Six selected cases with neonatal and fetal cochlear pathology are presented. Those denoting cochlear sepsis are: H. influenza purulent labyrinthitis, cytomegalovirus endolabyrinthitis, and aseptic meningitis and labyrinthitis. Those denoting cochlear neural aplasia are Goldenhar's syndrome, left-sided cardiac hypoplasia with partial basal turn neural aplasia, and cerebral cortical and ventricular hemorrhage with modiolar extension. These findings are compared to the fluorocitrate ototoxicity model for neural deafness in the guinea pig. This study suggests hypotheses for viral trophism in the inner ear and neural degeneration of the cochlea as mechanisms for sensorineural deafness.
Get full access to this article
View all access options for this article.
References
1.
McConnellEWardPH: Deafness in Childhood. Vanderbilt University Press, 1962, pp 69–169.
GacekR: The pathology of hereditary sensorineural hearing loss. Ann Otol Rhinol Laryngol80: 289–298, 1971.
9.
SchuknechtHF: Pathology of sensorineural deafness of genetic origin, in McConnellEWardPH, (eds): Deafness in Childhood. Vanderbilt University Press, 1962, chap 7.
10.
SchuknechtHFIgarashiMGacekRR: The pathological types of cochleo-saccular degeneration. Acta Otolaryngol59: 154, 1968.
11.
SchuknechtHF: Pathology of the Ear. Cambridge, MA: Harvard University Press, 1974, pp 388–403.
12.
SpectorGJCarrCD: Fluorocitrate ototoxicity: A morphologic and cytochemical model for primary neural degeneration in the guinea pig cochlea. Ann Otol Rhinol Laryngol85: 185–198, 1976.
13.
DavisGSpectorGJStraussM: Cytomegalovirus endolabyrinthitis. Arch Pathol, to be published.
AubreyMMorand: The epibulbar dermoid auricular appendix-preauricular fistula triad or Francheseetti-Goldenhar's syndrome. Ann Otolaryngol Chir Cervicofac81: 69–71, 1964.
16.
GoldenharM: Associations malformatives de l'oeil et l'oreille, en particulier le syndrome dermoide epibulbaire-appendices auri-culaires-fistula auris congenita et ses relations aves la depostose mandibulofacial. J Genet Humaine1: 243–283, 1952.
17.
NeimannNCordierJManeiauxM: Franchescetti's syndrome associated with Goldenhar's syndrome and with vertebral abnormalities. Ann Pediatr (Paris)7: 460, 1960.
18.
SmithillsRW: Oculo-auriculo-vertebral syndrome (Goldenhar's syndrome). Dev Med Child Neural6: 406, 1964.
19.
BuddenSSRobinsonGC: Oculoauricular vertebral dysplasia. Its association with sensorineural deafness and other abnormalities. Am J Dis Child125: 431–433, 1973.
20.
AndersonWAD: Pathology. St. Louis: C. V. Mosby, 1971, pp 725–726.
21.
KeithJD: Heart Diseases in Infancy and Childhood. New York: McMillan Co., 1967, pp 466–474.
22.
MeyersENStoolS: Cytomegalic inclusion diseases of the inner ear. Laryngoscope78: 1904–1915, 1968.
23.
DavisGL: Cytomegalovirus in the inner ear. Case report and electron microscopic study. Ann Otol Rhinol Laryngol78: 1179–1189, 1969.
24.
McCrackenGHJr.ShinefieldHRCobbK: Congenital cytomegalic inclusion disease. A longitudinal study of 20 patients. Am J Dis Child117: 522–529, 1969.
25.
ReynoldsDWStagnoSStubbsG: Inapparent congenital cytomegalovirus infection with elevated cord IgM levels. Casual relationship with auditory and mental deficiency. N Engl J Med290: 291–296, 1974.
26.
DahleAJMcCollisterFPHammerBA: Subclinical and congenital cytomegalovirus infection and hearing impairment. J Speech Hear Disord39: 320–329, 1974.
27.
ReynoldsDWStagnoSHostyJS: Maternal cytomegalovirus excretion and perinatal infection. N Engl J Med289: 1–5, 1973.
28.
KaneRCRousseauWENobleGR: Cytomegalovirus infection in a volunteer blood donor population. Infect Immun11: 719–723, 1975.
29.
YeagerAS: Transfusion-acquired cytomegalovirus infection in newborn infants. Am J Dis Child128: 478–483, 1974.
30.
DavisGLStraussM: Viral disease of a labyrinth. II. Ann Otol Rhinol Laryngol82: 584–593, 1973.
31.
FriedmannI: Cochlear pathology in viral disease. Adv Otorhinolaryngol20: 155–177, 1973.
WardPHKinneyCELindsayJR: Inner ear pathology in congenital deafness. Laryngoscope72: 435–455, 1962.
34.
SpectorGJLucenteFE: Aerobic metabolism of the inner ear. Results of a critical evaluation. Laryngoscope84: 1663–1672, 1974.
35.
SpectorGJ: The ultrastructural cytochemistry of lactic dehydrogenase, succinic dehydrogenase, dihydronicotinamide adenine dinucleotide diaphorase and cytochrome oxidase activities in hair cell mitochondria of the guinea pig cochlea. J Histochem Cytochem23: 216–234, 1975.
36.
SpectorGJCarrCDArenbergIK: Fluorocitrate neural dystrophy of the guinea pig cochlea. Proceedings 33rd Annual Proc Electron Microscopy Soc Amer, Las Vegas, Nevada, 1975. BaileyG. W. (ed).
37.
PetersRA: Mechanism of the toxicity of the active constituent of Dichapetalum cymosum and related compounds. Adv Enzymol18: 113–159, 1957.
38.
CarrellHLGluskerJPVillafrancaJJ: Fluorocitrate inhibition of aconitase: Relative configuration of inhibiting isomer by x-ray crystallography. Science170: 1412–1414, 1970.
39.
SpectorGJCarrCDArenbergIK: Fluorocitrate induced neural dystrophy in the guinea pig cochlea: An ultrastructural and cytochemical study. J Cell Biol67: 413a, 1975.
40.
SpectorGJCarrCD: The electron transport system in the cochlear hair cell: The ultrastructural cytochemistry of respiratory enzymes in hair cell mitochondria of the guinea pig. Laryngoscope84: 1673–1706, 1974.
41.
SpectorGJ: Fluorocitrate neurotoxicity of guinea pig cochlea: An ultrastructural cytochemical model of neural deafness. J Histochem Cytochem, to be published.
42.
KoenigH: Acute axonal dystrophy caused by fluorocitrate: The role of mitochondrial swelling. Science164: 310–312, 1969.
