the best available evidence to encourage the evolution of candidacy criteria. [ABSTRACT FROM AUTHOR]

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P <.001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted. [ABSTRACT FROM AUTHOR]

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation. [ABSTRACT FROM AUTHOR]