NF2 – Hearing Rehabilitation Options — SCE Neurology MCQ

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ModerateNeuro-oncologyNF2 – Hearing Rehabilitation OptionsSCE Neurology

A 45-year-old woman with known NF2 (bilateral vestibular schwannomas) asks about hearing rehabilitation options. Her hearing has deteriorated bilaterally. What hearing rehabilitation options are available for NF2?

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Correct answer: E — Hearing aids (for residual hearing), cochlear implants (if cochlear nerve is preserved), or auditory brainstem implants (ABI — for patients with bilateral vestibular schwannoma removal where cochlear nerves are non-functional)

Explanation lettering: E = shown as A · C = shown as B · D = shown as C · A = shown as D · B = shown as E

Hearing rehabilitation in NF2 follows a hierarchy: (1) hearing aids — for patients with residual hearing, (2) cochlear implants — if the cochlear nerve is preserved (requires intact nerve for electrical stimulation), (3) auditory brainstem implants (ABI) — for patients who have lost both cochlear nerves (typically after bilateral VS removal). ABIs directly stimulate the cochlear nucleus in the brainstem, bypassing the cochlear nerve. Sound quality is limited but provides environmental sound awareness and lip-reading support. (4) Communication support (lip-reading, sign language, communication aids). A: Multiple options exist. C/D/E: These are individual options within a broader rehabilitation framework.

Reference: NICE NG99; NF2 Hearing Guidelines