What is the management of sudden sensorineural hearing loss in a child?

Management of Sudden Sensorineural Hearing Loss in Children

Critical Initial Recognition

While established guidelines focus on adults aged 18 and older, pediatric sudden sensorineural hearing loss (SSNHL) requires urgent evaluation and treatment using adapted adult protocols, as prompt corticosteroid intervention can prevent permanent hearing loss in children. 1

The definition remains consistent: ≥30 dB hearing loss at 3 consecutive frequencies occurring within 72 hours, though pediatric cases are rare and often underrecognized. 1, 2

Immediate Diagnostic Steps

Distinguish Hearing Loss Type

• Perform tuning fork testing immediately (Weber and Rinne tests) to differentiate sensorineural from conductive hearing loss, as this determines the entire management pathway. 1
• Physical examination must identify obstructing cerumen, foreign bodies, tympanic membrane perforation, or middle ear fluid that would indicate conductive rather than sensorineural pathology. 1

Obtain Urgent Audiometry

• Formal audiometric testing should occur within days to confirm SSNHL diagnosis and establish baseline thresholds for monitoring treatment response. 1
• Calculate 4-frequency pure-tone average (500,1000,2000,4000 Hz) for each ear to quantify severity. 3

Focused History Elements

• Document exact timing of onset (must be ≤72 hours), presence of tinnitus (reported in majority of pediatric cases), vertigo, sensation of blocked ear, or otalgia. 1, 2, 4
• Specifically ask about recent viral illness, mumps exposure, head trauma, ototoxic medication exposure, and family history of enlarged vestibular aqueduct (EVA). 2, 5, 4
• Inquire about underlying conditions including sickle cell disease, which can cause bilateral profound SSNHL through vaso-occlusive mechanisms. 6

Essential Diagnostic Workup

Laboratory Testing

• Obtain serologic testing for Epstein-Barr virus (EBV) and cytomegalovirus (CMV), as viral infection is the most common identifiable etiology in pediatric SSNHL. 2, 4
• Consider Lyme titers in endemic areas, though routine laboratory panels are not recommended per adult guidelines. 1, 4

Imaging Studies

• Order temporal bone CT and/or MRI to identify structural abnormalities, particularly enlarged vestibular aqueduct syndrome (LVAS), which is the most common imaging-based cause of pediatric SSNHL. 5, 4
• LVAS children have mean vestibular aqueduct diameter >6.5 mm and are at high risk for sudden hearing deterioration with minor head trauma. 5
• MRI may identify late presentation of congenital CMV with characteristic temporal bone findings. 4
• Do not delay treatment while awaiting imaging, as these studies rarely change acute management but are essential for identifying underlying structural causes. 1, 4

Primary Treatment Protocol

Systemic Corticosteroids

• Initiate oral prednisolone 1-2 mg/kg/day (or equivalent dexamethasone) immediately upon diagnosis, as this is the cornerstone of treatment adapted from adult guidelines. 1, 5
• Treatment should begin as soon as possible, ideally within 2 weeks of onset, though earlier intervention correlates with better outcomes. 1, 5
• For children with LVAS, aggressive corticosteroid intervention at first sign of hearing deterioration achieves 85% response rate (defined as >10 dB improvement at ≥2 consecutive frequencies). 5

Intratympanic Steroid Therapy

• Consider intratympanic steroid perfusion for incomplete recovery after initial systemic treatment or as primary therapy in children who cannot tolerate systemic steroids. 1, 2
• This salvage therapy showed benefit in 42% of pediatric cases in one series, though evidence is limited. 2

What NOT to Do

• Do not routinely prescribe antivirals, antibiotics, thrombolytics, vasodilators, or vasoactive substances, as these lack evidence of efficacy per adult guidelines. 1
• Do not obtain routine head CT in initial evaluation unless focal neurological findings, trauma, or concern for intracranial pathology exists. 1

Special Considerations by Etiology

Enlarged Vestibular Aqueduct Syndrome

• Early detection and immediate corticosteroid treatment at each episode of sudden deterioration is crucial to preserve residual hearing. 5
• Counsel families about avoiding head trauma and activities with high risk of minor head injury. 5
• Mean age at LVAS diagnosis is 2.3 years, with bilateral involvement in all cases. 5

Sickle Cell Disease

• Consider both vaso-occlusive and inflammatory etiologies (such as Cogan's syndrome) and treat empirically for both if bilateral profound loss occurs. 6
• Check inflammatory markers and ophthalmologic examination for associated disease. 6

Monitoring and Follow-Up

Audiometric Reassessment

• Obtain follow-up audiometry at regular intervals to document treatment response and determine need for escalation. 1
• Complete recovery (hearing level matching unaffected ear) occurs in only 16-21% of pediatric cases. 2, 3
• Partial improvement occurs in 47% of cases, while 32% show no improvement despite treatment. 2

Rehabilitation Planning

• For children with residual hearing loss, counsel families about amplification, hearing-assistive technology, and audiologic rehabilitation as strong recommendation from adult guidelines. 1
• Early referral for cochlear implantation evaluation is essential if bilateral profound loss persists without recovery, ideally within 12 weeks. 6

Critical Pitfalls to Avoid

• Never dismiss sudden hearing loss in children as "ear infection" without formal audiometry, as delayed diagnosis compromises treatment efficacy. 1, 2
• Do not assume unilateral presentation, as 45% of pediatric SSNHL cases are bilateral. 4
• Recognize that most imaging studies (20/22 in one series) are non-diagnostic, but the 2 positive studies provided critical etiologic information (EVA, congenital CMV). 4
• Do not overlook non-organic hearing loss in the differential diagnosis, particularly in older children and adolescents. 4
• For children with known LVAS, educate families that any sudden hearing change requires immediate medical attention, as timing of corticosteroid intervention directly impacts recovery. 5

Patient and Family Education

• Explain that viral infection is the most common identifiable cause (63% in pediatric series), though most cases remain idiopathic. 2, 4
• Discuss realistic expectations: only 16-21% achieve complete recovery, but 63% show some degree of improvement with treatment. 2, 3
• Emphasize urgency of seeking care for any future episodes, particularly in children with structural abnormalities like LVAS. 5