A 5-year-old child with bilateral profound sensorineural hearing loss is evaluated for cochlear implantation. Imaging reveals bilateral cochlear aplasia (Michel deformity). The most appropriate management is:
- A Proceed with cochlear implantation bilaterally
- B Conventional hearing aids
- C Auditory brainstem implant (ABI) ✓
- D Bone-anchored hearing aid (BAHA)
Correct answer: C. Auditory brainstem implant (ABI)
Explanation
Michel deformity is complete absence of the cochlea and vestibule (total cochlear aplasia), making cochlear implantation impossible as there is no cochlea to insert the electrode array into. The auditory brainstem implant (ABI) stimulates the cochlear nucleus directly in the brainstem and is the only option for patients with absent or non-functional cochleae. BAHA is suitable for conductive or mixed hearing loss, not profound SNHL with cochlear aplasia.
Reference: Dhingra Diseases of Ear, Nose and Throat, 7th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.