Bilateral Sensorineural Hearing Loss in Children: Causes and Risk Factors

Genetic Causes

Approximately 77% of nonsyndromic genetic hearing loss cases follow autosomal recessive inheritance, 22% autosomal dominant, and 1% X-linked patterns, according to the American College of Medical Genetics 1
Over 90% of children with autosomal recessive profound hearing loss are born to hearing parents, making family history an unreliable screening tool, as noted by the American College of Medical Genetics 1
Syndromic genetic hearing loss comprises 3-4% of cases, including conditions like Waardenburg syndrome, Usher syndrome, Pendred syndrome, and Jervell and Lange-Nielsen syndrome, as reported by the American Academy of Pediatrics 2

Culture-positive postnatal bacterial and viral meningitis is a well-established risk factor for delayed-onset hearing loss, according to the American Academy of Pediatrics 2
Meningitis (infectious, inflammatory, or neoplastic) causes bilateral sudden hearing loss and is associated with headache, fever, abnormal cerebrospinal fluid, and possible other cranial nerve palsies, as noted by the American Academy of Otolaryngology-Head and Neck Surgery 3, 4
Congenital rubella, syphilis, toxoplasmosis, and herpes are prenatal infections associated with hearing loss, as reported by the American Academy of Pediatrics 2

Prematurity, asphyxia, and complicated perinatal courses account for approximately 10% of bilateral sensorineural hearing loss, with risk factors including NICU stay exceeding 5 days, ECMO, assisted ventilation, hyperbilirubinemia requiring exchange transfusion, and ototoxic medication exposure, as noted by the American Academy of Pediatrics 2

Aminoglycosides and cisplatin can cause bilateral sensorineural hearing loss, often with concurrent vestibular loss and oscillopsia, as reported by the American Academy of Otolaryngology-Head and Neck Surgery 3, 4

Craniofacial anomalies involving the pinna, ear canal, ear tags, ear pits, and temporal bone anomalies are associated with hearing loss, according to the American Academy of Pediatrics 2

Autoimmune inner ear disease presents with fluctuating bilateral hearing loss, often with vertigo, as noted by the American Academy of Otolaryngology-Head and Neck Surgery 3, 4
Cogan syndrome causes nonsyphilitic interstitial keratitis, bilateral hearing loss, and vertigo, as reported by the American Academy of Otolaryngology-Head and Neck Surgery 3, 4

Significant head trauma, basal skull/temporal bone fractures, and barotrauma can cause bilateral hearing loss, according to the American Academy of Otolaryngology-Head and Neck Surgery 3, 4 and the American Academy of Pediatrics 2

MELAS (metabolic encephalopathy, lactic acidosis, and stroke-like episodes) presents with confusion, elevated lactate, MRI white matter changes, and hearing loss, as noted by the American Academy of Otolaryngology-Head and Neck Surgery 3, 4
Other mitochondrial disorders have variable phenotypes and may account for 1-20% of cases depending on population, according to the American College of Medical Genetics 1