Mondini dysplasia and congenital cytomegalovirus infection

doi:10.1016/S0022-3476(94)70256-X

The Journal of Pediatrics

Volume 124, Issue 1, January 1994, Pages 71-78

https://doi.org/10.1016/S0022-3476(94)70256-X Get rights and content

Abstract

We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and co-workers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone. (J PEDIATR 1994;124:71-8)

Section snippets

CASE REPORT

A 1750 gm female infant was the product of a 32-week gestation in a 23-year-old mother (gravida 4, para 2, aborta 1). The pregnancy was complicated by intermittent vaginal spotting and premature labor suppressed with tocolytic agents. The mother reportedly was not immune to rubella. The infant's Apgar scores were 1 at 1 minute and 7 at 5 minutes. Examination of the infant at birth revealed petechiae without hepatosplenomegaly, head circumference of 30 cm, appropriate size for gestational age,

METHODS

To investigate the association between congenital CMV infection and temporal bone anomalies, we studied six children (including the index case) born between 1982 and 1989. All subjects had evidence of symptomatic congenital CMV infection, as established by a combination of clinical features, viral cultures, and serologic studies. Children were recruited from 20 patients with proved or presumed symptomatic congenital CMV infection (in 11, CMV was detected in urine obtained in the first 3 weeks

General findings

In three children (subjects 1, 4, 6), CMV infection was confirmed within the first 3 weeks of life by isolation of the virus from urine. The diagnosis in the remaining cases was highly suspected on the basis of the clinical examination, exclusion of other congenital infections by serologic studies, and CMV isolation at 3 months (subject 2), 4 months (subject 5), and 12 months (subject 3). The mother of subject 3 may have had chickenpox in her twentieth week of pregnancy, but results of the

DISCUSSION

Temporal bone anomalies result from teratogenic insults or hereditary factors affecting the developing labyrinth. The most severe anomalies, attributed to early gestational insults, are typically accompanied by severe SNHL. Insults occurring later in gestation cause less severe dysplasia and SNHL because the already-formed otic structures are less vulnerable to teratogenic agents. Because the severity of temporal bone anomalies is related to the stage of embryogenesis at the time of the insult,

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^☆: From the Departments of Otolaryngology, Pediatrics, Radiology, and Neurology, University of Iowa College of Medicine, Iowa City

^☆☆: Supported by grants from the Deafness Research Foundation and the Children's Miracle Network Telethon Research Fund of the Iowa Children's Center.

^★: Reprint requests: James F. Bale, Jr., MD, Department of Pediatrics, Room 2504 JCP, University of Iowa Hospitals, 200 Hawkins Dr., Iowa City, IA 52242-1083.

^★★: 0022-3476/94/$1.00 + .10 9/20/50953

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Mondini dysplasia and congenital cytomegalovirus infection☆,☆☆,★,★★

Abstract

Section snippets

CASE REPORT

METHODS

General findings

DISCUSSION

Pediatr Clin North Am

Am J Otolaryngol

Symptomatic congenital cytomegalovirus infection: neonatal morbidity and mortality

Pediatr Infect Dis J

Outcome of symptomatic congenital cytomegalovirus infection: results of long-term longitudinal follow-up

Pediatrics

Symptomatic congenital cytomegalovirus: disorders of language, learning, and hearing

Am J Dis Child

The outcome in children with congenital cytomegalovirus infection

Am J Dis Child

Auditory and visual defects resulting from symptomatic and subclinical congenital cytomegaloviral and toxoplasma infections

Pediatrics

Progressive hearing loss in infants with asymptomatic congenital CMV infection

Pediatrics

Hearing impairments and vestibular abnormalities among children with subclinical cytomegalovirus

Ann Otol Rhinol Laryngol

Cytomegalovirus labyrinthitis in an infant: morphological, virological and immunofluorescent studies

J Pathol Exp Neurol

Cytomegalovirus endolabyrinthitis

Arch Pathol Lab Med