Table 1 Diagnostic criteria for non-syndromic ANSD in this study

Audiological findings

• Presence of otoacoustic emissions and/or cochlear microphonics

• Absent or abnormal auditory brainstem responses

Medical history

• No evidence of acquired risk factors, such as prematurity, kernicterus, or perinatal insults

• No evidence of syndromic ANSD, such as autosomal dominant optic atrophy, Charcot-Marie-Tooth syndrome, or Friedreich’s ataxia

Imaging studies

• No evidence of inner ear malformations or cochlear nerve aplasia/hypoplasia