Hearing loss in inherited metabolic disorders: A systematic review

Inherited metabolic disorders (IMDs) have been observed in individuals with hearing loss (HL), but IMDs are rarely the cause of syndromic HL. With early diagnosis, management HL is more effective and cortical reorganization possible aids or cochlear implants. This review describes relationships between terms incidence, etiology HL, pathophysiology, treatment. Forty types described literature, mainly case reports. Management prognosis noted where existing. We also describe given age occurrence Reviewing main that associated may provide an additional clinical tool which to better diagnose