Hearing rehabilitation by Cochlea Implantat in a case of Auditory Synaptopathy

Auditory Synaptopathy and Auditory Neuropathy are characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. Patients suffer from hearing loss characterized by normal otoacustic emission (OAE). Hyperbilirubinemia, perinatal Hypoxia, demyelinating and axonal neuropathies, premature birth and genetic lesions are discussed to be risk factors.

The 24-year-old patient presented himself in our clinic with a progressive hearing loss that had been diagnosed for about 2 years with a limited understanding of speech. Other sounds could be heard well.

The ENT medical examination showed an inconspicuous status. In audiological diagnostics, a moderate pantonal inner ear hearing loss with 40dB was evident on both sides with normal OAE. In the speech audiogram, however, a speech understanding left of 10 % and right of 30 % at 65dB was shown with a maximum of 50 % at 95dB. In the BERA, no threshold could be detected up to 90 dB on both sides. In MRI and CT central pathologies were excluded.

With the help of the latest hearing aid technology, there was no improvement in speech understanding, here the monosyllabic understanding was still at 10 % at 65 dB and 50 % at 95dB.

We performed a complication-free cochlear implantation (CI) initially left. In the control after 8 months monosyllable understanding was found at 65dB of 60 % left with a maximal understanding of 85 % at 90dB. The patient was very satisfied.

In the literature so far only a small group of patients with Auditory Synaptopathy, which were supplied by CI, is described. Almost all patients benefited from the operation, further studies are needed.

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Publication History

Article published online:
10 June 2020