A case report of cochlear implantation in patients with osteopetrosis: surgical approaches and auditory results

  • Masoud Motasaddi Zarandy ORCID Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran AND Department of Otolaryngology, Head and Neck Surgery, Amir A’lam Hospital, Tehran University of Medical Sciences, Tehran, Iran
  • Hakima Abdullah ORCID Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  • Mina Motasaddi Zarandy ORCID Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, Canada
  • Mohammadreza Firouzifar ORCID Mail Otorhinolaryngology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  • Farzad Moubedshahi ORCID Amir A’lam Cochlear Implant Center, Tehran University of Medical Sciences, Tehran, Iran
Keywords:
Cochlear implantation, osteopetrosis, sensorineural hearing loss

Abstract

Background: Osteopetrosis (OP) is a rare disease of the skeletal system that can be associated with complications such as bone fracture, nerve dysfunction and deafness due to increased bone density and reduced bone quality. In this regard and due to the challenge that it can cause for cochlear implantation (CI), in this study we aimed to report CI conducted on two patients with OP in Iran.
The Case: Patients were two women diagnosed with OP and bilateral sensorineural hearing loss (SNHL) who underwent CI in the right ear. Preoperative PTA showed a hearing threshold decrease of more than 100 dB in both patients. A standard cochleostomy was performed in one patient and endoscopic surgery in the other pati­ent through the external ear canal. One month after surgery, the hearing threshold improved by 60−90 dB in both patients. No facial nerve palsy or implant extrusion/migration was observed after surgery.
Conclusion: Although technically challenging, CI seems to be a safe and effective method to improve the SNHL in patients with OP. The path for electrode insertion should be tailored to meet the conditions and anatomy of patients.

References

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Published
2020-12-19
How to Cite
1.
Motasaddi Zarandy M, Abdullah H, Motasaddi Zarandy M, Firouzifar M, Moubedshahi F. A case report of cochlear implantation in patients with osteopetrosis: surgical approaches and auditory results. Aud Vestib Res. 30(1):56-61.
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Case Report(s)