Cochlear dysplasia is a rare congenital anomaly. However, early detection of this anomaly can prevent serious consequences. We describe three cases of cochlear dysplasia that presented with recurrent meningitis and cerebro-spinal fluid (CSF) leak in children in which early diagnosis prevented further complications.
This is a preview of subscription content, log in to check access.
Buy single article
Instant access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Arellano B, Ramirez Camacho R, Garcia Berrocal JR, Villamar M, del Castillo I, Moreno F (2000) Sensorineural hearing loss and Mondini dysplasia caused by a deletion at locus DFN3. Arch Otolaryngol Head Neck Surg 126:1065–1069
Bergstrom L, Thompson P, Wood RP 2nd (1979) New patterns in genetic and congenital otonephropathies. Laryngoscope 89:177–194
Dodge PR, Davis H, Feigin RD, Holmes SJ, Kaplan SL, Jubelirer DP, Stechenberg BW, Hirsh SK (1984) Prospective evaluation of hearing impairment as a sequela of acute bacterial meningitis. N Engl J Med 311:869–874
Eisenberg LS, Luxford WM, Becker TS, House WF (1984) Electrical stimulation of the auditory system in children deafened by meningitis. Otolaryngol Head Neck Surg 92:700–705
Graham JM, Phelps PD, Michaels L (2000) Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity. J Laryngol Otol Suppl 25:1–14
Jackler RK, Luxford WM, House WF (1987) Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 97:2–14
Melnick M, Bixler D, Nance WE, Silk K, Yune H (1976) Familial branchio-oto-renal dysplasia: a new addition to the branchial arch syndromes. Clin Genet 9:25–34
Mondini C (1791) Anatomica surdi nati sectio. Bonosiensi scientarium et atrium instituto atqu academia commentarii Bononiae 7:419–428
Ohtani I, Kano M, Sagawa Y, Ogawa H, Suzuki C (2001) Temporal bone histopathology in trisomy 22. Int J Pediatr Otorhinolaryngol 59:137–141
Paparella MM (1980) Mondini’s deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 89:1–10
Phelps PD (1986) Congenital cerebrospinal fluid fistulae of the petrous temporal bone. Clin Otolaryngol Allied Sci 11:79–92
Phelps PD, King A, Michaels L (1994) Cochlear dysplasia and meningitis. Am J Otol 15:551–557
Propst EJ, Blaser S, Gordon KA, Harrison RV, Papsin BC (2005) Temporal bone findings on computed tomography imaging in branchio-oto-renal syndrome. Laryngoscope 115:1855–1862
Saito R, Takata N, Matsumoto N, Koide I, Fujita A, Ogura Y, Murakami M, Yanagida KK, Omazawa M (1982) Anomalies of the auditory organ in Potter’s syndrome. Histopathological findings in the temporal bone. Arch Otolaryngol 108:484–488
Usami S, Abe S, Weston MD, Shinkawa H, Van Camp GK, Imberling WJ (1999) Non-syndromic hearing loss associated with enlarged vestibular aqueduct is caused by PDS mutations. Hum Genet 104:188–192
About this article
Cite this article
Ben-Shoshan, M., DeRowe, A., Grisaru-Soen, G. et al. Recurrent meningitis and cerebrospinal fluid leak–two sides of the same vestibulocochlear defect: report of three cases. Eur J Pediatr 166, 269–272 (2007). https://doi.org/10.1007/s00431-006-0236-7
- Cochlear dysplasia
- Mondini malformation
- CSF leak