This paper presents the first case of human genetic hearing loss due to predominant inner hair cell damage investigated by psychoacoustic audiometry, auditory brainstem responses (ABR), evoked acoustic emissions (EAEs), and distortion product otoacoustic emissions (DPOEs). The genetic transmission was an autosomal dominant inheritance. The audiogram pattern was a dominant high-frequency hearing loss. EAE and DPOE properties were normal. Bronx waltzer mutant mouse with genetically determined predominant inner hair cell defects provide an interesting model to study this congenital deafness. This rare congenital deafness with predominant damage to inner hair cells cannot be detected by neonatal screening techniques using EAEs or DPOEs.