Skip to main content

Advertisement

SpringerLink
Log in

Hearing loss in hydrocephalus: a review, with focus on mechanisms

  • Review
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

While neither hydrocephalus nor cerebrospinal fluid (CSF) shunt placement is traditionally considered in the differential diagnosis of hearing loss, there is substantial evidence that CSF circulation and pressure abnormalities can produce auditory dysfunction. Several indirect mechanisms may explain association between hydrocephalus and hearing loss, including mass effect, compromise of the auditory pathway, complications of prematurity, and genetically mediated hydrocephalus and hearing loss. Nevertheless, researchers have proposed a direct mechanism, which we term the hydrodynamic theory. In this hypothesis, the intimate relationship between CSF and inner ear fluids permits relative endolymphatic or perilymphatic hydrops in the setting of CSF pressure disturbances. CSF is continuous with perilymph, and CSF pressure changes are known to produce parallel perilymphatic pressure changes. In support of the hydrodynamic theory, some studies have found an independent association between hydrocephalus and hearing loss. Moreover, surgical shunting of CSF has been linked to both resolution and development of auditory dysfunction. The disease burden of hydrocephalus-associated hearing loss may be large, and because hydrocephalus and over-shunting are reversible, this relationship merits broader recognition. Hydrocephalic patients should be monitored for hearing loss, and hearing loss in a patient with shunted hydrocephalus should prompt further evaluation and possibly adjustment of shunt settings.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Adeleye AO, Balogun JA (2009) Bilateral deafness and blindness from a IVth ventricular medulloblastoma. Br J Neurosurg 23:315–317

    Article  Google Scholar 

  2. Ahmmed AU, Mackenzie I, Das VK, Chatterjee S, Lye RH (1996) Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: a case report. J Laryngol Otol 110:1060–1064

    PubMed  CAS  Google Scholar 

  3. Al Sawaf S, Mayatepek E, Hoffmann B (2008) Neurological findings in Hunter disease: pathology and possible therapeutic effects reviewed. J Inherit Metab Dis 31:473–480

    Article  PubMed  CAS  Google Scholar 

  4. Albers FW, Ingels KJ (1993) Otoneurological manifestations in Chiari-I malformation. J Laryngol Otol 107:441–443

    Article  PubMed  CAS  Google Scholar 

  5. Aschoff A, Kremer P, Hashemi B, Kunze S (1999) The scientific history of hydrocephalus and its treatment. Neurosurg Rev 22:67–93

    Article  PubMed  CAS  Google Scholar 

  6. Bachor E, Byahatti S, Karmody CS (1997) The cochlear aqueduct in pediatric temporal bones. Eur Arch Otorhinolaryngol 254(Suppl 1):S34–S38

    Article  PubMed  Google Scholar 

  7. Bachor E, Byahatti S, Karmody CS (1999) New aspects in the histopathology of the cochlear aqueduct in children. Am J Otol 20:612–620

    PubMed  CAS  Google Scholar 

  8. Ballot DE, Rothberg AD, Katz BJ (1992) Speech and hearing problems in a high-risk population. S Afr Med J 82:23–26

    PubMed  CAS  Google Scholar 

  9. Baradaranfar MH, Atighechi S, Dadgarnia MH, Jafari R, Karimi G, Mollasadeghi A et al (2011) Hearing status in neonatal hyperbilirubinemia by auditory brain stem evoked response and transient evoked otoacoustic emission. Acta Med Iran 49:109–112

    PubMed  Google Scholar 

  10. Barajas-Barajas LO, Valdez LL, Gonzalez JR, Garcia-Garcia C, Rivera H, Ramirez L (2004) Sensorineural deafness in two infants: a novel feature in the 22q distal duplication syndrome. Cardinal signs in trisomies 22 subtypes. Genet Couns 15:167–173

    PubMed  CAS  Google Scholar 

  11. Barlas O, Gokay H, Turantan MI, Baserer N (1983) Adult aqueductal stenosis presenting with fluctuating hearing loss and vertigo. Report of two cases. J Neurosurg 59:703–705

    Article  PubMed  CAS  Google Scholar 

  12. Barnet AB, Weiss IP, Shaer C (1993) Evoked potentials in infant brainstem syndrome associated with Arnold-Chiari malformation. Dev Med Child Neurol 35:42–48

    Article  PubMed  CAS  Google Scholar 

  13. Bohmer A, Andrews JC (1989) Maintenance of hydrostatic pressure gradients in the membranous labyrinth. Arch Otorhinolaryngol 246:65–66

    Article  PubMed  CAS  Google Scholar 

  14. Bolisetty S, Dhawan A, Abdel-Latif M, Bajuk B, Stack J, Lui K et al (2014) Intraventricular hemorrhage and neurodevelopmental outcomes in extreme preterm infants. Pediatrics 133:55–62

    Article  PubMed  Google Scholar 

  15. Bondurant CP, Jimenez DF (1995) Epidemiology of cerebrospinal fluid shunting. Pediatr Neurosurg 23:254–258

    Article  PubMed  CAS  Google Scholar 

  16. Brown DH, McClure JA, Downar-Zapolski Z (1988) The membrane rupture theory of Meniere’s disease—is it valid? Laryngoscope 98:599–601

    PubMed  CAS  Google Scholar 

  17. Carlborg BI, Farmer JC (1983) Transmission of cerebrospinal fluid pressure via the cochlear aqueduct and endolymphatic sac. Am J Otolaryngol 4:273–282

    Article  PubMed  CAS  Google Scholar 

  18. Carlborg BI, Konradsson KS, Carlborg AH, Farmer JC, Densert O (1992) Pressure transfer between the perilymph and the cerebrospinal fluid compartments in cats. Am J Otol 13:41–48

    PubMed  CAS  Google Scholar 

  19. Chandrasekhar SS (2008) Meniere disease. In: Weber PC (ed) Vertigo and disequilibrium: a practical guide to diagnosis and management, 1st edn. Thieme, New York, pp 56–68

  20. Chatterjee S, Chatterjee U (2011) Overview of post-infective hydrocephalus. Childs Nerv Syst 27:1693–1698

    Article  PubMed  Google Scholar 

  21. Cinalli G, Sainte-Rose C, Simon I, Lot G, Sgouros S (1999) Sylvian aqueduct syndrome and global rostral midbrain dysfunction associated with shunt malfunction. J Neurosurg 90:227–236

    Article  PubMed  CAS  Google Scholar 

  22. Clarke LA, Heppner J (1993) Mucopolysaccharidosis Type I. In: Pagon RA, Adam MP, Ardinger HH, Bird TD, Dolan CR, Fong CT et al (eds) GeneReviews. University of Washington, Seattle

    Google Scholar 

  23. Coenraad S, Goedegebure A, van Goudoever JB, Hoeve LJ (2010) Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controls. Int J Pediatr Otorhinolaryngol 74:999–1002

    Article  PubMed  CAS  Google Scholar 

  24. Crea KN, Shivdasani MN, Argent RE, Mauger SJ, Rathbone GD, O’Leary SJ et al (2010) Acute cochlear nucleus compression alters tuning properties of inferior colliculus neurons. Audiol Neurootol 15:18–26

    Article  PubMed  Google Scholar 

  25. Cristobal R, Oghalai JS (2008) Hearing loss in children with very low birth weight: current review of epidemiology and pathophysiology. Arch Dis Child Fetal Neonatal Ed 93:F462–F468

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  26. Deck MD, Deonarine V, Potts DG (1973) The rate of cerebrospinal fluid formation proximal and distal to aqueductal obstruction in the dog. Radiology 108:607–611

    Article  PubMed  CAS  Google Scholar 

  27. Digilio MC, Pacifico C, Tieri L, Marino B, Giannotti A, Dallapiccola B (1999) Audiological findings in patients with microdeletion 22q11 (di George/velocardiofacial syndrome). Br J Audiol 33:329–333

    Article  PubMed  CAS  Google Scholar 

  28. Dinda AK, Mahapatra AK, Sarkar C, Dhir R, Kharbanda K (1994) Primary cerebellopontine angle choroid plexus papilloma: a case report and review of literature. Indian J Cancer 31:48–51

    PubMed  CAS  Google Scholar 

  29. Dixon JF, Jones RO (2012) Hydrocephalus-associated hearing loss and resolution after ventriculostomy. Otolaryngol Head Neck Surg 146:1037–1039

    Article  PubMed  Google Scholar 

  30. Dodge PR, Davis H, Feigin RD, Holmes SJ, Kaplan SL, Jubelirer DP et al (1984) Prospective evaluation of hearing impairment as a sequela of acute bacterial meningitis. N Engl J Med 311:869–874

    Article  PubMed  CAS  Google Scholar 

  31. Dolgun H, Turkoglu E, Kertmen H, Yilmaz ER, Sekerci Z (2009) Chiari Type I malformation presenting with bilateral hearing loss. J Clin Neurosci 16:1228–1230

    Article  PubMed  Google Scholar 

  32. Dorman PJ, Campbell MJ, Maw AR (1995) Hearing loss as a false localising sign in raised intracranial pressure. J Neurol Neurosurg Psychiatry 58:516

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  33. Edmond K, Clark A, Korczak VS, Sanderson C, Griffiths UK, Rudan I (2010) Global and regional risk of disabling sequelae from bacterial meningitis: a systematic review and meta-analysis. Lancet Infect Dis 10:317–328

    Article  PubMed  Google Scholar 

  34. Edwards CG, Durieux-Smith A, Picton TW (1985) Auditory brainstem response audiometry in neonatal hydrocephalus. J Otolaryngol Suppl 14:40–46

    PubMed  CAS  Google Scholar 

  35. Erduran E, Mocan H, Gedik Y, Kamaci R, Okten A, Deger O (1995) Hydrocephalus, corneal opacities, deafness, left ventricle hypertrophy, clinodactyly in an adolescent patient. A new syndrome associated with glucocerebrosidase deficiency. Genet Couns 6:211–215

    PubMed  CAS  Google Scholar 

  36. Fernell E, Hagberg G, Hagberg B (1994) Infantile hydrocephalus epidemiology: an indicator of enhanced survival. Arch Dis Child Fetal Neonatal Ed 70:F123–F128

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  37. Freeman SR, Jones PH (2002) Old age presentation of the Dandy-Walker syndrome associated with unilateral sudden sensorineural deafness and vertigo. J Laryngol Otol 116:127–131

    Article  PubMed  CAS  Google Scholar 

  38. Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H (2010) Congenital hydrocephalus—prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol 14:150–155

    Article  PubMed  Google Scholar 

  39. Gerganov VM, Pirayesh A, Nouri M, Hore N, Luedemann WO, Oi S et al (2011) Hydrocephalus associated with vestibular schwannomas: management options and factors predicting the outcome. J Neurosurg 114:1209–1215

    PubMed  Google Scholar 

  40. Giannini C, Scheithauer BW (2003) Tumors of the peripheral nervous system (including the craniospinal nerve roots). In: Nelson JS, Mena H, Parisi JE, Schochet SS (eds) Principles and Practice of Neuropathology, 2nd edn. New York, Oxford, pp 425–447

    Google Scholar 

  41. Gopen Q, Rosowski JJ, Merchant SN (1997) Anatomy of the normal human cochlear aqueduct with functional implications. Hear Res 107:9–22

    Article  PubMed  CAS  Google Scholar 

  42. Gordon AG (1984) Endolymphatic hydrops and CSF pressure. J Neurosurg 60:1332–1334

    PubMed  CAS  Google Scholar 

  43. Gordon AG (2013) Endolymphatic hydrops and cerebrospinal fluid pressure: a 30-year update. Otolaryngol Head Neck Surg 148:707

    Article  PubMed  Google Scholar 

  44. Gould DB, Jaafar MS, Addison MK, Munier F, Ritch R, MacDonald IM et al (2004) Phenotypic and molecular assessment of seven patients with 6p25 deletion syndrome: relevance to ocular dysgenesis and hearing impairment. BMC Med Genet 5:17

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  45. Greenberg MS (2010) Handbook of Neurosurgery, 7th edn. Thieme, New York

    Google Scholar 

  46. Guillaume DJ, Knight K, Marquez C, Kraemer DF, Bardo DM, Neuwelt EA (2012) Cerebrospinal fluid shunting and hearing loss in patients treated for medulloblastoma. J Neurosurg Pediatr 9:421–427

    Article  PubMed  Google Scholar 

  47. Hackmann K, Stadler A, Schallner J, Franke K, Gerlach EM, Schrock E et al (2013) Severe intellectual disability, West syndrome, Dandy-Walker malformation, and syndactyly in a patient with partial tetrasomy 17q25.3. Am J Med Genet A 161A:3144–3149

    Article  PubMed  CAS  Google Scholar 

  48. Hatayama T, Sekiya T, Suzuki S, Iwabuchi T (1999) Effect of compression on the cochlear nerve: a short- and long-term electrophysiological and histological study. Neurol Res 21:599–610

    PubMed  CAS  Google Scholar 

  49. Heuer GG, Gabel B, Lemberg PS, Sutton LN (2008) Chiari I malformation presenting with hearing loss: surgical treatment and literature review. Childs Nerv Syst 24:1063–1066

    Article  PubMed  Google Scholar 

  50. Hughson W (1932) A note on the relationship of cerebrospinal and intralabyrinthine pressures. Am J Physiol 101:396–407

    Google Scholar 

  51. Jeng S, Gupta N, Wrensch M, Zhao S, Wu YW (2011) Prevalence of congenital hydrocephalus in California, 1991–2000. Pediatr Neurol 45:67–71

    Article  PubMed  Google Scholar 

  52. Jindal M, Hiam L, Raman A, Rejali D (2009) Idiopathic intracranial hypertension in otolaryngology. Eur Arch Otorhinolaryngol 266:803–806

    Article  PubMed  Google Scholar 

  53. Johnson GD, Harbaugh RE, Lenz SB (1994) Surgical decompression of Chiari I malformation for isolated progressive sensorineural hearing loss. Am J Otol 15:634–638

    Article  PubMed  CAS  Google Scholar 

  54. Kalra SK, Vaid VK, Jaiswal AK, Behari S, Jain VK, Kumari N (2008) Primary midbrain germinoma. J Clin Neurosci 15:1282–1284

    Article  PubMed  Google Scholar 

  55. Kaufmann GE, Clark K (1970) Continuous simultaneous monitoring of intraventricular and cervical subarachnoid cerebrospinal fluid pressure to indicate development of cerebral or tonsillar herniation. J Neurosurg 33:145–150

    Article  PubMed  CAS  Google Scholar 

  56. Keane WM, Potsic WP, Rowe LD, Konkle DF (1979) Meningitis and hearing loss in children. Arch Otolaryngol 105:39–44

    Article  PubMed  CAS  Google Scholar 

  57. Klein OD, Backstrand K, Cotter PD, Marco E, Sherr E, Slavotinek A (2005) Case report: Y;6 translocation with deletion of 6p. Clin Dysmorphol 14:93–96

    Article  PubMed  Google Scholar 

  58. Lary S, De Vries LS, Kaiser A, Dubowitz LM, Dubowitz V (1989) Auditory brain stem responses in infants with posthaemorrhagic ventricular dilatation. Arch Dis Child 64:17–23

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  59. Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery 12:377–390

    Article  PubMed  CAS  Google Scholar 

  60. Lim HW, Shim BS, Yang CJ, Kim JH, Cho YH, Cho YS et al (2014) Hearing loss following ventriculoperitoneal shunt in communicating hydrocephalus patients: a pilot study. Laryngoscope 124:1923–1927

    Article  PubMed  Google Scholar 

  61. Lim ST, Potts DG, Deonarine V, Deck MD (1973) Ventricular compliance in dogs with and without aqueductal obstruction. J Neurosurg 39:463–473

    Article  PubMed  CAS  Google Scholar 

  62. Lin RJ, Cherry AM, Chen KC, Lyons M, Hoyme HE, Hudgins L (2005) Terminal deletion of 6p results in a recognizable phenotype. Am J Med Genet A 136:162–168

    Article  PubMed  Google Scholar 

  63. Lopponen H, Sorri M, Serlo W, von Wendt L (1989) Audiological findings of shunt-treated hydrocephalus in children. Int J Pediatr Otorhinolaryngol 18:21–30

    Article  PubMed  CAS  Google Scholar 

  64. Lowry RB, Gould DB, Walter MA, Savage PR (2007) Absence of PITX2, BARX1, and FOXC1 mutations in De Hauwere syndrome (Axenfeld-Rieger anomaly, hydrocephaly, hearing loss): a 25-year follow up. Am J Med Genet A 143A:1227–1230

    Article  PubMed  CAS  Google Scholar 

  65. Lutschg J, Meyer E, Jeanneret-Iseli C, Kaiser G (1985) Brainstem auditory evoked potentials in meningomyelocele. Neuropediatrics 16:202–204

    Article  PubMed  CAS  Google Scholar 

  66. Mademont-Soler I, Morales C, Armengol L, Soler A, Sanchez A (2010) Description of the smallest critical region for Dandy-Walker malformation in chromosome 13 in a girl with a cryptic deletion related to t(6;13)(q23;q32). Am J Med Genet A 152A:2308–2312

    Article  PubMed  Google Scholar 

  67. Marchbanks RJ, Reid A (1990) Cochlear and cerebrospinal fluid pressure: their inter-relationship and control mechanisms. Br J Audiol 24:179–187

    Article  PubMed  CAS  Google Scholar 

  68. Martinez-Cruz CF, Poblano A, Fernandez-Carrocera LA (2008) Risk factors associated with sensorineural hearing loss in infants at the neonatal intensive care unit: 15-year experience at the National Institute of Perinatology (Mexico City). Arch Med Res 39:686–694

    Article  PubMed  Google Scholar 

  69. Matsumoto M, Sekiya T, Kojima K, Ito J (2008) An animal experimental model of auditory neuropathy induced in rats by auditory nerve compression. Exp Neurol 210:248–256

    Article  PubMed  Google Scholar 

  70. McPherson DL, Amlie R, Foltz E (1985) Auditory brainstem response in infant hydrocephalus. Childs Nerv Syst 1:70–76

    Article  PubMed  CAS  Google Scholar 

  71. Merchant SN, Adams JC, Nadol JB (2005) Pathophysiology of Meniere’s syndrome: are symptoms caused by endolymphatic hydrops? Otol Neurotol 26:74–81

    Article  PubMed  Google Scholar 

  72. Merchant TE, Gould CJ, Xiong X, Robbins N, Zhu J, Pritchard DL et al (2004) Early neuro-otologic effects of three-dimensional irradiation in children with primary brain tumors. Int J Radiat Oncol Biol Phys 58:1194–1207

    Article  PubMed  Google Scholar 

  73. Michel O, Brusis T (1992) Hearing loss as a sequel of lumbar puncture. Ann Otol Rhinol Laryngol 101:390–394

    Article  PubMed  CAS  Google Scholar 

  74. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C et al (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017

    Article  PubMed  CAS  Google Scholar 

  75. Miyakoshi A, Kohno M, Nagata O, Sora S, Sato H (2013) Hydrocephalus associated with vestibular schwannomas: perioperative changes in cerebrospinal fluid. Acta Neurochir (Wien) 155:1271–1276

    Article  Google Scholar 

  76. Miyazaki Y, Tomii M, Sawauchi S, Ikeuchi S, Yuki K, Abe T (1997) A case of hearing loss caused by overdrainage of cerebrospinal fluid after ventriculo-peritoneal shunting procedure. No Shinkei Geka 25:367–371

    PubMed  CAS  Google Scholar 

  77. Mokry M, Flaschka G, Kleinert G, Kleinert R, Fazekas F, Kopp W (1990) Chronic Lyme disease with an expansive granulomatous lesion in the cerebellopontine angle. Neurosurgery 27:446–451

    Article  PubMed  CAS  Google Scholar 

  78. Moon WJ, Roh HG, Chung EC (2009) Detailed MR imaging anatomy of the cisternal segments of the glossopharyngeal, vagus, and spinal accessory nerves in the posterior fossa: the use of 3D balanced fast-field echo MR imaging. AJNR Am J Neuroradiol 30:1116–1120

    Article  PubMed  Google Scholar 

  79. Mori K, Nishimura T (1995) Electrophysiological studies on brainstem function in patients with myelomeningocele. Pediatr Neurosurg 22:120–131

    Article  PubMed  CAS  Google Scholar 

  80. Mukherjee KK, Banerji D, Sharma R (1999) Pineal cyst presenting with intracystic and subarachnoid haemorrhage: report of a case and review of the literature. Br J Neurosurg 13:189–192

    Article  PubMed  CAS  Google Scholar 

  81. Murphy TP (1991) Otologic manifestations of pseudotumor cerebri. J Otolaryngol 20:258–261

    PubMed  CAS  Google Scholar 

  82. Nadkarni TD, Menon RK, Shah AH, Goel A (2008) Chudley McCullough syndrome. Childs Nerv Syst 24:541–544

    Article  PubMed  Google Scholar 

  83. Nakaya M, Morita I, Horiuchi M (2005) Recurrent hearing loss after myelography treated with epidural blood patch. Auris Nasus Larynx 32:399–401

    Article  PubMed  Google Scholar 

  84. Nishimura T, Mori K, Uchida Y, Ohira T, Tamura K (1991) Brain stem auditory-evoked potentials in meningomyelocele: natural history of Chiari II malformations. Childs Nerv Syst 7:316–326

    Article  PubMed  CAS  Google Scholar 

  85. Ohyama K, Salt AN, Thalmann R (1988) Volume flow rate of perilymph in the guinea-pig cochlea. Hear Res 35:119–129

    Article  PubMed  CAS  Google Scholar 

  86. Patra K, Wilson-Costello D, Taylor HG, Mercuri-Minich N, Hack M (2006) Grades I-II intraventricular hemorrhage in extremely low birth weight infants: effects on neurodevelopment. J Pediatr 149:169–173

    Article  PubMed  Google Scholar 

  87. Patwardhan RV, Nanda A (2005) Implanted ventricular shunts in the United States: the billion-dollar-a-year cost of hydrocephalus treatment. Neurosurgery 56:139–144, discussion 144–5

    PubMed  Google Scholar 

  88. Paul KS, Lye RH, Strang FA, Dutton J (1983) Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58:183–187

    Article  PubMed  CAS  Google Scholar 

  89. Peters TA, Monnens LA, Cremers CW, Curfs JH (2004) Genetic disorders of transporters/channels in the inner ear and their relation to the kidney. Pediatr Nephrol 19:1194–1201

    Article  PubMed  Google Scholar 

  90. Pogodzinski MS, Shallop JK, Sprung J, Weingarten TN, Wong GY, McDonald TJ (2008) Hearing loss and cerebrospinal fluid pressure: case report and review of the literature. Ear Nose Throat J 87:144–147

    PubMed  Google Scholar 

  91. Rask-Andersen H, Stahle J, Wilbrand H (1977) Human cochlear aqueduct and its accessory canals. Ann Otol Rhinol Laryngol Suppl 86:1–16

    PubMed  CAS  Google Scholar 

  92. Round R, Keane JR (1988) The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology 38:1461–1464

    Article  PubMed  CAS  Google Scholar 

  93. Rubin RC, Hochwald GM, Tiell M, Epstein F, Ghatak N, Wisniewski H (1976) Hydrocephalus: III. Reconstitution of the cerebral cortical mantle following ventricular shunting. Surg Neurol 5:179–183

    PubMed  CAS  Google Scholar 

  94. Rudnick E, Sismanis A (2005) Pulsatile tinnitus and spontaneous cerebrospinal fluid rhinorrhea: indicators of benign intracranial hypertension syndrome. Otol Neurotol 26:166–168

    Article  PubMed  Google Scholar 

  95. Russell SM, Hoffman R, Jafar JJ (2001) Improvement of chronic hearing loss after shunt revision. A case report. Surg Neurol 56:185–188

    Article  PubMed  CAS  Google Scholar 

  96. Salem A, Krainik A, Helias A, Bouccara D, Gaillard S, Feydy A et al (2002) MRI findings in a case of a superficial siderosis associated with an ependymoma. J Neuroradiol 29:136–138

    PubMed  CAS  Google Scholar 

  97. Sammons VJ, Jacobson E, Lawson J (2009) Resolution of hydrocephalus-associated sensorineural hearing loss after insertion of ventriculoperitoneal shunt. J Neurosurg Pediatr 4:394–396

    Article  PubMed  Google Scholar 

  98. Scheibe F, Haupt H, Bergmann K (1984) On sources of error in the biochemical study of perilymph (guinea-pig). Arch Otorhinolaryngol 240:43–48

    Article  PubMed  CAS  Google Scholar 

  99. Schiffer P, Podvinec M, Hasler P (2006) Hearing loss after lumbar myelography. Praxis (Bern 1994) 95:855–857

    Article  CAS  Google Scholar 

  100. Schuknecht HF (1963) Meniere’s disease: a correlation of symptomatology and pathology. Laryngoscope 73:651–665

    Article  PubMed  CAS  Google Scholar 

  101. Schurr PH, McLaurin RL, Ingraham FD (1953) Experimental studies on the circulation of the cerebrospinal fluid and methods of producing communicating hydrocephalus in the dog. J Neurosurg 10:515–525

    Article  PubMed  CAS  Google Scholar 

  102. Sekiya T, Moller AR, Jannetta PJ (1986) Pathophysiological mechanisms of intraoperative and postoperative hearing deficits in cerebellopontine angle surgery: an experimental study. Acta Neurochir (Wien) 81:142–151

    Article  CAS  Google Scholar 

  103. Sekiya T, Canlon B, Viberg A, Matsumoto M, Kojima K, Ono K et al (2009) Selective vulnerability of adult cochlear nucleus neurons to de-afferentation by mechanical compression. Exp Neurol 218:117–123

    Article  PubMed  Google Scholar 

  104. Sismanis A (1987) Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management. Laryngoscope 97:1–17

    Article  PubMed  CAS  Google Scholar 

  105. Sismanis A, Callari RH, Slomka WS, Butts FM (1990) Auditory-evoked responses in benign intracranial hypertension syndrome. Laryngoscope 100:1152–1155

    PubMed  CAS  Google Scholar 

  106. Spector GJ, Pettit WJ, Davis G, Strauss M, Rauchbach E (1978) Fetal respiratory distress causing CNS and inner ear hemorrhage. Laryngoscope 88:764–784

    PubMed  CAS  Google Scholar 

  107. Sperling NM, Franco RA, Milhorat TH (2001) Otologic manifestations of Chiari I malformation. Otol Neurotol 22:678–681

    Article  PubMed  CAS  Google Scholar 

  108. Spirakis SE, Hurley RM (2003) Unilateral hearing loss in children with shunt-treated hydrocephalus. J Am Acad Audiol 14:510–517

    Article  PubMed  Google Scholar 

  109. Stephensen H, Tisell M, Wikkelso C (2002) There is no transmantle pressure gradient in communicating or noncommunicating hydrocephalus. Neurosurgery 50:763–771, discussion 771–3

    Article  PubMed  Google Scholar 

  110. Sterkers O, Ferrary E, Amiel C (1988) Production of inner ear fluids. Physiol Rev 68:1083–1128

    PubMed  CAS  Google Scholar 

  111. Stoeckli SJ, Bohmer A (1999) Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report. J Neurosurg 90:773–775

    Article  PubMed  CAS  Google Scholar 

  112. Strahle J, Garton HJ, Maher CO, Muraszko KM, Keep RF, Xi G (2012) Mechanisms of hydrocephalus after neonatal and adult intraventricular hemorrhage. Transl Stroke Res 3:25–38

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  113. Stratton RF, Bluestone DL (1991) Oto-palatal-digital syndrome type II with X-linked cerebellar hypoplasia/hydrocephalus. Am J Med Genet 41:169–172

    Article  PubMed  CAS  Google Scholar 

  114. Swartz JD, Mukherji SK (2011) The inner ear and osteodystrophies. In: Swartz JD, Loevner LA (eds) Imaging of the Temporal Bone, 4th edn. Thieme, New York, pp 298–411

    Google Scholar 

  115. Swigonski N, Shallop J, Bull MJ, Lemons JA (1987) Hearing screening of high risk newborns. Ear Hear 8:26–30

    Article  PubMed  CAS  Google Scholar 

  116. Tandon PN, Sinha A, Kacker SK, Saxena RK, Singh K (1973) Auditory function in raised intracranial pressure. J Neurol Sci 18:455–467

    Article  PubMed  CAS  Google Scholar 

  117. Todt I, Mazereeuw-Hautier J, Binder B, Willems PJ (2009) Dandy-Walker malformation in patients with KID syndrome associated with a heterozygote mutation (p.Asp50Asn) in the GJB2 gene encoding connexin 26. Clin Genet 76:404–408

    Article  PubMed  CAS  Google Scholar 

  118. van Veelen-Vincent ML, Delwel EJ, Teeuw R, Kurt E, de Jong DA, Brocaar MP et al (2001) Analysis of hearing loss after shunt placement in patients with normal-pressure hydrocephalus. J Neurosurg 95:432–434

    Article  PubMed  Google Scholar 

  119. Vandam LD, Dripps RD (1956) Long-term follow-up of patients who received 10,098 spinal anesthetics; syndrome of decreased intracranial pressure (headache and ocular and auditory difficulties). J Am Med Assoc 161:586–591

    Article  PubMed  CAS  Google Scholar 

  120. Venkataramana NK, Satishchandra P, Hegde AS, Reddy GN, Das BS (1988) Evaluation of brainstem auditory evoked responses in congenital hydrocephalus. Childs Nerv Syst 4:334–338

    Article  PubMed  CAS  Google Scholar 

  121. Walsted A, Salomon G, Olsen KS (1991) Low-frequency hearing loss after spinal anesthesia. Perilymphatic hypotonia? Scand Audiol 20:211–215

    Article  PubMed  CAS  Google Scholar 

  122. Walsted A, Garbarsch C, Michaels L (1994) Effect of craniotomy and cerebrospinal fluid loss on the inner ear. An experimental study. Acta Otolaryngol 114:626–631

    Article  PubMed  CAS  Google Scholar 

  123. Walsted A, Nielsen OA, Borum P (1994) Hearing loss after neurosurgery. The influence of low cerebrospinal fluid pressure. J Laryngol Otol 108:637–641

    Article  PubMed  CAS  Google Scholar 

  124. Walsted A (2000) Effects of cerebrospinal fluid loss on hearing. Acta Otolaryngol Suppl 543:95–98

    Article  PubMed  CAS  Google Scholar 

  125. Wlodyka J (1978) Studies on cochlear aqueduct patency. Ann Otol Rhinol Laryngol 87:22–28

    Article  PubMed  CAS  Google Scholar 

  126. Worley G, Erwin CW, Schuster JM, Park Y, Boyko OB, Griebel ML et al (1994) BAEPs in infants with myelomeningocele and later development of Chiari II malformation-related brainstem dysfunction. Dev Med Child Neurol 36:707–715

    Article  PubMed  CAS  Google Scholar 

  127. Yamamoto M, Teranishi M, Naganawa S, Otake H, Sugiura M, Iwata T et al (2010) Relationship between the degree of endolymphatic hydrops and electrocochleography. Audiol Neurootol 15:254–260

    Article  PubMed  Google Scholar 

  128. Yanagita N, Koide J, Yokoi H (1988) Morphological changes in the cochlear aqueduct following herpes simplex virus inoculation into the subarachnoid space. Acta Otolaryngol Suppl 456:106–110

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, University of Minnesota, D429 Mayo Memorial Building, 420 Delaware Street, SE, MMC 96, Minneapolis, MN, 55455, USA

    David Satzer & Daniel J. Guillaume

Authors
  1. David Satzer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniel J. Guillaume
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Daniel J. Guillaume.

Additional information

Comments

Yusuf Izci, Ankara, Turkey

Hydrocephalus is an important clinical condition in children and adults. Symptoms vary with age of the patient, cause and type of hydrocephalus, and tolerance of the patient to the size of ventricles. Recognition of these symptoms is imperative for an accurate diagnosis. Hearing loss (HL) is not a classical symptom of hydrocephalus and this disorder is not usually considered in the differential diagnosis of HL. In the recent years, scientists investigated the relationship between altered cerebrospinal fluid (CSF) dynamics and abnormal auditory functions in order to reveal the mechanism of HL in hydrocephalus.

The aim of this article is to make a systematic review on the mechanisms of HL in hydrocephalus. To achieve this goal, the authors discussed the theories and basic science evidence regarding HL in disorders of CSF circulation. They also provided an overview of reports of HL in patients with CSF hypotension or hypertension not resulting from hydrocephalus and they finally focused on auditory dysfunction in hydrocephalus and the effects of CSF shunting.

This is an important paper helping to understand the possible mechanisms of hearing loss in hydrocephalus. Although the coexistence of hydrocephalus and hearing disorder is explained anatomically and physiologically and a number of mechanisms of pressure homeostasis between the cochlear fluids and the CSF has been summarized in this article, the complete picture of this coexistence remains poorly understood.

The targets of this article are the neurosurgeons who perform surgeries for hydrocephalus, neurologists, audiologists, and otolaryngologists who deal with the differential diagnosis of HL. This paper showed that the patients with hydrocephalus should be evaluated with an audiogram because HL may especially be overlooked in infants and young children, in whom self-reporting is not trustworthy and hearing is critical for their development.

Based on this review, hydrocephalus should be on the list of differential diagnoses of HL. Suspicion should be particularly high in young patients, who are more likely to have patent cochlear aqueducts, and in patients who admit with accompanying headaches. An ophthalmological examination for papilledema and imaging study with computed tomography or magnetic resonance imaging will be useful in establishing the diagnosis of hydrocephalus in patients with unexplained HL.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Satzer, D., Guillaume, D.J. Hearing loss in hydrocephalus: a review, with focus on mechanisms. Neurosurg Rev 39, 13–25 (2016). https://doi.org/10.1007/s10143-015-0650-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-015-0650-2

Keywords

Search

Navigation