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Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system

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Abstract

Objective

To report the audiological outcomes of cochlear implantation in two patients with severe to profound sensorineural hearing loss secondary to superficial siderosis of the CNS and discuss some programming peculiarities that were found in these cases.

Method

Retrospective review. Data concerning clinical presentation, diagnosis and audiological assessment pre- and post-implantation were collected of two patients with superficial siderosis of the CNS.

Results

Both patients showed good hearing thresholds but variable speech perception outcomes. One patient did not achieve open-set speech recognition, but the other achieved 70% speech recognition in quiet. Electrical compound action potentials could not be elicited in either patient. Map parameters showed the need for increased charge. Electrode impedances showed high longitudinal variability.

Conclusion

The implants were fairly beneficial in restoring hearing and improving communication abilities although many reprogramming sessions have been required. The hurdle in programming was the need of frequent adjustments due to the physiologic variations in electrical discharges and neural conduction, besides the changes in the impedances. Patients diagnosed with superficial siderosis may achieve limited results in speech perception scores due to both cochlear and retrocochlear reasons. Careful counseling about the results must be given to the patients and their families before the cochlear implantation indication.

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Authors and Affiliations

  1. Department of Otolaryngology, Hospital das Clínicas, University of São Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255, 6º andar/sala 6167, CEP, São Paulo, SP, 05403-000, Brazil

    Aline Gomes Bittencourt, Maria Valéria Schmidt Goffi-Gomez, Mariana Hausen Pinna, Ricardo Ferreira Bento, Rubens de Brito & Robinson Koji Tsuji

Authors
  1. Aline Gomes Bittencourt
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  2. Maria Valéria Schmidt Goffi-Gomez
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  3. Mariana Hausen Pinna
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  4. Ricardo Ferreira Bento
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  5. Rubens de Brito
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  6. Robinson Koji Tsuji
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Corresponding author

Correspondence to Aline Gomes Bittencourt.

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Bittencourt, A.G., Goffi-Gomez, M.V.S., Pinna, M.H. et al. Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system. Eur Arch Otorhinolaryngol 269, 1555–1563 (2012). https://doi.org/10.1007/s00405-011-1850-1

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  • DOI: https://doi.org/10.1007/s00405-011-1850-1

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