Successful cochlear implantation in a patient with superficial siderosis of the central nervous system.

To the Editor: As an addendum to our report of 2 cases of unsuccessful cochlear implantation in 2 patients with superficial siderosis of the central nervous system (1), we would like to present briefly a successful outcome on another patient with the condition. A 56-year-old man presented with a 10-year history of progressive hearing loss and ataxia. A diagnosis of superficial siderosis of the central nervous system was made using magnetic resonance imaging. At the time of candidacy testing for cochlear implantation, there was no recordable hearing in either ear, and communication was only possible via written material on a whiteboard. Hearing in noise test revealed 0% auditory-alone and 21% auditory-visual. The patient underwent extensive counseling regarding cochlear implantation, including receiving a copy of our previous publication. A Nucleus Freedom device with Contour Advance electrode (Cochlear, Ltd., Lane Cove, New South Wales, Australia) was implanted on the patient’s left ear without incident. After the operation, the patient’s balance was significantly worse, but there were no other complications. The device was switched on 4 weeks later. The patient has received significant benefit from the device and is now just over 6 months after switch-on. The patient’s speech discrimination scores for the first 6 months after switchon are as follows:

[1]  M. Ulfendahl,et al.  Functional Evaluation of a Cell Replacement Therapy in the Inner Ear , 2009, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[2]  C. Petit,et al.  Cells of adult brain germinal zone have properties akin to hair cells and can be used to replace inner ear sensory cells after damage , 2008, Proceedings of the National Academy of Sciences.

[3]  R. Altschuler,et al.  Glutamatergic Neuronal Differentiation of Mouse Embryonic Stem Cells after Transient Expression of Neurogenin 1 and Treatment with BDNF and GDNF: In Vitro and In Vivo Studies , 2008, The Journal of Neuroscience.

[4]  D. Cotanche,et al.  Genetic and pharmacological intervention for treatment/prevention of hearing loss. , 2008, Journal of communication disorders.

[5]  E. Giles,et al.  Unsuccessful Cochlear Implantation in Two Patients With Superficial Siderosis of the Central Nervous System , 2008, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[6]  A. Edge,et al.  Hair cell regeneration , 2008, Current Opinion in Neurobiology.

[7]  T. Xue,et al.  Proliferation, multipotency and neuronal differentiation of cryopreserved neural progenitor cells derived from the olfactory neuroepithelium of the adult rat , 2008, Cell biology international.

[8]  K. Beisel,et al.  Stem cells and molecular strategies to restore hearing. , 2008, Panminerva medica.

[9]  M. Behlke,et al.  Advances in Molecular and Cellular Therapies for Hearing Loss. , 2008, Molecular therapy : the journal of the American Society of Gene Therapy.

[10]  R. Shepherd,et al.  Concise Review: The Potential of Stem Cells for Auditory Neuron Generation and Replacement , 2007, Stem cells.

[11]  J. T. Corwin,et al.  Inner ear hair cells produced in vitro by a mesenchymal-to-epithelial transition , 2007, Proceedings of the National Academy of Sciences.

[12]  M. Parker,et al.  Neural stem cells injected into the sound-damaged cochlea migrate throughout the cochlea and express markers of hair cells, supporting cells, and spiral ganglion cells , 2007, Hearing Research.

[13]  M. Ulfendahl,et al.  A cell therapy approach to substitute neural elements in the inner ear , 2007, Physiology & Behavior.

[14]  S. Oleskevich,et al.  Differentiation of Adult Mouse Olfactory Precursor Cells into Hair Cells In Vitro , 2007, Stem cells.

[15]  S. Heller,et al.  Bone marrow mesenchymal stem cells are progenitors in vitro for inner ear hair cells , 2007, Molecular and Cellular Neuroscience.

[16]  R K Shepherd,et al.  Auditory hair cell explant co-cultures promote the differentiation of stem cells into bipolar neurons. , 2007, Experimental cell research.

[17]  J. R. Holt,et al.  Differential Distribution of Stem Cells in the Auditory and Vestibular Organs of the Inner Ear , 2007, Journal of the Association for Research in Otolaryngology.

[18]  M. Ulfendahl,et al.  Cell replacement therapy in the inner ear. , 2006, Stem cells and development.

[19]  R. Haeusler,et al.  An international otology database. , 2005, Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology.

[20]  J. Frisén,et al.  Survival and neural differentiation of adult neural stem cells transplanted into the mature inner ear. , 2005, Experimental cell research.

[21]  E. Dzierzak,et al.  Cell‐Cell Contact and Anatomical Compatibility in Stromal Cell‐Mediated HSC Support During Development , 2004, Stem cells.

[22]  R. Davis.  Gradients of Neurotrophins, Ion Channels, and Tuning in the Cochlea , 2003, The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry.

[23]  Huawei Li,et al.  Pluripotent stem cells from the adult mouse inner ear , 2003, Nature Medicine.

[24]  J. Ito,et al.  Trophic support of mouse inner ear by neural stem cell transplantation , 2003, Neuroreport.

[25]  G. Rettinger,et al.  Perioperative glucocorticoid treatment does not influence early post-laser stapedotomy hearing thresholds. , 2000, The American journal of otology.

[26]  M. Saarma,et al.  Coordinated expression and function of neurotrophins and their receptors in the rat inner ear during target innervation , 1994, Hearing Research.

[27]  M. Saarma,et al.  Expression patterns of neurotrophin and their receptor mRNAs in the rat inner ear , 1993, Hearing Research.