What is the recommended management for unilateral sensorineural hearing loss in children?

Management of Unilateral Sensorineural Hearing Loss in Children

All children with unilateral sensorineural hearing loss should be assessed for hearing aid appropriateness and enrolled in early intervention services, regardless of the degree of hearing loss. 1

Immediate Actions After Diagnosis

Audiological Assessment and Amplification

• Assess every child with unilateral sensorineural hearing loss for hearing aid fitting, with the decision to amplify based on the degree of residual hearing in the affected ear 1, 2
• Do not use contralateral routing of signals (CROS) amplification in children with unilateral hearing loss—this is specifically contraindicated by the American Academy of Pediatrics 1, 2
• If amplification is elected, fit the hearing aid within 1 month of diagnosis 1
• For profound unilateral loss with minimal residual hearing, a hearing aid may not be indicated, but the child still requires intervention services 1

Medical Evaluation

• Refer to an otolaryngologist with pediatric hearing loss expertise for comprehensive diagnostic assessment 1
• Obtain ophthalmologic evaluation by an experienced pediatric ophthalmologist, as many causes of hearing loss have associated visual abnormalities 1
• Offer genetics consultation to all families, as over 90% of children with permanent hearing loss are born to hearing parents 1
• Consider temporal bone imaging (CT or MRI) to identify structural defects, particularly to evaluate for cochlear nerve aplasia, which is the most common cause of unilateral neural hearing loss in children (73% of cases) 1, 3

Early Intervention Services

Enrollment and Service Delivery

• Enroll all families of children with unilateral hearing loss in early intervention services—they are eligible regardless of whether hearing loss is the primary disability 1
• Initiate early intervention services by 6 months of age at the latest 1
• Services should be provided by professionals with specific expertise in hearing loss, including educators of the deaf, speech-language pathologists, and audiologists 1
• Offer both home-based and center-based intervention options to families 1

Individualized Service Plans

• Some families may choose only developmental assessment or occasional consultation for unilateral losses 1
• The intensity and type of services should be guided by family goals, the child's developmental needs, and the degree of functional impact 1

Surgical Options for Specific Etiologies

Bone-Anchored Hearing Aids

• For children with congenital aural atresia or malformations of the outer and middle ear causing permanent conductive or mixed hearing loss, consider bone-anchored hearing aids when the child reaches appropriate age for skull bone maturity 1, 2
• The American Academy of Otolaryngology-Head and Neck Surgery supports osseointegrated bone conductive devices for unilateral hearing loss rehabilitation, particularly when conventional amplification cannot be used 2

Cochlear Implantation

• For severe-to-profound unilateral sensorineural hearing loss with normal contralateral hearing, cochlear implantation can be considered when the interaural difference is significant (>15 dB) 4
• Cochlear implantation improves sound localization and hearing in noisy environments, which are major functional deficits with single-sided deafness 4
• Confirm intact cochlear nerves via MRI or CT before considering cochlear implantation 5

Ongoing Surveillance and Monitoring

Regular Developmental Assessment

• Perform regular surveillance of developmental milestones, auditory skills, parental concerns, and middle-ear status at all well-child visits 1
• Conduct objective standardized screening of global development at 9,18, and 24-30 months of age 1
• Refer for speech-language evaluation if the child fails the speech-language portion of screening or if there are any concerns 1

Otitis Media Management

• Promptly treat persistent otitis media with effusion, as it compounds the hearing loss and negatively affects hearing aid performance 1
• Refer to primary care physician or otolaryngologist for definitive resolution of middle ear fluid 1
• Never delay hearing aid fitting while treating otitis media with effusion 1

Critical Pitfalls to Avoid

• Never assume unilateral hearing loss is benign—mounting evidence shows these children experience educational difficulties, poor speech understanding in noise, impaired sound localization, and behavioral problems 6, 7
• Do not use CROS amplification systems in children 1, 2
• Do not delay intervention while pursuing etiological diagnosis—begin services while diagnostic workup proceeds 1
• Do not overlook the need for MRI in unilateral neural hearing loss patterns (present otoacoustic emissions with hearing loss), as this identifies cochlear nerve aplasia or unsuspected tumors 3