Congenital malformation of inner ear

This code signifies a birth defect affecting the innermost part of the ear, responsible for both hearing and balance. These malformations can range from subtle structural anomalies to complete absence of inner ear components, leading to varying degrees of sensorineural hearing loss and/or vestibular dysfunction.

Apply this code when documentation specifies a congenital structural abnormality of the cochlea, vestibule, semicircular canals, or other inner ear structures identified at birth or early childhood. Supporting documentation includes imaging reports (e.g., CT, MRI) detailing the malformation, audiology reports confirming sensorineural hearing loss, and physician notes describing the congenital nature of the defect.

• Differentiate from acquired inner ear conditions; this code is strictly for congenital anomalies.
• Specify laterality (left, right, bilateral) if documented, using appropriate subcodes.
• Code any associated hearing loss (H90.-) or vestibular dysfunction (H81.-) separately, as these are manifestations, not the primary malformation itself.