From the Guidelines
Bilateral sensorineural hearing loss in pediatric patients requires prompt evaluation and management, with a comprehensive audiological assessment, including auditory brainstem response testing and otoacoustic emissions, to determine the degree and configuration of hearing loss. The initial approach should include identifying risk indicators associated with permanent congenital, delayed-onset, and/or progressive hearing loss in childhood, such as caregiver concern regarding hearing, speech, language, or developmental delay, family history of permanent childhood hearing loss, and neonatal intensive care of more than 5 days or exposure to ototoxic medications 1. For mild to moderate hearing loss, hearing aids are typically the first-line intervention, while severe to profound hearing loss may warrant cochlear implantation, ideally before 12 months of age to maximize language development 1.
- Key considerations in the management of pediatric sensorineural hearing loss include:
- Genetic testing, as approximately 50-60% of pediatric hearing loss has genetic causes, with mutations in the GJB2 gene (encoding Connexin 26) being most common
- Imaging (MRI or CT) to evaluate inner ear structures
- CMV testing in infants
- Screening for associated syndromes, such as Usher syndrome, Waardenburg syndrome, and Pendred syndrome
- Early intervention services focusing on speech-language therapy and educational support are crucial, as timely auditory stimulation during critical developmental periods significantly impacts language acquisition and cognitive development 1.
- Family counseling and connection to support resources are also essential components of comprehensive care, with regular follow-up with a multidisciplinary team including otolaryngology, audiology, speech pathology, and genetics necessary to monitor progression and adjust interventions as needed 1.
From the Research
McCune-Albright Syndrome and Hearing Loss
- McCune-Albright syndrome (MAS) is a rare disorder characterized by endocrine disorders, café-au-lait spots, and fibrous dysplasia of bone that occurs early in life 2.
- The syndrome can involve several tissues, including bone, skin, and endocrine systems, but there is no direct mention of hearing loss as a common symptom of MAS 2, 3, 4.
- However, fibrous dysplasia (FD) is a common manifestation of MAS, and it can affect the skull, which may lead to neurosensory risks, including hearing loss 2, 3, 4.
Binaural Hearing and Cochlear Implants
- Binaural hearing refers to the ability to hear sounds with both ears, and it is important for sound localization and speech perception in noise 5.
- Cochlear implants can be used to restore hearing in individuals with severe to profound sensorineural hearing loss, including those with unilateral hearing loss 5, 6.
- The use of cochlear implants can improve binaural auditory outcomes, including speech perception in noise and sound localization, in individuals with acquired unilateral profound hearing loss 5.
Pediatric Hearing Loss
- Sensorineural hearing loss is the most common type of hearing impairment worldwide, and it can affect individuals of all ages, including children 6.
- The prevalence of hearing loss increases dramatically with age, but it can also be present at birth or develop during childhood due to various causes, including genetic mutations and environmental insults 6.
- There is no direct evidence in the provided studies to suggest a link between McCune-Albright syndrome and binaural sensirenol hearing loss in pediatric patients.