Temporal bone histopathology 14 years after cytomegalic inclusion disease: a case study.

Abstract

Temporal bones were examined from a 14-year-old male who died of sequelae of congenital cytomegalic inclusion disease (CID). Cytomegalovirus (CMV) was not isolated from inner ear fluid or multiple systemic tissues at the time of death. Examination of temporal bones revealed chronic pathology of both cochlear and vestibular sensory and nonsensory tissues. Endolymphatic hydrops was observed in the basal turn of the cochlear duct, while Reissner's membrane was collapsed in the more apical turns. Strial atrophy and a loss of cochlear hair cells were observed along the entire length of the basilar membrane. Vestibular neuroepithelial regions were degenerated and fibrosis was seen within the vestibular perilymphatic tissue spaces, suggesting prior labyrinthitis within the perilymph compartment in addition to the more typical pattern of endolabyrinthitis associated with human CMV infection. Distention of the saccular membrane was evident. In both cochlear and vestibular tissues, there were isolated regions of calcifications that appeared characteristic to that reported in other organ systems of individuals with CID. Collectively, these chronic, pathological findings in this case of CID demonstrate more extensive injury than has been identified in the previously reported acute temporal bone pathology of CID.