{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T01:20:22Z","timestamp":1768267222133,"version":"3.49.0"},"reference-count":32,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:p>Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated. The proposed causes include prior intradural surgery, prior trauma, tumors, vascular abnormalities, nerve root avulsion, and dural abnormalities.<\/jats:p><jats:p>Surgical repair of a dural defect associated with SS has been shown to be efficacious at preventing symptomatic progression. There have been several reports of dural defects within the spinal canal treated with surgery. Here, the authors present the first known case of a dural defect of the ventral skull base, namely a clival meningocele, presumed to be causing SS. In this case report, a 10-year-old girl with a history of head trauma at the age of 3 years was found to have a clival meningocele 3 years after her original trauma. On follow-up imaging, the patient was found to have radiographic growth of the meningocele along with evidence of SS of the CNS. The patient was treated conservatively until she began to have progressive hearing loss. It was presumed that the growing meningocele was the source of her SS. An endoscopic endonasal transclival approach with a multilayer dural reconstruction was performed to fix the dural defect and repair the meningocele in hopes of mitigating the progression of her symptoms. At her 12-month postoperative follow-up, she was doing well, with audiometry showing a slightly decreased hearing threshold in the left ear but improved speech discrimination bilaterally. Postoperative MRI showed a stable level of hemosiderin deposition and meningocele repair. Long-term follow-up will be necessary to evaluate for continued clinical stabilization or possible improvement.<\/jats:p>","DOI":"10.3171\/2017.11.peds17302","type":"journal-article","created":{"date-parts":[[2018,2,16]],"date-time":"2018-02-16T14:55:36Z","timestamp":1518792936000},"page":"498-503","source":"Crossref","is-referenced-by-count":4,"title":["Clival meningocele causing bilateral hearing loss in a child due to superficial siderosis of the central nervous system: case report"],"prefix":"10.3171","volume":"21","author":[{"given":"Stephen J.","family":"Johans","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin N.","family":"Swong","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel J.","family":"Burkett","sequence":"additional","affiliation":[{"name":"Loyola University Chicago Stritch School of Medicine, Loyola University Medical Center, Maywood, Illinois; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael P.","family":"Wemhoff","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sean M.","family":"Lew","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Medical College of Wisconsin\/Children\u2019s Hospital of Wisconsin, Milwaukee, Wisconsin"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chirag R.","family":"Patel","sequence":"additional","affiliation":[{"name":"Otolaryngology, Loyola University Medical Center;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anand V.","family":"Germanwala","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"},{"name":"Otolaryngology, Loyola University Medical Center;"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1","series-title":"J Neuroimaging","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1111\/j.1552-6569.2007.00187.x","article-title":"CSF rhinorrhea from a transclival meningocele: a case report","volume":"18","author":"Akyuz M","year":"2008","unstructured":"Akyuz M, Arslan G, Gurkanlar D, Tuncer R: CSF rhinorrhea from a transclival meningocele: a case report. J Neuroimaging 18:191\u2013193, 20081829868110.1111\/j.1552-6569.2007.00187.x"},{"key":"b2","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1212\/WNL.52.1.163","article-title":"Superficial siderosis of the central nervous system: a late complication of cerebellar tumors","volume":"52","author":"Anderson NE","year":"1999","unstructured":"Anderson NE, Sheffield S, Hope JK: Superficial siderosis of the central nervous system: a late complication of cerebellar tumors. Neurology 52:163\u2013169, 1999992186510.1212\/WNL.52.1.163"},{"key":"b3","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"931","DOI":"10.3171\/jns.1994.80.5.0931","article-title":"Superficial siderosis of the central nervous system after brachial plexus injury. Case report","volume":"80","author":"Bonito V","year":"1994","unstructured":"Bonito V, Agostinis C, Ferraresi S, Defanti CA: Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg 80:931\u2013934, 199410.3171\/jns.1994.80.5.09318169638"},{"key":"b4","series-title":"Med Hypotheses","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1016\/j.mehy.2011.02.028","article-title":"A proposed mechanism of superficial siderosis supported by surgical and neuroimaging findings","volume":"76","author":"Cheng CY","year":"2011","unstructured":"Cheng CY, Chen MH, Wang SJ, Lin KP: A proposed mechanism of superficial siderosis supported by surgical and neuroimaging findings. Med Hypotheses 76:823\u2013826, 201110.1016\/j.mehy.2011.02.02821392892"},{"key":"b5","series-title":"J Neurosurg Spine","doi-asserted-by":"publisher","first-page":"206","DOI":"10.3171\/spi.2005.2.2.0206","article-title":"Central nervous system superficial siderosis following spinal surgery","volume":"2","author":"Cohen-Gadol AA","year":"2005","unstructured":"Cohen-Gadol AA, Atkinson PP, Krauss WE: Central nervous system superficial siderosis following spinal surgery. J Neurosurg Spine 2:206\u2013208, 200510.3171\/spi.2005.2.2.020615739535"},{"issue":"5","key":"b6","series-title":"Neurosurg Focus","doi-asserted-by":"publisher","first-page":"E10","DOI":"10.3171\/foc.2004.16.5.11","article-title":"Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases","volume":"16","author":"Cohen-Gadol AA","year":"2004","unstructured":"Cohen-Gadol AA, Krauss WE, Spinner RJ: Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 16(5):E10, 200410.3171\/foc.2004.16.5.1115174830"},{"key":"b7","series-title":"J Neurosurg Spine","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1212\/01.wnl.0000172345.05810.14","article-title":"Dural closure for the treatment of superficial siderosis","volume":"18","author":"Egawa S","year":"2013","unstructured":"Egawa S, Yoshii T, Sakaki K, Inose H, Kato T, Kawabata S, : Dural closure for the treatment of superficial siderosis. J Neurosurg Spine 18:388\u2013393, 20132343232210.1212\/01.wnl.0000172345.05810.14"},{"key":"b8","series-title":"Brain","doi-asserted-by":"publisher","first-page":"1051","DOI":"10.1093\/brain\/118.4.1051","article-title":"Superficial siderosis of the central nervous system","volume":"118","author":"Fearnley JM","year":"1995","unstructured":"Fearnley JM, Stevens JM, Rudge P: Superficial siderosis of the central nervous system. Brain 118:1051\u20131066, 199510.1093\/brain\/118.4.1051"},{"issue":"3 Suppl Operative","key":"b9","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"ons150","DOI":"10.1227\/01.NEU.0000383130.80179.41","article-title":"Endoscopic endonasal minimal access approach to the clivus: case series and technical nuances","volume":"67","author":"Fraser JF","year":"2010","unstructured":"Fraser JF, Nyquist GG, Moore N, Anand VK, Schwartz TH: Endoscopic endonasal minimal access approach to the clivus: case series and technical nuances. Neurosurgery 67 (3 Suppl Operative):ons150\u2013ons158, 201010.1227\/01.NEU.0000383130.80179.41"},{"key":"b10","series-title":"Clin Neurol Neurosurg","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1002\/ana.410400620","article-title":"CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: two case reports","volume":"112","author":"Ikeda T","year":"2010","unstructured":"Ikeda T, Noto D, Noguchi-Shinohara M, Ono K, Takahashi K, Ishida C, : CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: two case reports. Clin Neurol Neurosurg 112:62\u201364, 20101974776610.1002\/ana.410400620"},{"key":"b11","series-title":"Otol Neurotol","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1097\/00129492-200301000-00019","article-title":"Superficial siderosis of the meninges and its otolaryngologic connection: a series of five patients","volume":"24","author":"Kale SU","year":"2003","unstructured":"Kale SU, Donaldson I, West RJ, Shehu A: Superficial siderosis of the meninges and its otolaryngologic connection: a series of five patients. Otol Neurotol 24:90\u201395, 200310.1097\/00129492-200301000-0001912544036"},{"issue":"1","key":"b12","series-title":"Neurosurg Focus","doi-asserted-by":"publisher","first-page":"E6","DOI":"10.3171\/foc.2005.19.1.7","article-title":"Expanded endonasal approach: fully endoscopic, completely transnasal approach to the middle third of the clivus, petrous bone, middle cranial fossa, and infratemporal fossa","volume":"19","author":"Kassam AB","year":"2005","unstructured":"Kassam AB, Gardner P, Snyderman C, Mintz A, Carrau R: Expanded endonasal approach: fully endoscopic, completely transnasal approach to the middle third of the clivus, petrous bone, middle cranial fossa, and infratemporal fossa. Neurosurg Focus 19(1):E6, 200510.3171\/foc.2005.19.1.716078820"},{"key":"b13","series-title":"Ann Neurol","doi-asserted-by":"publisher","first-page":"646","DOI":"10.3171\/foc.2004.16.5.11","article-title":"The pathogenesis of superficial siderosis of the central nervous system","volume":"34","author":"Koeppen AH","year":"1993","unstructured":"Koeppen AH, Dickson AC, Chu RC, Thach RE: The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34:646\u2013653, 1993823955810.3171\/foc.2004.16.5.11"},{"key":"b14","series-title":"Acta Neuropathol","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1007\/s00401-008-0421-z","article-title":"The pathology of superficial siderosis of the central nervous system","volume":"116","author":"Koeppen AH","year":"2008","unstructured":"Koeppen AH, Michael SC, Li D, Chen Z, Cusack MJ, Gibson WM, : The pathology of superficial siderosis of the central nervous system. Acta Neuropathol 116:371\u2013382, 200810.1007\/s00401-008-0421-z18696091"},{"key":"b15","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"718","DOI":"10.3171\/foc.2005.19.1.7","article-title":"Superficial siderosis of the central nervous system from a bleeding pseudomeningocele. Case illustration","volume":"100","author":"Kole MK","year":"2004","unstructured":"Kole MK, Steven D, Kirk A, Lownie SP: Superficial siderosis of the central nervous system from a bleeding pseudomeningocele. Case illustration. J Neurosurg 100:718, 20041507013110.3171\/foc.2005.19.1.7"},{"key":"b16","series-title":"AJNR Am J Neuroradiol","doi-asserted-by":"publisher","first-page":"5","DOI":"10.3174\/ajnr.A1628","article-title":"Neuroimaging in superficial siderosis: an in-depth look","volume":"31","author":"Kumar N","year":"2010","unstructured":"Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol 31:5\u201314, 201010.3174\/ajnr.A162819729538"},{"key":"b17","series-title":"Arch Neurol","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1001\/archneur.64.4.491","article-title":"Superficial siderosis: associations and therapeutic implications","volume":"64","author":"Kumar N","year":"2007","unstructured":"Kumar N: Superficial siderosis: associations and therapeutic implications. Arch Neurol 64:491\u2013496, 20071742031010.1001\/archneur.64.4.491"},{"key":"b18","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"1144","DOI":"10.1212\/01.wnl.0000208510.76323.5b","article-title":"Superficial siderosis","volume":"66","author":"Kumar N","year":"2006","unstructured":"Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE: Superficial siderosis. Neurology 66:1144\u20131152, 200610.1212\/01.wnl.0000208510.76323.5b16636229"},{"key":"b19","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1212\/01.wnl.0000342457.22536.af","article-title":"Superficial siderosis: sealing the defect","volume":"72","author":"Kumar N","year":"2009","unstructured":"Kumar N, Lane JI, Piepgras DG: Superficial siderosis: sealing the defect. Neurology 72:671\u2013673, 20091922130310.1212\/01.wnl.0000342457.22536.af"},{"key":"b20","series-title":"Neurology","first-page":"486","article-title":"Role of dynamic CT myelography in identifying the etiology of superficial siderosis","volume":"65","author":"Kumar N","year":"2005","unstructured":"Kumar N, Lindell EP, Wilden JA, Davis DH: Role of dynamic CT myelography in identifying the etiology of superficial siderosis. Neurology 65:486\u2013488, 200516087925"},{"key":"b21","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"925","DOI":"10.1212\/01.wnl.0000267847.69896.be","article-title":"Superficial siderosis and CSF hypovolemia: the defect (dural) in the link","volume":"69","author":"Kumar N","year":"2007","unstructured":"Kumar N, McKeon A, Rabinstein AA, Kalina P, Ahlskog JE, Mokri B: Superficial siderosis and CSF hypovolemia: the defect (dural) in the link. Neurology 69:925\u2013926, 20071772429710.1212\/01.wnl.0000267847.69896.be"},{"key":"b22","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"1326","DOI":"10.3171\/2014.12.JNS141920","article-title":"Superficial siderosis of the central nervous system associated with incomplete dural closure following posterior fossa surgery: report of 3 cases","volume":"123","author":"Kumar R","year":"2015","unstructured":"Kumar R, Jacob JT, Welker KM, Cutrer FM, Link MJ, Atkinson JL, : Superficial siderosis of the central nervous system associated with incomplete dural closure following posterior fossa surgery: report of 3 cases. J Neurosurg 123:1326\u20131330, 20152606761910.3171\/2014.12.JNS141920"},{"key":"b23","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"553","DOI":"10.3171\/jns.1980.53.4.0553","article-title":"CSF rhinorrhea from a transclival meningocele demonstrated with metrizamide CT cisternography. Case report","volume":"53","author":"Lockwood AH","year":"1980","unstructured":"Lockwood AH, Quencer RM, Page LK: CSF rhinorrhea from a transclival meningocele demonstrated with metrizamide CT cisternography. Case report. J Neurosurg 53:553\u2013555, 1980742018010.3171\/jns.1980.53.4.0553"},{"key":"b24","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1326","article-title":"Superficial siderosis of the central nervous system many years after neurosurgical procedures","volume":"74","author":"McCarron MO","year":"2003","unstructured":"McCarron MO, Flynn PA, Owens C, Wallace I, Mirakhur M, Gibson JM, : Superficial siderosis of the central nervous system many years after neurosurgical procedures. J Neurol Neurosurg Psychiatry 74:1326\u20131328, 200312933948"},{"issue":"Suppl 1","key":"b25","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"S51","DOI":"10.1007\/BF02278119","article-title":"Superficial siderosis of the central nervous system: MRI findings and clinical significance","volume":"38","author":"Offenbacher H","year":"1996","unstructured":"Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G: Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 38 (Suppl 1):S51\u2013S56, 199610.1007\/BF02278119"},{"key":"b26","series-title":"Skull Base","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1055\/s-0030-1253573","article-title":"How to choose? Endoscopic skull base reconstructive options and limitations","volume":"20","author":"Patel MR","year":"2010","unstructured":"Patel MR, Stadler ME, Snyderman CH, Carrau RL, Kassam AB, Germanwala AV, : How to choose? Endoscopic skull base reconstructive options and limitations. Skull Base 20:397\u2013404, 201010.1055\/s-0030-125357321772795"},{"key":"b27","series-title":"Am J Otol","first-page":"415","article-title":"Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia","volume":"15","author":"Pribitkin EA","year":"1994","unstructured":"Pribitkin EA, Rondinella L, Rosenberg Si, Yousem DM: Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia. Am J Otol 15:415\u2013418, 19948579152"},{"key":"b28","series-title":"Spine J","doi-asserted-by":"publisher","first-page":"e16","DOI":"10.1016\/j.spinee.2009.03.004","article-title":"Surgical management of superficial siderosis","volume":"9","author":"Shih P","year":"2009","unstructured":"Shih P, Yang BP, Batjer HH, Liu JC: Surgical management of superficial siderosis. Spine J 9:e16\u2013e19, 200910.1016\/j.spinee.2009.03.004"},{"key":"b29","series-title":"Ann Neurol","first-page":"936","article-title":"Surgical management of superficial siderosis following cervical nerve root avulsion","volume":"40","author":"Tapscott SJ","year":"1996","unstructured":"Tapscott SJ, Eskridge J, Kliot M: Surgical management of superficial siderosis following cervical nerve root avulsion. Ann Neurol 40:936\u2013940, 19969007103"},{"key":"b30","series-title":"Br J Neurosurg","doi-asserted-by":"publisher","first-page":"188","DOI":"10.1080\/02688690151127626","article-title":"Occipito-clival intradiploic meningocoele following skull fracture in infancy","volume":"15","author":"Tizzard S","year":"2001","unstructured":"Tizzard S, Gleave J, Antoun N, Macfarlane R: Occipito-clival intradiploic meningocoele following skull fracture in infancy. Br J Neurosurg 15:188\u2013190, 20011136039110.1080\/02688690151127626"},{"key":"b31","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1007\/BF01411183","article-title":"CSF rhinorrhea from a transclival meningocele demonstrated by MR","volume":"138","author":"Turanzas FS","year":"1996","unstructured":"Turanzas FS, Lobato RD, Le\u00f3n PG, G\u00f3mez PA: CSF rhinorrhea from a transclival meningocele demonstrated by MR. Acta Neurochir (Wien) 138:595\u2013596, 1996880033810.1007\/BF01411183"},{"key":"b32","series-title":"Clin Imaging","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1016\/S0899-7071(96)00050-2","article-title":"Superficial siderosis of the central nervous system. Its MRI manifestations","volume":"21","author":"Uchino A","year":"1997","unstructured":"Uchino A, Aibe H, Itoh H, Aiko Y, Tanaka M: Superficial siderosis of the central nervous system. Its MRI manifestations. Clin Imaging 21:241\u2013245, 199710.1016\/S0899-7071(96)00050-29215469"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/21\/5\/article-p498.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/21\/5\/article-p498.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/21\/5\/article-p498.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T13:43:37Z","timestamp":1681566217000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/21\/5\/article-p498.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,5]]},"references-count":32,"journal-issue":{"issue":"5"},"URL":"https:\/\/doi.org\/10.3171\/2017.11.peds17302","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,5]]}}}