Timing of cochlear implantation in auditory neuropathy patients with OTOF mutations: Our experience with 10 patients

Auditory neuropathy spectrum disorder (ANSD) can be caused by a variety of pathologies ranging from inner hair cells, synapses, spiral ganglion neurons, the auditory nerve, to brainstem auditory nuclei.¹ For ANSD patients who derive limited benefit from hearing aids, cochlear implantation constitutes the treatment of last resort. However, corresponding to the heterogeneity in pathology, the outcomes with cochlear implants (CIs) vary significantly among ANSD patients.¹

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