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Acta Otorrinolaringol Esp. 2011;62(5):385---391

www.elsevier.es/otorrino

REVIEW ARTICLE

Vestibular Examination of Children With Alterations in Balance (II):

Results by Pathologies,
Beatriz Gonzlez del Pino,a Paola Femia,b Nicols Prez-Fernndezc,
a

Departamento de Otorrinolaringologa, Clnica Universitaria Reina Fabiola, Crdoba, Argentina

Departamento de Otorrinolaringologa, Hospital de Clnicas Jos de San Martn, Buenos Aires, Argentina
c
Departamento de Otorrinolaringologa, Clnica Universidad de Navarra, Pamplona, Spain
b

Received 8 October 2010; accepted 3 January 2011

KEYWORDS
Vertigo;
Dizziness;
Instability

PALABRAS CLAVE
Vrtigo;
Mareo;
Inestabilidad

Abstract We review the findings of vestibular examinations in children according to the disease. Just as in adults, the dizziness can be classified following a physiopathological scheme.
2010 Elsevier Espa
na, S.L. All rights reserved.

Exploracin vestibular de ni
nos con alteraciones del equilibrio (II): resultados por
enfermedades
Resumen En este trabajo se va a revisar el resultado de la exploracin vestibular en las
diversas formas de enfermedad que padecen los ni
nos. Al igual que en el adulto, las alteraciones
vestibulares pueden ser clasificadas siguiendo un esquema de corte fisiopatolgico.
2010 Elsevier Espa
na, S.L. Todos los derechos reservados.

Introduction
This article reviews the results of vestibular examination in
the various forms of the disease experienced by children. As

in adults, vestibular disorders can be classified according to

a physiopathological scheme.1

Otitis Media With Effusion

Children With Otitis Media With Effusion

Please cite this article as: Gonzlez del Pino B, et al. Exploracin
vestibular de ni
nos con alteraciones del equilibrio (II): resultados
por enfermedades. Acta Otorrinolaringol Esp. 2011;62:385---391.
This work was prepared for the Congress of the European Society of Pediatric Otolaryngology celebrated in 2010, in Pamplona,
Spain.
Corresponding author.
E-mail address: nperezfer@unav.es (N. Prez-Fernndez).

According to the findings of the Peabody Developmental

Motor Scale (PDMS), the most serious involvement occurs
in children with bilateral otitis media with effusion (OME),
whereas no significant differences are observed between
normal controls and children aged 1---4 years old with unilateral involvement. Surprisingly, there is no correlation
between the information given by parents about the balance

2173-5735/$ see front matter 2010 Elsevier Espa

na, S.L. All rights reserved.

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386
of children and the examination findings, as detailed below.
Differences exist both in the overall score and in some of the
test categories such as: (1) dynamic equilibrium in which
the childrens centres of mass move continuously in various directions (standing on one foot, grasping a toy), and
(2) locomotion tests in which children are asked to move
in order to carry out an activity.2 Consequently, these are
children with alterations in the acquisition of gross motor
requiring dynamic balance. The involvement is possibly due
to the fact that weight information for balance from the
inner ear is erroneous, which makes the child dependent
on visual and kinaesthetic information. The most interesting point is that receiving only some correct vestibular
information from the healthy ear is sufficient to correct
the deficit. This situation is reflected in the posturography
results obtained when maintaining children in a colourful
and stimulating visual environment: children with OME show
more balancing and destabilisation than normal controls.3
However, given the age of the children, there are no data for
a specific test that locates vestibular function (neither clinical tests, with cephalic impulse, nor instrumental ones, with
caloric or rotary test) that can ascertain the pathophysiological basis of vestibular damage. In fact, studies carried out
using electronystagmography (ENG) show no actual record of
caloric nystagmus. Instead, this is obtained of only spontaneous and positional nystagmus (with little or scarce locator
value), which shows highly pathological values in 58% of
children with otitis media.4 The need for an accurate assessment of this type is greater if we consider that the only study
carried out using rotational stimulation in children aged
4---9 years showed no significant differences in the findings of
children with OME with respect to those in normal controls.5

Children With Otitis Media With Effusion Before

and After Placement of Eardrum Drainage Tubes

B. Gonzlez del Pino et al.

These results were worse than in the control group9 and,
in the long term, this phenomenon became more evident
in the vestibulo-oculomotor reflex (VOR), which is clearly
hypometric in children who have suffered repeated OME.10
The ENG data did not provide information of interest in these
children, as they were frequently normal or the findings had
no locator value.11

Benign Paroxysmal Vertigo of Childhood

This form of disease, listed within the periodic syndromes
of childhood, is the most frequently diagnosed, regardless of the age of the child.12 Benign paroxysmal vertigo of
childhood (BPVC) is possibly a central syndrome, given the
examination findings during the intercritical phase: (1) when
confronted with an optokinetic (cortical) or visual-vestibular
interaction (subcortical) visual preference paradigm, the
children show an abnormal preponderance for the latter13 ;
(2) the dissociation in the response in vestibular evoked
myogenic potentials (VEMP) depending on the location of
registration and therefore on the stimulation of the inner
ear and the neurological pathway followed, since the ocular are usually conserved and the cervical show a significant
latency delay14 ; (3) the caloric test is normal in 75% of diagnosed children15 ; (4) both children with BPVC and children
with migraine show a similar behavioural disorder, indistinguishable but mild (emotional difficulty, anxiety, depression,
symptoms of hyperactivity), in the limit of normality and
(5) 33% of children with migraine without vestibular symptoms present vestibular function abnormalities, which are
therefore subclinical.16 Beyond the terminological confusion, the separation line between this form of vertigo and
the idiopathic benign paroxysmal positional form (BPPV) in
children is sometimes very tenuous.

Immune-mediated Inner Ear Disease

From the time of placement of the tubes, it is possible
to observe that both spontaneous nystagmus and positional
nystagmus disappear in 96% of children who showed this sign
before surgery.4 The static posturography of children with
bilateral OME shows a significant increase in the length and
area of balance, which is increased by 20% when modifying the study conditions, both closing the eyes and testing
on foam; the two alterations are not added together when
the alterations are combined. When the otitis is resolved,
the abnormal values of path followed and area generated
by the centre of pressures become normalised.6
Regarding the reliability of the information provided
by parents, 29% mentioned that their children had symptoms of instability, dizziness and frequent falls and that,
in general, they were more awkward than expected. However, 61% of children with OME showed a significantly worse
score than the normal population in the efficacy test or
Bruininks-Oseretsky test of motor proficiency (BOTMP), and
this occurred both in children with unilateral involvement
and with bilateral involvement, regardless of the degree of
hearing loss. More specifically, the score was worse in the
categories of balance, bilateral coordination, running speed
and agility.7 After the placement of the tubes, 90% of parents
mentioned a noticeable improvement in the stability of the
child, and the BOT2 become normalised in 86% of children.8

As in adults, immune-mediated inner ear disease (IMIED)

is very rare in children. The differential diagnosis of
localised forms is sudden and rapidly progressive hearing loss, and of systemic forms: Cogans disease, lupus
erythematosus, antiphospholipid syndrome, antiphospholipid syndrome, Sjgrens syndrome, Behc
ets disease,
Wegeners granulomatosis, idiopathic juvenile arthritis and
Hashimotos thyroiditis. In the few cases of sudden hearing
loss described, the vestibular function was normal according
to the caloric test result17 but, as in adults, the association
of vertigo to the episode of hearing loss adds a poor hearing
prognosis. Fluctuations in uneven vestibular function have
been described in Cogans disease: intense in the caloric and
rotatory tests and hardly any in VEMP tests.18 In all these
cases, the evolution of both hearing and balance should be
monitored.

Congenital or Acquired Hearing Loss

Children With Congenital or Acquired Hearing Loss
The children were divided into several groups according to
computerized dynamic posturography (CDP) results.

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Vestibular Examination of Children With Alterations in Balance (II): Results by Pathologies

1) With normal vestibular function: Children who showed
balance and motor development similar to that of children without hearing or vestibular deficit.
2) With vestibular deficits (most frequently bilateral
hypofunction): Children who showed few problems
in motor skills expect for balance and in whom
balancing was significantly higher in conditions requiring vestibular support (Conditions 5 and 6 of the
CDP).
3) With sensory organisation deficit and normal vestibular function: Children showing further deterioration in all measurements of motor skills including
balance.19

Such evident alterations of balance in Conditions 5 and 6

of the CDP reflect, among other things, a change in the coordination of muscle response in the lower extremities, where
it has been found that the response of the tibialis anterior
muscle is slower and more prolonged than in normal children
when they are subjected to a disturbance of the supporting
surface. Children in group 2 would benefit from a vestibular
rehabilitation plan that could work on several actions aimed
at maintaining balance in demanding environments and surface conditions, while children in group 3 would require a
global plan to tackle problems such as coordination and ability to solve conflicting sensory situations.20 This plan should
take into account the need to work on the visual acuity
deficits suffered by children with vestibular hypofunction
due to the profound alteration of the VOR that so intensely
affects the ability to read, which is significantly hindered as
a result.21

Children With Congenital or Acquired

Severe-Profound Hearing Loss
Tests conducted prior to the placement of a cochlear
implant: caloric test with ice water, rotatory test and VEMP.
In general terms, 85% of children with severe-profound
hearing loss present a certain degree of vestibular deficit.
Patients were divided into 4 groups: (1) normal vestibular function (15%), in which the response was normal in
all tests; (2) asymmetric caloric response (35%), in which
the caloric response was different in both ears, with the
value exceeding 30% but the test results of the rotary test
and VEMP were normal; (3) bilateral vestibular hypofunction (25%), where the caloric response and the amplitude of
the VEMP were less intense than normal, while the response
to the rotatory stimulus was normal and (4) bilateral areflexia (25%), where the caloric response was zero, as in
the VEMP and no nystagmic or postrotatory bursts were
recorded.
In general, taking into account the limitations of the
tests performed, it can be considered that in children with
severe-profound hearing loss, the canalicular function is
more vulnerable than the otolithic; canalicular function
suffers a significant deterioration in a situation of severe
hearing loss and the latter shows severe deterioration only
occasionally in the case of profound hearing loss, while both
are affected in a situation of cophosis.22

387

Changes Resulting From Implantation in the

Cochlea: Vestibular Function
Tests performed: clinical examination of VOR, bi-thermal
caloric test, rotational and VEMP. In a study with a large
number of children assessed systematically before surgery,
the results of a caloric test allowed them to be grouped
into 4 groups: (1) normal (50%); (2) unilateral hypofunction
(22.5%); (3) bilateral hypofunction (7.5%); and (4) bilateral areflexia (20%). Unlike in other studies (with a smaller
number of patients), there was no correlation between the
degree of hearing loss and vestibular deficit. Placing an
implant in the cochlea produced the following changes: (1)
canalicular function deteriorated in 31% of children who
had a normal previous response (most frequently generating hypoexcitability, not areflexia); (2) otolithic function
deteriorated in 55% of children with normal preoperative
response; (3) 27% of children presented symptoms consistent with acute vestibulopathy and (4) changes could occur
even months after the intervention. In general terms, 30%
presented alterations in all tests after implantation and it
is considered that the changes have already taken place
definitively after 3 months.23 It is in that period of time
that a more complete and efficient vestibular compensation
process can be expected.
The most abnormal result in the caloric test (vestibular
areflexia) is the subject of another study in a similar group of
children who underwent a complete videonystagmography
(VNG); this revealed that 49% of children referred vestibular symptoms (without specifying their type or degree) in the
immediate postoperative period, that those children who
had a normal caloric response went from 63% before surgery
to 39% after surgery, and that those with caloric areflexia
went from 14% before surgery to 34% after surgery. This relevant change in the vestibular function is compensated in
the vast majority of children without creating medium-term
symptoms except in a few; so far, only an alteration in visual
suppression of caloric nystagmus has been identified as a risk
factor for poor prognosis for such an eventuality.24
The abnormal postoperative response is also detected
effectively with VEMP. This test is recommended for its
simplicity and consistency of response. The percentage of
children who presented alterations in the VEMP before
implantation was high and correlated well with age of onset
of hearing loss and motor development, in that the earlier hearing loss became established, the worse the result
in VEMP and motor development tests. The VEMP revealed
that, although there was a certain degree of postoperative
impairment in 80% of children, this depended directly on
the surgical inner ear approach, namely the performance
of the cochleostomy and the implantation procedure. However, when studying a population of children implanted in a
single ear, there were no differences in the various parameters of the VEMP obtained between the implanted ear and
the non-implanted.25

Changes Resulting From Implantation in the

Cochlea, Vestibular Function and Motor Skills
Children with congenital or acquired severe-profound hearing loss users of a cochlear implant: Tests performed:

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388
exploration of dynamic equilibrium (BOTMP, balance subtest) and rotatory test with an active implant. The average
value of the BOT2 was 13 5, which was significantly different from the value in the normal population; there was
a trend towards improved results when the implant was
active with respect to when it was turned off. There was
also good correlation between the findings and developments in the BOT2 and the gain and phase values of the
VOR at intermediate stimulation frequencies from 0.16 to
0.64 Hz.26 It is interesting to observe that the children who
used cochlear implants showed an increased visual dependence compared to those with normal hearing. In general
terms, when analysing multiple variables (many of them
related to each other), it is possible to find good correlation between degree of balance and aetiology (worse if the
cause of deafness was meningitis or a cochlear-vestibular
malformation), but not with age of implantation or time of
implant use.27
In these children, it is particularly necessary to implement a vestibular rehabilitation plan that aims to encourage
substitution through exercises for visual-vestibular facilitation and proprioceptive function enhancement, similarly
to those mentioned previously. In a study with implanted
children with varying degrees of severe-profound hearing
loss and bilateral vestibulopathy, it was possible to achieve
better and more complete sensory organisation and coordination and, ultimately, better balance.28 The improvement
was objectified in the PDMS score and postural analysis with
Condition 3 of the CDP and the somatosensory and visual
indexes, which showed improvement nearing the values of
normal children. This study observed that the group of children with no rehabilitation evidenced a worsening of results
between studies in the previously mentioned tests. Consequently, it is possible to state that it was also possible to
stop the motor delays that these children suffered additionally. As with auditory rehabilitation, there seems to exist a
period that should not be exceeded or else there will be a
significant limitation on the recovery of balance and posture. Similarly, we must not forget that these are children
with bilateral vestibulopathy and the results should thus be
evaluated in the long term or a maintenance plan to retain
the gain without reverting to motor impairment should even
be implemented. It is possible that over time they could be
engaged in more complex exercises interfering or distracting vision and proprioception. The compensation capability
is documented and depends on the age of onset of damage;
both in congenital and acquired cases, they will be able to
obtain a normal Romberg, but only congenital cases will be
able to stand on one foot with eyes open, perform a tandem
walk, ride a bicycle or even swim, activities which the latter
will be able to do with errors or difficulty. In contrast, it will
be difficult for both (but especially for the latter) to stand
on one foot with closed eyes or walk on a balance bar.11
Alterations do not consist of only the deleterious effect of
vestibular damage. Hearing loss in itself generates a series
of changes in neuropsychological development of children,
among which is a relevant delay in visual-spatial coordination and maturation (without reaching a pathological range),
which disappears in the group of children implanted in a
single ear.29 A cascade of alterations takes place, which
become difficult to restore after some time as motor development follows a predetermined sequence or program. In

B. Gonzlez del Pino et al.

this sequence, auditory development takes place very early,
already before birth and, among this cascade, vestibular
function probably acts as a catalyst of deterioration.

Genetic Alterations
Given the high number of genetic alterations and the frequent association of hearing loss, we will review a small
number of alterations that are worth clarifying because of
their uniqueness or relevance. Hypoplasia of the semicircular canals is very frequent in the CHARGE complex. This leads
to 52% of children presenting vestibular symptoms or a significant alteration in vestibular testing; surprisingly, it seems
that only vestibular alteration is specific for the mutation
CHD7.30 In the case of mutations of the connexin gene associated with non-syndromic hearing loss, we can expect some
degree of vestibular alteration since, in Cx30/ mutant
mice, there is degeneration evident only in saccule cells.
Gap junctions are probably hybrid and require both Cx26
and Cx30; in contrast, in the case of isolated deletion of
Cx30, only the saccular ones are lost, which can be retrieved
experimentally by overexpressing Cx26.31 This phenomenon
may underlie the disorder found in children with the A1555G
mutation in whom the frequency of vestibular symptoms
is variable depending on the series, but who show a curious combination of complete VEMP involvement with normal
caloric response in 60% of cases.32 This finding is identical
to that obtained in patients with mitochondrial pathology
in whom the saccule endolymphatic space is collapsed (as
described histologically), which in turn generates the fundamental vestibular damage.33

Motion Sickness
Despite being classified as one of the forms of physiological dizziness, the impact of motion sickness is extremely
high in the daily evolution of children and their families. It
is the combination of 2 different mechanisms: (1) inconsistency of sensory information converging at a given time at
the central level and (2) excessive release of acetylcholine
in the vestibular nuclei. The above factors eventually lead
to confusion or loss of spatial orientation. Children are influenced by several factors: (1) size, which makes them lose
visual references; (2) incongruous physical activity, natural
in childhood games, which facilitates overstimulation and
(3) lack of habituation. The smaller the child, the less susceptibility to motion sickness there is, but, surprisingly, the
only symptom may be a postural disorder and headache in
the smallest children; as the vestibular system matures, the
postural disorder decreases in severity but the vegetative
symptoms increase.34

Head Traumatism
Children with Minor Head Traumatism
The frequency of vertigo or instability in children who have
suffered cerebral concussion is 51% and the percentage of
children referring noise intolerance during the first days
is 65%; in any case, these figures are not different from

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Vestibular Examination of Children With Alterations in Balance (II): Results by Pathologies

those found in children who have suffered banal non-cranial
trauma, which leads to doubts about the existence of postconcussion syndrome in children.35 In BOT2, the result is
lower than expected at 1, 4 and 12 weeks after the traumatism, but a progressive improvement is documented. The
situation is notably worse when children must perform exercises on the balance beam such as standing on one foot
with eyes closed, walking in tandem and overcoming an
obstacle. These are exercises that not only alter the sensory information, they also require a higher level of motor
coordination.36 The level of demand lies in the anticipatory
activity for balance, in other words, that which enables an
individual to prepare and organise the motor activity necessary to overcome a new or complex situation. These are
not mechanisms subject to feedback control, which would
explain the difficulty or near impossibility to recover them
and hence adapt.37 In children with mild head traumatism
who do not improve at the beginning with conventional
measures of resting and vigilance after the traumatism,
vestibular rehabilitation has shown an improvement in symptoms (more intense in children than in adults) and in the
results obtained in Conditions 1 and 2 of the CDP.38 We
must not forget that BPPV in its various forms is also
within the post-traumatic vestibular manifestations in children that require precise clinical assessment and adequate
treatment.39
Among the most unusual but potentially dangerous posttraumatic phenomena is dissection of the vertebral artery,
which occurs occasionally after a forced cervical manoeuvre (for example, during sports practice). In these cases,
ataxia and vertigo are severe enough to insist on the differential diagnosis even though the initial radiological studies
do not include an examination of the neck and its vascular
contents.40

Emergencies
Despite being very uncommon, the desire for good management of children with vertigo in the emergency service
seems to be one of the priorities in GP environments.41
It is possible to diagnose and treat systemic disorders
that often begin with a vague and imprecise feeling of
dizziness, as occurs in arterial hypertension and metabolic
disorders or specifically otological emergencies, such as
an exacerbation of OME in the form of acute otitis media
leading to labyrinthitis. This is a true emergency, which
requires an adequate medical and surgical approach. As
for the former, one should consider that a bacterial cause
of a labyrinthisation process of the middle ear is only
detected in 65% of children, without there being a specific
predisposition for a specific agent.42

Tumours of the Internal Auditory Canal

and Posterior Fossa and Central Vestibular
Syndrome
The incidence of vestibular schwannoma (outside the context of neurofibromatosis) is very low and there are fewer
than 50 cases described in the literature. The onset symptoms are otological in only 60% of cases and, given that there
is a predominance of intracranial hypertension (headache

389

and vomiting), cerebellar syndrome (ataxia and falls) and

paralysis of several cranial nerves (predominantly of the
facial), it is necessary to maintain a high degree of suspicion if these symptoms appear in the clinical evolution of
the vestibular process of the child.43 In contrast, the most
common symptoms of tumours of the posterior fossa and
brainstem include vertigo and ataxia with headache (and
less frequently tinnitus) and, almost as an oddity, hearing
loss; in terms of exploration, ocular motility (tracking) is
highly abnormal, as is the optokinetic nystagmus, and there
is also nystagmus evoked by lateral gazing, looking vertically downwards or even opsoclonus, with normal caloric
test results in which the visual suppression of caloric or
per-rotatory nystagmus was normal.44
In addition to tumours in the locations mentioned above,
other causes of central vestibular syndrome are encephalitis and multiple sclerosis. In children with hydrocephalus
treated by means of a shunt, irrespective of the aetiology, it is common to find signs of central location during
vestibular exploration (82%). However, 46% also show signs
of peripheral location. This high rate of mixed findings may
well explain the high rate of motor development that they
present.45
Along with the alterations mentioned above, one cause
of suspicion that should lead to a radiological study is
cranial traumatism. In general, the presence of persistent
headaches and/or neurological signs indicates the need for
a radiological study; isolated vertigo does not seem to be
relevant enough to provide information both in the MRI and
in the CT.46

Temporal Bone Abnormalities

Cochlear-vestibular nerve aplasia represents a diagnostic
challenge that requires a complete functional study, not only
auditory or vestibular but also radiological; when in doubt,
the vestibular examination can be very useful, in particular
the study of per-rotatory nystagmus and the precise levels
of its gain and simmetry.47
Dilatation of the vestibular duct affects the canal and
endolymphatic sac. The prevailing symptoms are auditory but 50% of children (as in adults) present additional
vestibular symptoms (episodic vertigo, motor retardation or
instability), although this incidence increases in the case of
children who carry the Pendred disease mutation.48 Vestibular disorders are usually found in the caloric exploration:
unilateral or bilateral hypofunction. VEMP studies find a significant decrease in the response threshold, possibly due to
a third-window phenomenon in the inner ear.

Mnires Disease
Mnires disease can be manifested in surprising manners,
such as cyclical vomiting without other apparent symptoms,
until after inquiry by the physician, perhaps due to how
strange or peculiar it may be for a child to perceive a sound
in the ear.49 It is common to find a high degree of canalicular paresis in children, despite the process being nascent
and undeveloped, due to a phenomenon of fluctuation of
vestibular function as intense as that of hearing function;
this is a phenomenon that is less common in adults.50 The

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390
glycerol test and the electrocochleography provide highly
pathological data in children.51
Delayed endolymphatic hydrops (DEH) is related to
Mnires disease. It appears in adults who have suffered
a severe-profound hearing loss in childhood; in general, in
the case of children with severe unilateral hearing loss, the
risk of suffering both ipso- and contralateral DEH is 17% in
the first 15 years and 30% for the rest of their lives.52

Somatoform Disorders
The incidence of somatoform disorders is almost 3% among
children with vertigo. Risk factors that must be taken into
consideration for an adequate approach include: frequent
crises or continuous vertigo with associated headache,
low class attendance and relationship problems with family
or friends.53

Conflict of Interests
The authors have no conflicts of interest to declare.

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