Key points • Autosomal-recessive (AR) nonsyndromic hearing loss is usually prelingual and frequently results in severe hearing loss, although milder and progressive hearing loss forms also exist. GJB2 and SLC26A4…
Key points • Syndromic hearing loss (SHL) is a form of hearing loss (HL) accompanied by additional clinical features in the visual, nervous system, endocrine, and other systems. The most…
Key points • Effect, directly or indirectly, is on neural processing of auditory stimuli. ○ Physiologic measures are needed to accurately characterize patients with auditory neuropathy spectrum disorder (ANSD). ○…
Key points • Lack of binaural input results in difficulties with sound localization and understanding speech in noisy backgrounds. • Children with unilateral hearing loss (UHL) are at risk for…
Key points • Early diagnosis of hearing loss with a combination of electrophysiological and behavioral test measures is critical in initiating treatment during a period of maximal neural plasticity. •…
Key points • Children with conductive hearing loss (CHL) are identified at several possible diagnostic points of care: newborn hearing screening, parental concern for hearing loss, hearing screening in the…
Key points • Management of children with hearing loss requires collaborative, interprofessional teams focused on patient-centered goals and shared problem solving. • Referrals to otolaryngologists, audiologists, and speech-language pathologists with…
Key points • Sensorineural hearing loss (SNHL) occurs in approximately 2 to 4 per 1000 live births. • The cause of pediatric SNHL may be genetic, acquired, or idiopathic. •…
Key points • High-resolution CT of the temporal bone offers excellent visualization of the osseous anatomy of the temporal bone, but has some limitations in the evaluation of soft tissue….
Key points • Hearing loss is the most common congenital sensory impairment, with an incidence of 4/1000 live births. This number rises to approximately 20% after the age of 12 years…
