International Journal of Pediatric Otorhinolaryngology, 22 (1991) 39-47

0 1991 Elsevier Science Publishers B.V. 016%5876/91/$03.50

PEDOT

39

00723

Pregnancy-induced

hypertension
hearing loss
Meher

and congenital

D. Wells

William Hawey Hospital, Ashford, Kent (U.K.) and UniL1ersit.v

College and Middlesex School of Medicine,
Institute of Laryngology and Otoloa, London (iY.K)

(Revised

Key words: Deafness;

Congenital;

(Received 21 June 1990)

version received 25 January
(Accepted 30 January 1991)

Maternal

hypertension

1991)

(pre-eclampsia)

Abstract

It has been suggested that pregnancy-induced

hypertension (PIH - preeclampsia or toxaemia of pregnancy) may cause sensorineural hearing loss (SNHL)
in the offspring. To establish the validity of this concept a clinical survey of the
prevalence of congenita1 hearing Ioss in relation to PIH in the South East Kent
Health District in the United Kingdom over a period of 4 years was undertaken.
Description of the temporal bones in a case of PIH is presented. The total number
of live births in this period was 12,927, out of which 512 mothers (3.9%) were
diagnosed as having PIH. To date 17 cases of bilateral SNHL have been diagnosed
in this period (excluding known syndromes, conductive hearing loss and unilateral
SNHL). One of the mothers of these children had PIH. It is possible that
otologists, in the absence of any obvious cause, have attributed the cause of
bilateral SNHL to PIH. Histopathological
findings in temporal bones from a
29-week fetus, whose cause of death was severe maternal hypertension, showed
massive haemorrhages in the inner ear and middle ear and internal auditory
meatus, a frequent finding in temporal bones obtained at autopsy from fetuses and
neonates who were born prematurely. This study suggests that PIH per se is
unlikely to cause SNHL in the newborn.

Correspondence: M.D. Wells,

Kent TN24 OLZ, U.K.

Dept.

of ENT,

The William

Harvey

Hospital,

Willesborough,

Ashford,

40

Introduction
Pregnancy-induced hypertension, also known as pre-eclampsia, is the development of hypertension with proteinuria, oedema or both after the twentieth week of
gestation or occasionally earlier. The complication of PIH by convulsions is called
eclampsia. The aetiology of PIH remains unknown and its pathophysiology is
incompletely understood.
Although the association of PIH with congenital hearing loss has been mentioned, neither the prevalence of children with sensorineural hearing loss attributed to maternal PIH, nor its relationship to the severity of PIH is clear. To
attempt answering these questions all children in the South East Kent Health
District (SEKHD) born from 1st January 1985 to 31st December 1988 were
surveyed to determine the prevalence of sensorineural hearing loss (SNHL) in
children whose mothers had PIH and of SNHL due to all other causes. The
temporal bone findings of a fetus who died in utero from maternal PIH are also
presented.

Material and methods

SEKHD comprises a population of 256,000. Accurate obstetric registers with
complete patient details are available in the district from January 1985. The total
number of live births in 1985, 1986, 1987 and 1988 were 3110,3189, 3266 and 3362
respectively. A diagnosis of PIH was made in 122 (3.9%) women in 1985, 123
(3.8%) in 1986, 143 (4.3%) in 1987 and 133 (3.1%) in 1988. The mean prevalence
of PIH in SEKHD was 3.9%. There was one fetal and maternal death at 40 weeks
gestation due to PIH during this period in SEKHD. The fetus died in utero during
labour; at autopsy that fetus had no congenital anomalies and there was no
evidence of intracranial haemorrhage.
All children were routinely screened by trained Health Visitors for hearing loss
between the ages of 6 to 8 months and then at the ages of 1 and 3 years, in
conjunction with assessment of speech, language and general development. Suspicion of hearing loss at the initial examination and/or delay in speech development
was followed by referral to the specialist team consisting of the Senior Medical
Officer for deaf children and the visiting teacher of the deaf. When indicated the
patient was seen by the otologist for further investigations, treatment and/or
rehabilitation. At risk neonates including preterm babies with complications, those
with Rh incompatibility, maternal rubella and other infections were referred
directly by the paediatricians to the otologist.

Results
During the 4-year period included in the clinical survey there were only 17 cases
of bilateral congenital sensorineural hearing loss; of these 9 children had a

41

hereditary SNHL, in 4 no obvious aetioIogy was found and one child was a preterm
birth at 31 weeks of gestation who had intraventricular haemorrhage. One child
was a preterm birth at 29 weeks of gestation, delivered by emergency caesarean
section for hand presentation and fetal distress and was also anoxic at birth and
one child had cerebral palsy. Known syndromes, unilateral SNHL and congenital
conductive hearing loss were excluded. Only one child had sensorineural hearing
loss whose mother had PIH and congenital sensorineural hearing loss. The onset
of labour was at 36 weeks of gestation, emergency caesarean section was performed for fetal distress. The neonate was asphyxiated at birth.

Case report
Temporal bone findings in pregnancy-induced

hypertension

This mother was admitted at 29 weeks of gestation with a uterus that was small
for dates. The fundus was 27 weeks in height. The mother showed the features of
PIH; she was oedematous, had proteinuria and her blood pressure was 225/145
mmHg. She was given antihypertensive drugs to reduce the blood pressure, but
went into premature labour. The fetal heart ceased to be heard during labour and
she was delivered of a fresh stillbirth.

Fig. 1. Horizontal section of the right temporal bone showing haemorrhage (h) and embryonic
mesenchyme (em) in the middle ear. External auditory meatus (earn), malleus Cm), incus (i), chorda
tympani nerve (ct) and facial nerve (fn).

42

Fig. 2. Mid modiolar section of the right temporal bone showing haemorrhage in the Scala vestibuli (v),
Scala tympani (t), modiolus and internal auditory meatus (arrows), cochlear (c) and vestibular (vt)
divisions of the auditory nerve.

Postmortem
Postmortem was performed 48 h after death. The body weight was 1005 g and
the crown/heel
length was 39 cm. The skull was normal. There were large
extravasations of blood into the subarachnoid space over both temporal lobes and
the cerebellum including the cistema magna. The tentorium cerebelli was intact
and the brain substance was healthy. The right side of the heart was dilated and
the right kidney showed malposition. The left kidney was normal in place and all
other organs were normal. Intrauterine death was thought to be due to maternal
PIH of marked severity.
Histopathology
Right temporal bone, Both temporal bones from this stillborn infant were
removed at autopsy, decalcified and embedded in celloidin. Horizontal serial
sections (20 pm) were cut and one section in every 10 was stained with haematoxylin and eosin.
Development of the temporal bone was consistent with 29 weeks gestation, The
middle ear contained a moderate amount of embryonic mesenchyme with massive
haemorrhage. The Eustachian tube was filled with pink-stained fluid. The scalae
vestibuli and tympani also contained similar fluid with haemorrhage. Degenerative
changes were seen in the stria vascularis and organ of Corti in all the cochlear
turns. The spiral ganglion cells appeared normal in number. These pathological
changes in the membranous structures may be largely attributed to autolysis as

Fig. 3. High-power

view of part of Fig. 2, showing haemorrhage

in modiolus and internal
meatus (arrows), cochlear division Cc) of the auditory nerve.

Fig. 4. High-power

view of part of Fig. 2 showing

haemorrhage

(h) in the scala tympani.

auditory

44

Fig. 5. High-power view of part of Fig. 2 showing haemorrhage (h) in the scala media.

Fig: 6. High-power view of part of Fig. 2 showing haemorrhage (h) in the modiolus.

postmortem was performed 48 h after death. Haemorrhage was also seen in the
Scala media, the modiolus and internal auditory meatus. Pink-staining fluid was
also present in the endolymphatic space of the lateral semicircular canal (Figs.
l-6).
Left temporal bone. Histopathological findings in the left temporal bone were
similar to those of the right side.

Discussion
Pregnancy complicated by PIH may result in a normal full-term delivery and a
normal healthy neonate. It may also result in spontaneous premature birth as seen
in one case in this survey and in some cases may necessitate premature induction
of labour. Some cases of severe PIH may result in the death of the fetus as seen in
the case described here. Raymond et al. [3] have found that deaths of neonates
whose mothers had PIH were due to complications of prematurity. There was no
evidence of intrauterine or intrapartum asphyxia found in these neonates. A study
of 18 infants by auditory brainstem evoked responses did not show any difference
between babies after normal pregnancies compared with those after hypertensive
pregnancies [l]. Oxford clinical studies have shown that at the age of 4 years
health, weight, prevalence of sight, hearing and speech problems did not differ in
children whose mothers were receiving specific treatment with methyldopa for PIH
or from mothers who were receiving no treatment for PIH and a sample in whom
mothers did not have PIH. The first two groups of children were further studied
for ill health, impaired vision and hearing, weight, height, learning and reading
ability at the age of 7.5 years; no adverse effect was observed on the development
of these children [2].
The majority of neonates in intensive care units are preterm and many of the
survivors have major neurological and developmental handicaps including hearing
and visual defects which are often the sequelae of intracranial haemorrhage. These
may be subdural, subarachnoid, intraventricular and intracerebellar. There was
massive haemorrhage in the middle and inner ear, internal auditory meatus and
subarachnoid haemorrhage in the case described, which is the first description of
pathological changes in the temporal bones from PIH-associated fetal death.
Similar temporal bone findings are seen in premature neonates who have died
from complications like intracranial haemorrhage and asphyxia.
Spector et al. [5] have studied 52 consecutive temporal bones from infants who
died neonatally or in utero of natural causes. Complete autopsies were performed.
Twenty-eight infants had a variety of pulmonary disorders which had resulted in
respiratory distress prior to their death. Of these the majority had bleeding
intracranially and into the inner ear. They suggest that the pathway of haemorrhage from subarachnoid haemorrhage involving the inner ear included the modiolus or the cochlear aqueduct, or that it was retrograde via the cochlear veins, or
that spontaneous bleeding occurred in the various compartments of the inner ear
and otic capsule. Volpe [7] has extensively discussed the pattern of physiology and

46

neuropathology
and the clinical features of intracranial haemorrhage in the
neonatal period. He has suggested that the two main pathogenic factors are
hypoxia and trauma. Seventy-five percent of neonates with primary subarachnoid
haemorrhage are seen among those who are born prematurely. Towbin [6], in an
analysis of 120 cases, showed that cerebellar and intraventricular haemorrhage was
common in infants born between 22 and 35 weeks gestation and that prevalence
was highest in the group born at 28 weeks gestation. A frequent finding in the
maternal history was episodes of vaginal bleeding and PIH in the period prior to
parturition. Prematurity, hypoxia and systemic circulatory failure were considered
to be contributing factors. Examination of the temporal bones was not carried out
in that study. There was no evidence of intracranial haemorrhage in the fetus who
died in utero at 40 weeks gestation (SEKHD case). The temporal bones were not
examined in that case.
Haemorrhage
in the temporal bones of premature neonates may also be
associated with hypoxia, maternal viral infection and postnatal infection. In a study
of 174 temporal bones (108) from fetuses, neonates, spontaneous abortions and
stillbirths, out of which only 7 cases were born at or after 37 weeks gestation,
haemorrhages in the inner ear and internal auditory canal were seen in 38% of the
cases [S]. Intracranial haemorrhage, hypoxia, infection and birth trauma were the
most frequently associated factors. It thus seems likely that the histopathological
changes observed in the fetus described here, who died in utero at 29 weeks
gestational age in association with maternal PIH, are in keeping with prematurity
and intracranial haemorrhage. Blood in the inner ear fluids may cause biochemical
changes leading to degeneration of the hair cells of the organ of Corti and hearing
loss, although in the present case degeneration of the organ of Corti was largely
due to postmortem autolysis. Electron microscopic studies of the organ of Corti
have shown degeneration of the hair cells caused by haemorrhages in the inner ear
in a case with intracranial haemorrhage [4].
In the absence of any other cause, otologists may tend to attribute sensorineural
hearing loss in young children to PIH. Hearing loss in such cases may, however, be
inherited on an autosomal recessive basis and not be related to PIH. PIH may
precipitate premature labour or may necessitate premature induction of labour,
leading to the problems inherent in prematurity (hypoxia, intracranial haemorrhage and other factors) which may cause a sensorineural hearing loss. In this
clinical survey of 512 mothers who had PIH only one child had a sensorineural
hearing loss, which could be attributed to prematurity, fetal distress and birth
asphyxia and heredity, so that the prevalence of such a direct association must be
very low, if it exists at all.

Conclusion
At present PIH per se should not be regarded as a significant cause of acquired
congenital sensorineural hearing loss, unless it has resulted in premature birth
complicated by intracranial haemorrhage or severe anoxia.

Acknowledgements
My thanks are due to Mr. A. Frohlich for his expert help in preparing
histopathological sections and also to Miss S. Dick for typing the manuscript.

the

References
1 Diven, D.G., Vertes, D. and Kelly, R., Effects of hypertension in pregnancy on neurological
maturation in neonates, Am. J. Obstet. Gynecol., 153 (1985 709-710.
2 Ounsted, M., The children of women who had hypertension drliing pregnancy. In P.C. Rubin (Ed.),
Handbook of Hypertension, Vol. 10; Hypertension in Pregnancy, Elsevier, Amsterdam, 1988. pp.
341-362.
3 Raymond, P., Naden, M.D. and Redman, C.W.G., Antihypertensive drugs in pregnancy. In W.J.
Lamsback, M.D. Lindheimer and W.M. Baron (Eds.), Clinics in Perinatology, Vol. 12, No. 3.
Saunders, Philadelphia, 1985, pp. 521-538.
4 Slack, R.W.T., Wright, A., Michaels, L. and Frohlich, A., Inner hair cell loss and intra-cochlear clot
in the very preterm infant, Clin. Otolaryngol., 11 (1986) 443-446.
5 Spector, G.J., Pettit, W.J., Davis, G., Strauss, M. and Rauchback, E., Fetal respiratory distress
causing CNS and inner ear haemorrhage, Laryngoscope, 88 (1978) 764-786.
6 Towbin, A., Cerebral intraventricular haemorrhage and subependymal matrix infarction in the fetus
and premature newborn, Am. J. Pathol., 52 (1968) 121-140.
7 Volpe, J.J., Neonatal intracranial haemorrhage, pathophysiology, neuropathology and clinical features, Clin. Perinatol., 4 (1977) 77-108.
8 Wells, M.D., Clinico-pathological Study of the Fetal and Neonatal Ear, M.D. Thesis, University of
London, 1990.