Hereditary deafness with hydrops and anomalous calcium phosphate deposits

Lars Göran Johnsson, Roland C. Rouse, Joseph E. Hawkins, Thomas C. Kingsley, Charles G. Wright

Research output: Contribution to journalArticlepeer-review

15 Scopus citations


The temporal bones from a 58-year-old white woman who had had hereditary congenital deafness were examined with the techniques of microdissection and surface preparations followed by sectioning of the modiolus. There was bilateral, almost total sensorineural degeneration, which also involved the saccule. The degeneration of the distal processes of the cochlear neurons in the osseous spiral lamina was almost complete, whereas numerous ganglion cells and proximal processes remained in the modiolus and the internal auditory canal. Severe cochleo-saccular hydrops was present in the left ear with Reissner's membrane bulging into the horizontal canal. X-ray diffraction and electron probe analysis were used to study the abnormal crystalline deposits in both ears. On the left side the saccular otoconia were composed of calcite, but the utricular macula was covered by a crust of apatite spherulites. More apatite occurred around the maculae and in the scala media. The cupulae were petrified and were composed of apatite and octacalcium phosphate. On the right side the utricular otoconia were of normal calcite, but there was a deposit of apatite on the macula sacculi. The upper part of the scala media was completely filled by a deposit of apatite and octacalcium phosphate.

Original languageEnglish (US)
Pages (from-to)284-298
Number of pages15
JournalAmerican Journal of Otolaryngology--Head and Neck Medicine and Surgery
Issue number4
StatePublished - Sep 1981

ASJC Scopus subject areas

  • Otorhinolaryngology


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