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Vertigo In children

       Mohamed I Shabana
Professor of Audiological Medicine
         Cairo University
Vertigo in Children
Developmental
Children under the age of 4 months
- Tonic neck reflexes predominate
- These reflexes can be demonstrated by passive or active
   motions of the head relative to the position of the body
- This reflex is due to movement of endolymphatic fluid
   through the semiciruclar canals. These tonic neck
   reflexes are dependent on the integrity of vestibular and
   proprioceptive systems.

  Neck righting: In this test active / passive rotation of head
  from the midline to one side when the infant is lying supine
  will cause a rotation of the whole body .
Developmental
4 - 6 months.
 Babies in this age group vary in their
developmental achievements. Many normal
infants still have residual primitive tonic neck
reflexes, while in others, righting responses
will appear. Both these conditions are normal.
Developmental
6 - 18 months.
 This is a period of rapid motor and sensory
development. The pyramidal tract becomes
myelinated. Integration of visual, labyrinthine
and proprioceptive stimuli occurs during this
phase. Righting reflexes are elicited by an
abrupt tilt of the patient to change the
patient's centre of gravity.
Developmental
Since the optical and vestibular righting responses
  are identical the baby must be tested blind folded
  in order to eliminate visual cues. The most
  important of the righting reflexes is the head
  righting response. This can be obtained by
  picking up the infant from prone / supine position
  and bringing it to upright position by tilting the
  infant sideways, forwards or backwards. Every
  abrupt change of the head position in space will
  elicit vestibular head righting response. At the
  same time propping reactions of the extremities
  may be seen.
Prevalence of dizziness in children:
• The population-based prevalence of vertigo and dizziness
  among school children has been reported to be 15%. In the
  literature, vertigo in children has received much less
  attention than vertigo occurring in adults. Even among
  otologists and child neurologists, the key clinicians
  providing appropriate diagnosis and treatment for
  vertiginous children, the differential diagnosis is not well
  established. The clinical picture of vertigo in children
  deviates from vertigo in adults, since young children do
  adapt very well to vertigo and dizziness and compensate a
  vestibular deficit quicker than adults (Niemensivu et al.,
  2006).
What is your complaint son ??


                  I am Dizzy
How are they Complaining??
• - Delayed walking
• - Clumsiness
• - nausea
• - episodic pallor and fatigue
• - difficulty walking in Darkness, or uneven
  surface
• - Headache blurred vision
• - Difficulty reading in moving Vehicle
• - Gaze stabilization problems
• Vertigo in children differs from that in adults, because of
  three main reasons.

• Firstly, vestibular disorders are often ignored in children,
  because vertiginous manifestations are usually attributed to
  lack of coordination or behavioural problems.

• Secondly, as children often lack the communication ability
  to describe accurately their symptoms, diagnosis is based
  less in history and much more in clinical examination and
  laboratory investigations.

• Finally, although most diseases that cause vertigo in
  adulthood occur in childhood as well, their frequency may
  be different, depending on the age of the patient.
Dizzychilddubai
WHAT ARE OUR KEY ELEMENTS IN the
           HISTORY??
                                   Knowledge of the
      Parents
                                       causes


                Investigations &
                 interpretation
Ravid,elal (2003)
Dizzychilddubai
Arabic Version of
         Pediatric Dizziness Inventory Questionnaire

Presentation for discussion of a Thesis Submitted For Fulfilment of the Master Degree in Audiology
                                                 By:
                                 Mariam Magdy Medhat
                                           M.B., B. CH.
                                           Supervisors:
                        Prof. Dr. Mohamed Ebrahim Shabana
                                       Professor of Audiology,
                                        Faculty of Medicine,
                                          Cairo University

                                Dr. Abeir Osman Dabbous
                                  Assistant Professor of Audiology,
                                        Faculty of Medicine,
                                          Cairo University

                                     Dr. Noha Ali Hosni
                                        Lecturer of Audiology,
                                         Faculty of Medicine,
                                           Cairo University,

                                   Kasr El-Aini Faculty of Medicine
                                           Cairo University,
                                                 2011
Aim of the work
•   To develop an Arabic paediatric dizziness inventory
    questionnaire for the parents of dizzy children to
    address the balance complaints of their children by the
    information gathered from it. This evaluation will help
    to identify any balance dysfunction and to quantify the
    impact of dizziness on daily living and to describe the
    dizziness complaint, and helps to reach diagnoses of the
    balance dysfunction in children and directs us towards
    the necessary investigations to confirm this diagnosis.
Dizzychilddubai
Dizzychilddubai
Dizzychilddubai
Dizzychilddubai
Figure (2) : Distribution of the conclusion reached from the
                    questionnaire in the cases.

                  1                             Vestibular
             1   5%
       1    5%
                                                Cervical
      5%

                                                General
 2
10%
                                                Ocular
                                         9
                                        45%
                                                General/CVS

 1
5%                                              Neurological/Ocular

  1
 5%                                             Ocular/Cervical



            3                                   Vestibular / CVS

           15%         1
                      5%                        Non specific (Ocular/General/
                                                Neurological/Audiological
                                                association)
Figure (6): The ability of the questionnaire to match the diagnosis according to
                      the referral for different categories.


                   100%
                    90%
                    80%
                    70%
                    60%
      Percentage




                                                                  Not matching
                    50%
                                                                  Matching
                    40%
                    30%
                    20%
                    10%
                     0%                            S




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                                     r/c ar
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Conclusions:
• We have developed an Arabic pediatric dizziness inventory questionnaire
  for the parents of dizzy children. A scoring system has been developed to
  address the balance complaints in children by the information gathered
  from it. Evaluation of dizzy children using our Arabic pediatric dizziness
  inventory questionnaire helped to identify balance dysfunction and was
  able to categorize the dizzy children by the affected system/systems.

• The questionnaire and its scoring system were valid, being
  comprehensive enough to collect all the information needed to address
  the balance problem. The questionnaire was able to quantify the impact
  of dizziness on daily living, to describe the dizziness complaint that
  helped to reach a diagnosis of the balance dysfunction in children and to
  direct the clinician towards the necessary investigations to confirm this
  diagnosis.
Conclusions:

• The Arabic dizzy children questionnaire's categories matched
  the diagnosis on referral in 75% of cases. The sensitivity of
  the questionnaire in reaching the diagnosis was calculated at
  75%. Its sensitivity in diagnosing vestibular category was
  88.89%. The sensitivity in multi-system affection was 83.3%.


• The Arabic dizzy children questionnaire defined a matched
  specific diagnosis for the cause of dizziness in 11/20 (55%) of
  cases.
What are
you going
  to do
•OBSERVATION
Low muscle tone

– Delay in holding head up

– “Snuggly” baby

– “Floppy baby”

– Arching of back
Delayed disappearance of newborn
             reflexes

– Moro
– ATNR: Asymmetric tonic next response
– Usually disappear by 6-7 months
Dizzychilddubai
Delayed motor milestones
– Average deaf child walks at 14 months

– Average child with Usher’s Type 1 walks at 20 mos

– Delays sitting, crawling, climbing steps, hopping…

– Speech delays
What do older children look like?
• Clumsy

• Unable to walk on a balance beam
• Problems standing with feet together and eyes
  closed (Romberg test)
What do older children look like?
• Love spinning, merry-go-rounds, water
  activities

• Weak VOR: Challenges with reading
  – Gaze instability causes problems with acuity
Signs of poor vestibular function
• Low muscle tone
• Delayed loss of primitive reflexes
• Delayed gross motor milestones
•   Developmental delays
•   Seizures
•   Nystagmus
•   Easy fatigability
•   Torticollis
Dizzychilddubai
Causes of dizziness in children
    A) Otologic:

 Congenital disorders:
 Syndromic hearing loss and vestibular dysfunction:
       –
       –
            Usher syndrome
            Pendred syndrome
                                                          Examination
       –    Enlarged vestibular Aqueduct syndrome
       –    Congenital Long-QT Syndrome
       –    CHARGE Syndrome
 Non-syndromic hearing loss and vestibular dysfunction
 Congenital anomalies of the skull base


 Traumatic disorders:
•     Head Trauma
•     Paroxysmal Positional Vertigo
•     Perilymphatic Fistula
•     Cochlear Implant Surgery                              History
Syndromes
  Over 500 nDNA syndromes known to affect the
            audiovestibular (AV) system.
• Usher’s Syndrome (Type 1)
• Waardenburg Syndrome
• Pendred syndrome

• CHARGE Syndrome
• Brachio-oto-renal syndrome
Retinitis pigmentosa




                       <>
Retinitis pigmentosa




     http://www.blindness.org/content.asp?id=45
Waardenburg Syndrome




      http://www.werathah.com/deafness/waardenburg.htm
Pendred Syndrome
CHARGE Syndrome




www.charrgesydnrome.org
Causes of dizziness in children
    A) Otologic:
Inflammatory disorders:
•     Otitis Media-related vertigo
•     Chronic Suppurative Otitis Media and Cholesteatoma
•
•
      Vestibular neuronitis
      Labyrinthitis
                                                           Examination
•      Bacterial meningitis



Idiopathic:
 Endolymphatic hydrops:
       –   Menière's disease
       –   Delayed endolymphatic hydrops                   investigation
 Motion Sickness
 Autoimmune Disorders
      Post Cochlear Implant
Causes of dizziness in children
B) Neurological disorders:
 Migraine variants and complicated migraine :
   1- Paroxysmal Torticollis
   2- Cyclical Vomiting                                History
   3-Basilar Artery Migraine
   4-Familial Hemiplegic Migraine
   5- Abdominal Migraine
                                                    Investigation
   6- Idiopathic benign paroxysmal vertigo
 Migraine-associated dizziness
 Epilepsy
 Episodic ataxia
 Multiple sclerosis
 Vascular Occlusion                             investigation
 Brain tumors
Benign Paroxysmal Vertigo
• * Common un recognized condition
• * Paroxysm, Recurrent, non epileptic
• * Pale, Sweaty, Fearful, May sway
• * sudden onset, seconds to minutes duration
• * no loss of conscious, with complete
  recovery
• * Diagnosed By exclusion
• * Migraine precursor
Causes of dizziness in children
C) Psychological dizziness
D) Ocular disorders
E) Systemic disorders (General causes)
F) oto-toxic drugs



             Mainly History
Hearing Screening
Do we have Vestibular screening
Dizzychilddubai
Dizzychilddubai
How can we examine the children?
Investigation
• CT of temporal bone
• Vestibular testing
• Physical, occupational, ? cognitive therapies
• Genetic appointment
   – Strongly consider testing for Usher’s mutations

• Vision evaluation
   – ?ERG
Enlarged Vestibular Aqueducts
Ossification
Dynamic Imbalance Testing
                    VOR testing
•   Head thrust maneuver
•   Post-headshake nystagmus
•   Dix-Hallpike maneuver
•   Dynamic Visual Acuity
•   Gait
Dizzychilddubai
Head Thrust test
Dynamic Visual Acuity
Dizzychilddubai
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Posture Control and Gait
The Foam test
Dizzychilddubai
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Static Balance Testing
                Posturography •
Dynamic Stability in Walking
     Gait Laboratory
Walking test
Vestibular testing

• Fukuda Stepping Test
• Vestibular Ocular Reflex Screening-Swivel
  Chair with Video-oculographic (VOG)
  Recording
• ENG/VNG
• Rotary Chair Testing
• VEMPs
ENG/VNG
Caloric irrigation: This test is performed only in
  children aged 4 and older. Ideally performed
  with the baby blind folded, in the supine
  position, with the head ventroflexed at 30
  degrees. The child is also restrained. A ten
  second irrigation is a must for adequate
  stimulus. Recording should start immediatly
  after the onset of irrigation.
ENG/VNG
If the child is sleepy or irritable during the test
   the response may not be accurate. This test
   is a rather crude way of testing vestibular
   response to a stimulus. This test is hence
   performed only in cases of extreme doubts
   regarding the function of vestibular apparatus.

Make it the last examination
ENG/VNG
There is a maturation pattern in the
  development of caloric evoked nystagmus
  response. The amplitude and the number of
  beats increase in the first three months of
  life. The intensity of the nystagmus is directly
  proportional to the gestational age and the
  weight at birth. The latency of the response
  decreases with the gestational age
  and increasing birth weight.
ENG/VNG
Optokinetic stimulation:
Optokinetic nystagmus can be evaluated in most
 children within three to six months of
 birth. As the child grows older, they learn to
 pay more attention to the moving images and
 better responses can be obtained in
 them. This nystagmus can be recorded in
 response to two speeds of rotation i.e. 3
 degrees and 16 degrees per second.
ENG/VNG
The frequency, amplitude and speed of the slow
  component can be analysed in response to the
  two rotational speeds. The information
  obtained is helpful in the evaluation of overall
  quality of neurovestibular function.
Dizzychilddubai
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Dizzychilddubai
DR. ABEIR OSMAN DABBOUS
  Assistant Professor of Audiology,
    Kasr El-Aini, Cairo University.
• The impairment of saccular function,
  indicated by the abnormal findings in the
  VEMP , is often associated with SNHL in the
  pediatric population.


• With the increasing occurrence of pediatric
  patients with symptoms of dizziness, VEMP
  testing may be a means to evaluate unilateral
  vestibular function (Honaker and Samy,
  2007).
Vestibular evoked myogenic potential (VEMP)
                                           inferior vestibular nerve

                       Saccule

                                    medial vestibulospinal tract


                                                                   accessory
                                                                       nerve

                                      ipsilatral SCM



  The function of this sacculo-collic reflex is to stabilize the •
 head in response to unpredictable displacements (Halmagyi &
                                                       Curthoys 2000).
VEMP Method

           Surface Electrodes : •
Non-inverting active: middle third –
             of each SCM muscle,

Inverting reference: supra-sternal –
notch, or at each sternal insertion

               Ground: forehead. –
Dizzychilddubai
VEMP waveform




                       (Murofushi and Kaga, 2009).

                                  Latency (in msec),
      P1 latency decreases with increasing rate.
VEMP response
                                     Waveform:
                                   Latency (in msec), •
                                   Threshold: (dBSPL) •
                                   Amplitude (in μV), •
      N23


             Amplitude= 77.81uv




            Our laboratory norms (mean 2SD) for the different
P13                         studied VEMP parameters were:
                             N13 latency: 12.89 1.9 msec; •
                            P23 latency: 21.31 4.02 msec; •
                 P13-N23 latency Interval: 8.42 3.54 msec; •
              P13-N23 amplitude Interval: 80.95 36.84 V; •
                         IAD: -0.01 0.16. (Dabbous, 2007). •
Amplitude (in μV),
• decreases with increasing rate above 5-Hz

• EP ratio or the inter-aural difference ratio (IAD):
  [(Ar−Al)/ (Ar+Al), x 100],
     • a ratio of > 3:1 abnormal
An example of Normal
               IAD


                       N23
               P13
                     = 23.0   Amplitude= 38.13 uv
           = 14.6

Rt

                              IAD =0.023


 Lt
        P13                   Amplitude= 39.94 uv
                      N23
      = 15.2
                     = 22.2
N23 = 20.8
                                          An example of
                                          abnormal IAD
                                           Amplitude= 77.81 uv

Rt
      P13 = 12.2

                        N23 = 22.0
                                                          IAD = 0.45

 Lt




                                         Amplitude= 29.56 uv
           P13 = 14.2
VEMP amplitude
depends on:
 1.   Saccular function
 2.   Stimulus intensity, air-conduction
 3.   Electrode conduction & location
 4.   Linearly increases with the EMG level
Clinical utility of VEMP testing :
         sacculo-vestibular nerve function.
           assessment of vestibular nerve function:    .1
                              acoustic neuromas –
                            vestibular neuronitis –
                                multiple sclerosis –
diagnosis of superior semicircular canal dehiscence    .2
                                         syndrome,
                 evaluation of Menière's syndrome      .3
                        Sensori-neural hearing loss.   .4
VEMPs in a large Vestibular Aqueduct
Most common anomaly
Sudden fluctuation in pressure:
1. progression of SNHL after head trauma,
2. VEMP has greater amplitude and lower
   threshold (Sheykholesami et al, 2004).
VEMP in diagnosis of
                   Superior Canal Dehiscence Syndrome
                                    Rare    •
a ‘third window’ :                    •
pseudo-conductive HL, ABG at                     –
low frequencies,
Tullio phenomenon of –
acoustically evoked vertigo &
nystagmus,
VEMP : –
increased amplitudes –
lowered threshold (70 dB) –
(Colebatch et al., 1998; Streubel et al., 2001
Brantberg et al., 1999; Ostrowski et al., 2001
Minor et al., 2003; Mikulec et al., 2004).
Chronic otitis media
• Chronic OM could delay and reduce the energy
  transfer of sound to the inner ear.
• Improvement of postoperative VEMP response rate
  and p13 latencies in the patients with and without
  improvement in postoperative 500 Hz - ABG,
  provide evidence that the sound energy inducing a
  VEMP might be different from the energy producing
  the auditory perception (Wang et al., 2008).
Migraine and its equivalents
• Migraine: the most common cause of episodic vertigo in
  children.
• Allena et al., (2007) postulated that VEMP abnormalities in
  migraine are due to reduced serotonergic control of the reflex
  circuit, in particular of the vestibular nuclei.

• Benign Recurrent (Paroxysmal) Vertigo or benign
  recurrent vertigo (BRV):
   – a major cause of vertigo in children
   – 30% have abnormal caloric responses,
   – 50% have abnormal VEMP responses (Ozeki et al., 2008).
Our VEMP Studies: in Migraineurs

                                         N23




normal                             P13
 25%

                                          N23


             VEMP
          abnormalities           P13
              75%


                       delayed
               latencies of P13
                     and N23.
VEMPs in Children with Cochlear Implants

                                     traumatic damage →
                                absent VEMPs or decreased
                                                amplitude



                                                  With CI on:
                                 electrical current spread at
                                  C level, apical channels →
                                stimulates the IVN: present
                                 VEMPs or absent VEMPs if
                                    requiring higher current
 present VEMPs >50%                 intensities, but difficult
                                        (pain or facial nerve
                                          stimulation) (Jin et
                                                   al., 2008).
Thank
 you
  all

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Dizzychilddubai

  • 1. Vertigo In children Mohamed I Shabana Professor of Audiological Medicine Cairo University
  • 3. Developmental Children under the age of 4 months - Tonic neck reflexes predominate - These reflexes can be demonstrated by passive or active motions of the head relative to the position of the body - This reflex is due to movement of endolymphatic fluid through the semiciruclar canals. These tonic neck reflexes are dependent on the integrity of vestibular and proprioceptive systems. Neck righting: In this test active / passive rotation of head from the midline to one side when the infant is lying supine will cause a rotation of the whole body .
  • 4. Developmental 4 - 6 months. Babies in this age group vary in their developmental achievements. Many normal infants still have residual primitive tonic neck reflexes, while in others, righting responses will appear. Both these conditions are normal.
  • 5. Developmental 6 - 18 months. This is a period of rapid motor and sensory development. The pyramidal tract becomes myelinated. Integration of visual, labyrinthine and proprioceptive stimuli occurs during this phase. Righting reflexes are elicited by an abrupt tilt of the patient to change the patient's centre of gravity.
  • 6. Developmental Since the optical and vestibular righting responses are identical the baby must be tested blind folded in order to eliminate visual cues. The most important of the righting reflexes is the head righting response. This can be obtained by picking up the infant from prone / supine position and bringing it to upright position by tilting the infant sideways, forwards or backwards. Every abrupt change of the head position in space will elicit vestibular head righting response. At the same time propping reactions of the extremities may be seen.
  • 7. Prevalence of dizziness in children: • The population-based prevalence of vertigo and dizziness among school children has been reported to be 15%. In the literature, vertigo in children has received much less attention than vertigo occurring in adults. Even among otologists and child neurologists, the key clinicians providing appropriate diagnosis and treatment for vertiginous children, the differential diagnosis is not well established. The clinical picture of vertigo in children deviates from vertigo in adults, since young children do adapt very well to vertigo and dizziness and compensate a vestibular deficit quicker than adults (Niemensivu et al., 2006).
  • 8. What is your complaint son ?? I am Dizzy
  • 9. How are they Complaining?? • - Delayed walking • - Clumsiness • - nausea • - episodic pallor and fatigue • - difficulty walking in Darkness, or uneven surface • - Headache blurred vision • - Difficulty reading in moving Vehicle • - Gaze stabilization problems
  • 10. • Vertigo in children differs from that in adults, because of three main reasons. • Firstly, vestibular disorders are often ignored in children, because vertiginous manifestations are usually attributed to lack of coordination or behavioural problems. • Secondly, as children often lack the communication ability to describe accurately their symptoms, diagnosis is based less in history and much more in clinical examination and laboratory investigations. • Finally, although most diseases that cause vertigo in adulthood occur in childhood as well, their frequency may be different, depending on the age of the patient.
  • 12. WHAT ARE OUR KEY ELEMENTS IN the HISTORY?? Knowledge of the Parents causes Investigations & interpretation
  • 15. Arabic Version of Pediatric Dizziness Inventory Questionnaire Presentation for discussion of a Thesis Submitted For Fulfilment of the Master Degree in Audiology By: Mariam Magdy Medhat M.B., B. CH. Supervisors: Prof. Dr. Mohamed Ebrahim Shabana Professor of Audiology, Faculty of Medicine, Cairo University Dr. Abeir Osman Dabbous Assistant Professor of Audiology, Faculty of Medicine, Cairo University Dr. Noha Ali Hosni Lecturer of Audiology, Faculty of Medicine, Cairo University, Kasr El-Aini Faculty of Medicine Cairo University, 2011
  • 16. Aim of the work • To develop an Arabic paediatric dizziness inventory questionnaire for the parents of dizzy children to address the balance complaints of their children by the information gathered from it. This evaluation will help to identify any balance dysfunction and to quantify the impact of dizziness on daily living and to describe the dizziness complaint, and helps to reach diagnoses of the balance dysfunction in children and directs us towards the necessary investigations to confirm this diagnosis.
  • 21. Figure (2) : Distribution of the conclusion reached from the questionnaire in the cases. 1 Vestibular 1 5% 1 5% Cervical 5% General 2 10% Ocular 9 45% General/CVS 1 5% Neurological/Ocular 1 5% Ocular/Cervical 3 Vestibular / CVS 15% 1 5% Non specific (Ocular/General/ Neurological/Audiological association)
  • 22. Figure (6): The ability of the questionnaire to match the diagnosis according to the referral for different categories. 100% 90% 80% 70% 60% Percentage Not matching 50% Matching 40% 30% 20% 10% 0% S l r/c ar r ar S l al c ca ca la e n CV ifi V l er ul vi cu cu vi /C ec en tib er er l/o / O al sp ar G C es er ca on ul V la gi tib N cu lo G es ro O V eu N
  • 23. Conclusions: • We have developed an Arabic pediatric dizziness inventory questionnaire for the parents of dizzy children. A scoring system has been developed to address the balance complaints in children by the information gathered from it. Evaluation of dizzy children using our Arabic pediatric dizziness inventory questionnaire helped to identify balance dysfunction and was able to categorize the dizzy children by the affected system/systems. • The questionnaire and its scoring system were valid, being comprehensive enough to collect all the information needed to address the balance problem. The questionnaire was able to quantify the impact of dizziness on daily living, to describe the dizziness complaint that helped to reach a diagnosis of the balance dysfunction in children and to direct the clinician towards the necessary investigations to confirm this diagnosis.
  • 24. Conclusions: • The Arabic dizzy children questionnaire's categories matched the diagnosis on referral in 75% of cases. The sensitivity of the questionnaire in reaching the diagnosis was calculated at 75%. Its sensitivity in diagnosing vestibular category was 88.89%. The sensitivity in multi-system affection was 83.3%. • The Arabic dizzy children questionnaire defined a matched specific diagnosis for the cause of dizziness in 11/20 (55%) of cases.
  • 27. Low muscle tone – Delay in holding head up – “Snuggly” baby – “Floppy baby” – Arching of back
  • 28. Delayed disappearance of newborn reflexes – Moro – ATNR: Asymmetric tonic next response – Usually disappear by 6-7 months
  • 30. Delayed motor milestones – Average deaf child walks at 14 months – Average child with Usher’s Type 1 walks at 20 mos – Delays sitting, crawling, climbing steps, hopping… – Speech delays
  • 31. What do older children look like? • Clumsy • Unable to walk on a balance beam • Problems standing with feet together and eyes closed (Romberg test)
  • 32. What do older children look like? • Love spinning, merry-go-rounds, water activities • Weak VOR: Challenges with reading – Gaze instability causes problems with acuity
  • 33. Signs of poor vestibular function • Low muscle tone • Delayed loss of primitive reflexes • Delayed gross motor milestones • Developmental delays • Seizures • Nystagmus • Easy fatigability • Torticollis
  • 35. Causes of dizziness in children A) Otologic:  Congenital disorders:  Syndromic hearing loss and vestibular dysfunction: – – Usher syndrome Pendred syndrome Examination – Enlarged vestibular Aqueduct syndrome – Congenital Long-QT Syndrome – CHARGE Syndrome  Non-syndromic hearing loss and vestibular dysfunction  Congenital anomalies of the skull base  Traumatic disorders: • Head Trauma • Paroxysmal Positional Vertigo • Perilymphatic Fistula • Cochlear Implant Surgery History
  • 36. Syndromes Over 500 nDNA syndromes known to affect the audiovestibular (AV) system. • Usher’s Syndrome (Type 1) • Waardenburg Syndrome • Pendred syndrome • CHARGE Syndrome • Brachio-oto-renal syndrome
  • 38. Retinitis pigmentosa http://www.blindness.org/content.asp?id=45
  • 39. Waardenburg Syndrome http://www.werathah.com/deafness/waardenburg.htm
  • 42. Causes of dizziness in children A) Otologic: Inflammatory disorders: • Otitis Media-related vertigo • Chronic Suppurative Otitis Media and Cholesteatoma • • Vestibular neuronitis Labyrinthitis Examination • Bacterial meningitis Idiopathic:  Endolymphatic hydrops: – Menière's disease – Delayed endolymphatic hydrops investigation  Motion Sickness  Autoimmune Disorders Post Cochlear Implant
  • 43. Causes of dizziness in children B) Neurological disorders:  Migraine variants and complicated migraine : 1- Paroxysmal Torticollis 2- Cyclical Vomiting History 3-Basilar Artery Migraine 4-Familial Hemiplegic Migraine 5- Abdominal Migraine Investigation 6- Idiopathic benign paroxysmal vertigo  Migraine-associated dizziness  Epilepsy  Episodic ataxia  Multiple sclerosis  Vascular Occlusion investigation  Brain tumors
  • 44. Benign Paroxysmal Vertigo • * Common un recognized condition • * Paroxysm, Recurrent, non epileptic • * Pale, Sweaty, Fearful, May sway • * sudden onset, seconds to minutes duration • * no loss of conscious, with complete recovery • * Diagnosed By exclusion • * Migraine precursor
  • 45. Causes of dizziness in children C) Psychological dizziness D) Ocular disorders E) Systemic disorders (General causes) F) oto-toxic drugs Mainly History
  • 46. Hearing Screening Do we have Vestibular screening
  • 49. How can we examine the children?
  • 50. Investigation • CT of temporal bone • Vestibular testing • Physical, occupational, ? cognitive therapies • Genetic appointment – Strongly consider testing for Usher’s mutations • Vision evaluation – ?ERG
  • 53. Dynamic Imbalance Testing VOR testing • Head thrust maneuver • Post-headshake nystagmus • Dix-Hallpike maneuver • Dynamic Visual Acuity • Gait
  • 68. Static Balance Testing Posturography •
  • 69. Dynamic Stability in Walking Gait Laboratory
  • 71. Vestibular testing • Fukuda Stepping Test • Vestibular Ocular Reflex Screening-Swivel Chair with Video-oculographic (VOG) Recording • ENG/VNG • Rotary Chair Testing • VEMPs
  • 72. ENG/VNG Caloric irrigation: This test is performed only in children aged 4 and older. Ideally performed with the baby blind folded, in the supine position, with the head ventroflexed at 30 degrees. The child is also restrained. A ten second irrigation is a must for adequate stimulus. Recording should start immediatly after the onset of irrigation.
  • 73. ENG/VNG If the child is sleepy or irritable during the test the response may not be accurate. This test is a rather crude way of testing vestibular response to a stimulus. This test is hence performed only in cases of extreme doubts regarding the function of vestibular apparatus. Make it the last examination
  • 74. ENG/VNG There is a maturation pattern in the development of caloric evoked nystagmus response. The amplitude and the number of beats increase in the first three months of life. The intensity of the nystagmus is directly proportional to the gestational age and the weight at birth. The latency of the response decreases with the gestational age and increasing birth weight.
  • 75. ENG/VNG Optokinetic stimulation: Optokinetic nystagmus can be evaluated in most children within three to six months of birth. As the child grows older, they learn to pay more attention to the moving images and better responses can be obtained in them. This nystagmus can be recorded in response to two speeds of rotation i.e. 3 degrees and 16 degrees per second.
  • 76. ENG/VNG The frequency, amplitude and speed of the slow component can be analysed in response to the two rotational speeds. The information obtained is helpful in the evaluation of overall quality of neurovestibular function.
  • 82. DR. ABEIR OSMAN DABBOUS Assistant Professor of Audiology, Kasr El-Aini, Cairo University.
  • 83. • The impairment of saccular function, indicated by the abnormal findings in the VEMP , is often associated with SNHL in the pediatric population. • With the increasing occurrence of pediatric patients with symptoms of dizziness, VEMP testing may be a means to evaluate unilateral vestibular function (Honaker and Samy, 2007).
  • 84. Vestibular evoked myogenic potential (VEMP) inferior vestibular nerve Saccule medial vestibulospinal tract accessory nerve ipsilatral SCM The function of this sacculo-collic reflex is to stabilize the • head in response to unpredictable displacements (Halmagyi & Curthoys 2000).
  • 85. VEMP Method Surface Electrodes : • Non-inverting active: middle third – of each SCM muscle, Inverting reference: supra-sternal – notch, or at each sternal insertion Ground: forehead. –
  • 87. VEMP waveform (Murofushi and Kaga, 2009). Latency (in msec), P1 latency decreases with increasing rate.
  • 88. VEMP response Waveform: Latency (in msec), • Threshold: (dBSPL) • Amplitude (in μV), • N23 Amplitude= 77.81uv Our laboratory norms (mean 2SD) for the different P13 studied VEMP parameters were: N13 latency: 12.89 1.9 msec; • P23 latency: 21.31 4.02 msec; • P13-N23 latency Interval: 8.42 3.54 msec; • P13-N23 amplitude Interval: 80.95 36.84 V; • IAD: -0.01 0.16. (Dabbous, 2007). •
  • 89. Amplitude (in μV), • decreases with increasing rate above 5-Hz • EP ratio or the inter-aural difference ratio (IAD): [(Ar−Al)/ (Ar+Al), x 100], • a ratio of > 3:1 abnormal
  • 90. An example of Normal IAD N23 P13 = 23.0 Amplitude= 38.13 uv = 14.6 Rt IAD =0.023 Lt P13 Amplitude= 39.94 uv N23 = 15.2 = 22.2
  • 91. N23 = 20.8 An example of abnormal IAD Amplitude= 77.81 uv Rt P13 = 12.2 N23 = 22.0 IAD = 0.45 Lt Amplitude= 29.56 uv P13 = 14.2
  • 92. VEMP amplitude depends on: 1. Saccular function 2. Stimulus intensity, air-conduction 3. Electrode conduction & location 4. Linearly increases with the EMG level
  • 93. Clinical utility of VEMP testing : sacculo-vestibular nerve function. assessment of vestibular nerve function: .1 acoustic neuromas – vestibular neuronitis – multiple sclerosis – diagnosis of superior semicircular canal dehiscence .2 syndrome, evaluation of Menière's syndrome .3 Sensori-neural hearing loss. .4
  • 94. VEMPs in a large Vestibular Aqueduct Most common anomaly Sudden fluctuation in pressure: 1. progression of SNHL after head trauma, 2. VEMP has greater amplitude and lower threshold (Sheykholesami et al, 2004).
  • 95. VEMP in diagnosis of Superior Canal Dehiscence Syndrome Rare • a ‘third window’ : • pseudo-conductive HL, ABG at – low frequencies, Tullio phenomenon of – acoustically evoked vertigo & nystagmus, VEMP : – increased amplitudes – lowered threshold (70 dB) – (Colebatch et al., 1998; Streubel et al., 2001 Brantberg et al., 1999; Ostrowski et al., 2001 Minor et al., 2003; Mikulec et al., 2004).
  • 96. Chronic otitis media • Chronic OM could delay and reduce the energy transfer of sound to the inner ear. • Improvement of postoperative VEMP response rate and p13 latencies in the patients with and without improvement in postoperative 500 Hz - ABG, provide evidence that the sound energy inducing a VEMP might be different from the energy producing the auditory perception (Wang et al., 2008).
  • 97. Migraine and its equivalents • Migraine: the most common cause of episodic vertigo in children. • Allena et al., (2007) postulated that VEMP abnormalities in migraine are due to reduced serotonergic control of the reflex circuit, in particular of the vestibular nuclei. • Benign Recurrent (Paroxysmal) Vertigo or benign recurrent vertigo (BRV): – a major cause of vertigo in children – 30% have abnormal caloric responses, – 50% have abnormal VEMP responses (Ozeki et al., 2008).
  • 98. Our VEMP Studies: in Migraineurs N23 normal P13 25% N23 VEMP abnormalities P13 75% delayed latencies of P13 and N23.
  • 99. VEMPs in Children with Cochlear Implants traumatic damage → absent VEMPs or decreased amplitude With CI on: electrical current spread at C level, apical channels → stimulates the IVN: present VEMPs or absent VEMPs if requiring higher current present VEMPs >50% intensities, but difficult (pain or facial nerve stimulation) (Jin et al., 2008).
  • 100. Thank you all