Case Report

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

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.

1. Stark Z, Savarirayan R. Osteopetrosis. Orphanet J Rare Dis. 2009;4:5. doi: https://doi.org/10.1186/1750-1172-4-5
2. Szymanski M, Zasławska K, Trojanowska A, Szymanska A, Zadrozniak M. Osteopetrosis of the temporal bone treated with cochlear implant. J Int Adv Otol. 2015;11(2):173-5. doi: 10.5152/iao.2015.449
3. Dozier TS, Duncan IM, Klein AJ, Lambert PR, Key LL Jr. Otologic manifestations of malignant osteopetrosis. Otol Neurotol. 2005;26(4):762-6. doi: 10.1097/01.mao.0000178139.27472.8d
4. Gniady JP, Ronis ML. Progressive mixed type hearing loss in an adult male with osteopetrosis. Laryngoscope. 2009;119(S1):S39-S39. doi: 10.1002/lary.20364
5. Sennaroglu L, Ziyal I. Auditory brainstem implantation. Auris Nasus Larynx. 2012;39(5):439-50. doi: 10.1016/j.anl.2011.10.013
6. Verhulst S, Jagadeesh A, Mauermann M, Ernst F. Individual differences in auditory brainstem response wave characteristics: relations to different aspects of peripheral hearing loss. Trends Hear. 2016;20:1-20. doi: 10.1177/2331216516672186
7. Motasaddi Zarandy M, Saki S, Saki N. The first step toward improving cochlear implant insertion using a new technique called slide method (Motasaddi Method). Int J Pharm Res Allied Sci. 2016;5(2):110-3.
8. Hsieh HS, Wu CM, Zhuo MY, Yang CH, Chao H, Hwang CF. Intraoperative facial nerve monitoring during cochlear implant surgery. an observational study. Medicine (Baltimore). 2015;94(4):e456. doi: 10.1097/MD.0000000000000456
9. Rathod AK, Dhake RP, Borde MD. Traumatic multiple cervical spine injuries in a patient with osteopetrosis and its management. Eur Spine J. 2017;26:229-35. doi: 10.1007/s00586-017-5083-x
10. Aslan A, Baykal YB, Uysal E, Atay T, Kirdemir V, Baydar ML, et al. Surgical treatment of osteopetrosis-related femoral fractures: two case reports and literature review. Case Rep Orthop. 2014;2014:891963. doi: 10.1155/2014/891963
Files
IssueVol 30 No 1 (2021) QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/avr.v30i1.5312
Keywords
Cochlear implantation osteopetrosis sensorineural hearing loss
Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
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. 2020;30(1):56-61.