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Cochlear implantation is a therapeutic option for superficial siderosis patients with sensorineural hearing loss

Published online by Cambridge University Press:  09 February 2016

R Omichi ,
S Kariya ,
Y Maeda  and
K Nishizaki
R Omichi
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan
S Kariya*
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan
Y Maeda
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan
K Nishizaki
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan
*
Address for correspondence: Dr Shin Kariya, Department of Otolaryngology – Head and Neck Surgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikata-Cho, Kita-Ku, Okayama 700-8558, Japan Fax: 81 86 235 7308 E-mail: [email protected]
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Abstract

Background:

Superficial siderosis is a rare disease that results from chronic bleeding in the subarachnoid space. Haemosiderin deposits throughout the subpial layers of the brain and spinal cord lead to progressive sensorineural hearing loss, which is seen in 95 per cent of patients with superficial siderosis. The impact of cochlear implantation on the quality of life of superficial siderosis patients is under debate.

Case report:

A 38-year-old male with superficial siderosis presented with bilateral progressive sensorineural hearing loss. The patient underwent cochlear implantation and his quality of life was improved as evaluated by the Abbreviated Profile of Hearing Aid Benefit inventory.

Conclusion:

The remarkable improvement in Abbreviated Profile of Hearing Aid Benefit scores shown in this study indicates that cochlear implantation leads to a better quality of life in superficial siderosis patients.

Keywords

SiderosisHearing LossCochlear ImplantationsQuality Of LifeQuestionnaires
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 130 , Issue 4 , April 2016 , pp. 408 - 411
Copyright
Copyright © JLO (1984) Limited 2016 

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References

1Hamill, R. Report of a case of melanosis of the brain, cord and meninges. J Nerv Ment Dis 1908;35:594CrossRefGoogle Scholar
2Sugimoto, H, Ito, M, Hatano, M, Yoshizaki, T. Cochlear implantation in a patient with superficial siderosis. Auris Nasus Larynx 2012;39:623–6Google Scholar
3Fearnley, JM, Stevens, JM, Rudge, P. Superficial siderosis of the central nervous system. Brain 1995;118:1051–66Google Scholar
4Grisoli, M, Maccagnano, E, De Simone, T, Savoiardo, M. Superficial siderosis of the CNS: selective central myelin vulnerability and peripheral myelin sparing demonstrated by MRI. Eur J Neurol 2007;14:e2–3CrossRefGoogle ScholarPubMed
5Koeppen, AH, Hurwitz, CG, Dearborn, RE, Dickson, AC, Borke, RC, Chu, RC. Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 1992;112:38–45Google Scholar
6Nadol, JB Jr, Adams, JC, O'Malley, JT. Temporal bone histopathology in a case of sensorineural hearing loss caused by superficial siderosis of the central nervous system and treated by cochlear implantation. Otol Neurotol 2011;32:748–55CrossRefGoogle Scholar
7Modest, MC, Carlson, ML, Wanna, GB, Driscoll, CL. Cochlear implantation in patients with superficial siderosis: seven cases and systematic review of the literature. Otol Neurotol 2015;36:1191–6Google Scholar
8Cox, RM, Alexander, GC. The abbreviated profile of hearing aid benefit. Ear Hear 1995;16:176–86CrossRefGoogle ScholarPubMed
9Yamana, T, Suzuki, M, Kitano, H. Neuro-otologic findings in a case of superficial siderosis with bilateral hearing impairment. J Otolaryngol 2001;30:187–9CrossRefGoogle Scholar
10Dodson, KM, Sismanis, A, Nance, WE. Superficial siderosis: a potentially important cause of genetic as well as non-genetic deafness. Am J Med Genet A 2004;130A:22–5Google Scholar
11Irving, RM, Graham, JM. Cochlear implantation in superficial siderosis. J Laryngol Otol 1996;110:1151–3CrossRefGoogle ScholarPubMed
12Takahashi, K, Kakoi, H, Takasuka, N, Kitamura, K. Cochlear implant for totally deaf patient with superficial hemosiderosis of the central nervous system. Adv Otorhinolaryngol 2000;57:138–40Google Scholar
13Dhooge, I, De Vel, E, Urgell, H, Gallego, S, Vinck, B. Cochlear implantation in a patient with superficial siderosis of the central nervous system. Otol Neurotol 2002;23:468–72CrossRefGoogle Scholar
14Kim, CS, Song, JJ, Park, MH, Kim, YH, Koo, JW. Cochlear implantation in superficial siderosis. Acta Otolaryngol 2006;126:892–6Google Scholar
15Hathaway, B, Hirsh, B, Branstetter, B. Successful cochlear implantation in a patient with superficial siderosis. Am J Otolaryngol 2006;27:255–8Google Scholar
16Yoshikawa, N, Hirsh, B. Cochlear implantation in a patient with superficial siderosis: an update. Am J Otolaryngol 2010;31:390–1Google Scholar
17Wood, VH, Bird, PA, Giles, EC, Baber, WJ. Unsuccessful cochlear implantation in two patients with superficial siderosis of the central nervous system. Otol Neurotol 2008;29:622–5Google Scholar
18Sydlowski, A, Cevette, MJ, Shallop, J, Barrs, DM. Cochlear implant patients with superficial siderosis. J Am Acad Audiol 2009;20:348–52Google Scholar
19Bird, PA, Monteath, P, Healy, L. Successful cochlear implantation in a patient with superficial siderosis of the central nervous system. Otol Neurotol 2010;31:177CrossRefGoogle Scholar
20Vanat, ZH, Harris, F, Axon, PR, Donnelly, N. Cochlear implant failure secondary to superficial haemosiderosis: exploring implantation in the contralateral ear with either a cochlear implant or an auditory brainstem implant. Cochlear Implants Int 2010;11(suppl 2):136–40Google Scholar
21Berrettini, S, De Vito, A, Bruschini, L, Fortunato, S, Forli, F. Cochlear implantation in patients affected by superficial hemosiderosis of the central nervous system. Eur Arch Otorhinolaryngol 2012;269:25–31Google Scholar
22Grover, N, Whiteside, OJ, Ramsden, JD. Cochlear implantation in superficial siderosis: a viable option? Cochlear Implants Int 2011;12:241–3Google Scholar
23Bittencourt, AG, Goffi-Gomez, MV, Pinna, MH, Bento, RF, de Brito, R, Tsuji, RK. Programming peculiarities in two cochlear implant users with superficial siderosis of the central nervous system. Eur Arch Otorhinolaryngol 2012;269:1555–63CrossRefGoogle ScholarPubMed
24Nogueira, C, Meehan, T. Successful outcome of cochlear implantation in a patient with superficial siderosis. B-ENT 2012;8:57–9Google Scholar
25Ryan, M, Piplica, D, Zhang, M. Case report: cochlear implantation in a patient with superficial siderosis. Otol Neurotol 2014;35:1742–5Google Scholar
26Buchman, CA, Dillon, MT, King, ER, Adunka, MC, Adunka, OF, Pillsbury, HC. Influence of cochlear implant insertion depth on performance: a prospective randomized trial. Otol Neurotol 2014;35:1773–9Google Scholar