![]() ![]() Recently, however, studies showed that CI could help to develop auditory skill in some of the patients with auditory neuropathy, yet the benefits were uncertain due to a wide range of etiologies ( Rance et al., 1999 Berlin et al., 2010 Humphriss et al., 2013). However, in patients who have failed auditory skills development with HA or who are with severe to profound hearing loss, CI is considered the only possible effective treatment ( Yawn et al., 2019).Īs the transmission of the signal from electrical stimulation of the spiral ganglion provided by the cochlear implants could be affected, it had been thought that the CI outcomes were relatively poor in patients with auditory neuropathy ( Starr et al., 1996). For hearing rehabilitation in patients with auditory neuropathy, both cochlear implantation (CI) and wearing hearing-aids (HA) are options. Individuals with auditory neuropathy typically show normal or near-normal otoacoustic emission (OAE) or cochlear microphonics (CM), but absent or abnormal auditory brainstem response (ABR) and/or middle ear muscle reflexes, usually accompanied by poor speech discrimination scores and poor understanding ( Starr et al., 1996). Also, the factors that may still influence the CI outcomes in patients with OTOF mutations are discussed.Īuditory neuropathy is a type of hearing loss caused by dysfunction of the synapse of the inner hair cells, the auditory nerve, and/or the auditory nerve itself ( Hayes and Sininger, 2008). We will focus on whether biallelic OTOF mutations are ideal indications for CI in patients with auditory neuropathy. In this review, we describe the CI outcomes in patients with OTOF mutations. ![]() Thus, OTOF-related auditory neuropathy is expected to have good CI performances. The site of lesion in patients with auditory neuropathy caused by biallelic OTOF mutations ( OTOF-related auditory neuropathy) is presumed to be presynaptic, leaving auditory nerve function intact. ![]() Mutations in the OTOF gene were the first identified and the most common cause of congenital auditory neuropathy. It is accepted that the exact sites of lesion causing auditory neuropathy determine the CI performance. For patients with auditory neuropathy who have severe to profound hearing loss or failed auditory skills development with hearing-aids, cochlear implantation (CI) serves as the only possible effective treatment.
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