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    Vestibular 

    neuritis in 

    children 

    and 

    adolescents: 

    Clinical 

    features

    and 

    recovery§

     Jacob R. Brodsky a,b,*,  Brandon A. Cusicka,b, Guangwei Zhoua,b

    aDepartment   of   Otolaryngology  and  Communication  Enhancement   (J.R.B.,  B.A.C.,  GW.Z.),  Boston  Children’s  Hospital,  Boston,  MA,  USAbDepartment   of   Otology  and  Laryngology  (J.R.B.,  GW.Z.),  Harvard  Medical  School,  Boston,  MA,  USA

    1.  Introduction

    Vestibular  neuritis  (VN)  is  an  acute  inflammatory  process

    typically  affecting  the  superior  vestibular  nerve  that  results  in  an

    acute,  unilateral,  peripheral  vestibular  loss  that  preferentially

    affects 

    the 

    utricle 

    and 

    the 

    anterior 

    and 

    lateral 

    semicircular 

    canals.

    The  initial  clinical  presentation  is  characterized  by  the  sudden

    onset  of   rotary   vertigo,  imbalance,  and  nausea,  which  generally

    lasts  for  a  few  days.  The  specific  etiology  of   VN  remains

    controversial, 

    though 

    an 

    association 

    with 

    herpes 

    simplex 

    virus

    infection  has  been  demonstrated  [1–4]. The  presentation,  recovery,

    and  treatment  of   VN  in  adults  have  been  well  described  [4–7]. A

    high  incidence  of   incomplete  recovery  (IR)  has  been  described  in

    adults,  which  is  associated  with  long  term  residual  vestibular

    symptoms   and  deficits  after  a  case  of   VN  [7]. Vestibular

    rehabilitation 

    (VR) 

    and 

    oral 

    corticosteroids 

    have 

    both 

    been 

    shown

    efficacy  at  reducing  the  risk  of   IR   following  VN  in  adults  [8–10].

    Epidemiological  reports  of   pediatric  vertigo  generally  report   VN

    to  represent  between  1  and  5%  of   cases  of   pediatric  vertigo  [11–15],

    though 

    descriptions 

    of  

    the 

    clinical 

    features, 

    vestibular 

    test 

    findings,

    and  recovery  course  of   VN  in  pediatric  patients  has  been  very

    limited  and  have  also  excluded  adolescents  [16–18]. Children  are

    thought  to  recover  from  acute  vestibular  losses  more   efficiently

    than 

    adults 

    [19], but 

    the 

    risk 

    of  

    IR  

    after 

    VN 

    across 

    the 

    pediatric 

    age

    spectrum 

    is 

    still 

    unclear. 

    The 

    goal 

    of  

    this 

    study 

    was 

    to 

    describe 

    the

    clinical  features,  vestibular  test  findings,  and  recovery  course  of 

    children  and  adolescents  diagnosed  with  VN.

    International   Journal  of   Pediatric  Otorhinolaryngology  83  (2016)   104–108

    A  R   T  I   C  L   E  I   N  F   O

     Article history:

    Received 

    December 

    2015Accepted  23   January  2016

    Available online 1 February 2016

    Keywords:

    Vestibular  neuritis

    Vestibular  neuronitis,  Pediatric  dizziness,

    Pediatric  vestibular  testing,  Childhood

    vertigo,  Video  head  impulse  test

    A  B  S  T  R   A  C  T

    Objective: 

    Describe the clinical presentation and recovery of vestibular neuritis in children and

    adolescents.Study design: Retrospective case series.

    Setting: Pediatric tertiary care center.

    Subjects and methods: Eleven patients diagnosed with vestibular neuritis were identified from a

    database of 301 patients evaluated at our pediatric vestibular clinic from January 2012 through January

    2015. Medical records were reviewed to determine clinical presentation, vestibular testing results,

    treatment, and recovery. Incomplete recovery was defined as residual dizziness or imbalance at most

    recent follow-up >30 days from symptom onset.

    Results: Patients were 5–19 years old (mean 13.1 5.34)  and  included  6  boys  and  5  girls.  All  presented

    with 

    a sudden 

    rotational 

    vertigo, 

    imbalance, 

    and 

    nausea 

    for 

    an 

    average 

    of  

    10 

    dayswithout 

    other 

    associated

    symptoms.  Testing   included  rotary  chair  (8 of  9  abnormal),  caloric  (2 of  2  abnormal),  video  head   impulse  (5  of 

    8  abnormal),  subjective  visual   vertical  (4  of   8  abnormal),  and  cervical  vestibular   evoked   myogenic  potential

    (0 

    of  

    abnormal) 

    tests. 

    All 

    patients 

    with 

    incomplete 

    recovery 

    (n = 

    4; 36%) 

    were  15 years  old  at  symptom

    onset.   All patients with   incomplete  recovery  that  underwent  vestibular   rehabilitation   (n = 2) initiated  it 90

    days 

    from 

    symptom 

    onset, 

    while 

    out 

    of  

    patients 

    with 

    complete 

    recovery 

    that 

    underwent 

    vestibular

    rehabilitation 

    initiated 

    it  14 days

     

    from 

    symptom 

    onset. 

    Two 

    patients 

    received 

    oral 

    steroids, 

    neither 

    of whom 

    had 

    incomplete 

    recovery.

    Conclusion: Vestibularneuritis should be considered in pediatricpatients with vertigo andmay result in

    longstanding symptoms, particularly in adolescents. The treatment of pediatric vestibular neuritis with

    rehabilitation and steroids deserves further study.

    2016 Elsevier Ireland Ltd. All rights reserved.

    § Presented  at  the  American  Academy  of   Otolaryngology—Head  &  Neck  Surgery

    Annual  Meeting,  Dallas,  TX,  U.S.A.,  September  27,  2015.

    *  Corresponding   author  at:  Department  of   Otolaryngology  and  Communication

    Enhancement,  Boston   Children’s  Hospital,  300  Longwood   Avenue,  Boston,   MA

    02115,   USA.  Tel.:  +1  781  216  2849;   fax:  +1  781  216  3155.

    E-mail  address:   [email protected]  (J.R.  Brodsky).

    Contents 

    lists 

    available 

    at 

    ScienceDirect

    International Journal of Pediatric Otorhinolaryngology

    jo urn al  hom ep ag e: www.els evier  .c om/locat  e/ i jp  o r  l

    http://dx.doi.org/10.1016/j.ijporl.2016.01.027

    0165-5876/ 2016  Elsevier  Ireland  Ltd.  All  rights  reserved.

    http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027mailto:[email protected]:[email protected]://www.sciencedirect.com/science/journal/01655876http://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://www.elsevier.com/locate/ijporlhttp://dx.doi.org/10.1016/j.ijporl.2016.01.027http://dx.doi.org/10.1016/j.ijporl.2016.01.027http://www.elsevier.com/locate/ijporlhttp://www.sciencedirect.com/science/journal/01655876mailto:[email protected]://dx.doi.org/10.1016/j.ijporl.2016.01.027http://crossmark.crossref.org/dialog/?doi=10.1016/j.ijporl.2016.01.027&domain=pdfhttp://crossmark.crossref.org/dialog/?doi=10.1016/j.ijporl.2016.01.027&domain=pdf

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    2.  Materials  and  methods

     2.1.  Patients

    This  retrospective  study  was  approved  by  the  Institutional

    Review 

    Board 

    of  

    Boston 

    Children’s 

    Hospital. 

    We 

    retrospectively

    reviewed 

    our 

    internal 

    database 

    of  

    all 

    patients 

    seen 

    at 

    the 

    Balance

    and  Vestibular  Program  clinic  at  Boston  Children’s  Hospital  from

     July  2012  to   January  2015  to  identify  all  patients  19  years  of   age

    that 

    were 

    diagnosed 

    with 

    VN. 

    Clinical 

    presentation 

    consistent 

    with

    VN 

    was 

    defined 

    as 

    single, 

    isolated 

    episode 

    of  

    acute 

    onset 

    rotary

    vertigo  and  imbalance  lasting  several  days  followed  by  either

    spontaneous  complete  symptom  resolution  or  by  a  prolonged

    period 

    of  

    general 

    disequilibrium 

    with 

    or 

    without 

    imbalance 

    lasting

    for 

    several 

    days 

    afterward. 

    Vestibular 

    testing 

    results 

    consistent

    with  VN  were   defined  by  evidence  of   an  acute,  unilateral,

    peripheral  vestibular  loss  in  the  absence  of   hearing  loss.  The

    electronic 

    medical 

    records 

    of  

    all 

    included 

    patients 

    were 

    reviewed

    to 

    determine 

    clinical 

    presentation, 

    diagnostic 

    test 

    results, 

    and

    treatment  responses.  Incomplete  recovery  (IR)  was  defined  as  the

    presence  of   residual  dizziness  and/or  imbalance  symptoms   at  the

    most 

    recent 

    follow-up 

    appointment 

    >30 

    days 

    from 

    symptom

    onset.

    All  subjects  had  a  normal  otologic  and  neurologic  examinationat  the  time  of   presentation  with  the  exception  of   variable

    combinations 

    of  

    the 

    following: 

    Presence 

    of  

    spontaneous 

    nystag-

    mus, 

    abnormal 

    head 

    impulse 

    test, 

    abnormal 

    head 

    shake 

    maneuver,

    abnormal   Romberg   test,  abnormal  Fukuda  march  test,  and/or

    positive  findings  on  ipsilateral  Dix-Hallpike  maneuver.  Patients

    with 

    an 

    abnormal 

    Dix-Hallpike 

    maneuver 

    did 

    undergo 

    an 

    Epley

    maneuver 

    on 

    the 

    affected 

    side 

    prior 

    to 

    undergoing 

    vestibular

    testing.  No  subjects  had  a  head  injury  immediately  prior  to  onset  of 

    symptoms.

     2.2.  Vestibular   testing 

    All 

    patients 

    completed 

    variable 

    combination 

    of  

    objective

    vestibular  and  balance  tests.  All  testing  was  conducted  in  ourclinical  vestibular  laboratory  at  the  Balance  and  Vestibular

    Program  at  Boston  Children’s  Hospital  by  a  licensed  audiologist

    (GWZ), 

    with 

    the 

    support 

    of  

    trained 

    assistant. 

    Testing 

    was

    performed 

    at 

    the 

    time 

    of  

    initial 

    patient 

    presentation 

    to 

    our 

    clinic.

    Sinusoidal  harmonic  rotary   chair,  videonystagmography  (VNG),

    static  subjective  visual  vertical  (SVV),  and  binaural,  bithermal

    water 

    caloric 

    tests 

    were 

    performed 

    using 

    Micromedical 

    equipment

    (System 

    2000 

    and 

    VisualEyes 

    with 

    AquaStim; 

    Micromedical

    Technologies,  Chatham,  Illinois).  Computerized  dynamic  postur-

    ography  (CDP)  was  conducted  with  NeuroCom  SMART  EquiTest,

    and 

    cervical 

    vestibular 

    evoked 

    myogenic 

    potential 

    testing 

    (cVEMP)

    was 

    recorded 

    with 

    Bio-logic 

    Navigator 

    Pro 

    Evoked 

    Potential

    system  (Natus  Medical  Inc,  San  Carlos,  California).  Video  head

    impulse 

    testing 

    (VHIT) 

    was 

    performed 

    using 

    the 

    ICS 

    Impulsesystem

     

    (GN 

    Otometrics, 

    Denmark). 

    The 

    majority 

    of  

    patients

    underwent 

    rotary 

    chair 

    testing 

    instead 

    of  

    caloric 

    testing, 

    since

    we  prefer  rotary   chair  testing  for  pediatric  patients  in  our  lab  as  it  is

    better  tolerated.  SVV  testing  was  not  administered  to  children  6

    years 

    old 

    due 

    to 

    their 

    limited 

    ability 

    to 

    follow 

    the 

    instructions

    adequately.

    Balance  and  vestibular  testing  results  were  compared  to

    manufacturer-supplied  age-specific  norms,  with  the  exception  of 

    the 

    cVEMP, 

    SVV, 

    and 

    VHIT 

    tests, 

    which 

    were 

    compared 

    to 

    our

    normative 

    pediatric 

    data 

    established 

    in 

    prior 

    studies 

    [20–22].

    Rotary   chair  results  were  considered  abnormal  if   gains  for  all

    frequencies  tested  were  below  the  age-adjusted  normal  range,

    phase 

    leads 

    for 

    the 

    lowest 

    frequencies 

    tested 

    were 

    above 

    the 

    age-

    adjusted 

    normal 

    range, 

    and 

    the 

    time 

    constant 

    was 

    less 

    than 

    12 

    s.

    Caloric  testing  was  considered  abnormal  if   a  reduced  vestibular

    response 

    of   20%  was  observed  for  the  affected  ear.  VHIT   testing

    was  considered  abnormal  if   the  vestibulo-ocular  reflex  gain  was

    30

    days 

    from 

    symptom 

    onset. 

    All 

    patients 

    were 

    first 

    diagnosed 

    with 

    VN

    upon 

    initial 

    presentation 

    to 

    our 

    clinic, 

    though 

    many 

    had 

    been

    followed  my  multiple  outside  providers  for  their  symptoms   prior  to

    receiving  the  diagnosis.  All  4  patients  that  were  diagnosed  with

    concurrent  BPPV  also  reported  symptoms  of  worsening  rotary  vertigowith

     

    the 

    affected 

    ear 

    down 

    when 

    supine 

    in 

    bed, 

    which 

    resolved

    following  an  Epley  repositioning  maneuver   in  the  clinic   directed  at

    the  affected  ear.  All  patients  had  a  normal   audiogram.   Testing  results

    are 

    outlined 

    in 

    Table 

    2. 

    IR  

    occurred 

    in 

    patients 

    (36%), 

    all 

    of  

    whom

    were 

    >15 

    years 

    old 

    at 

    time 

    of  

    symptom 

    onset. 

    month 

    course 

    of 

    vestibular  rehabilitation  (VR)  was  completed  by  6   patients  immedi-

    ately  following  their  initial  evaluation  at  our  program.  Three  of   the

    patients 

    that 

    underwent 

    VR  

    initially 

    presented 

    to 

    us 

    within 

    14 

    days 

    of 

    symptom 

    onset, 

    and 

    none 

    of  

    these 

    patients 

    experienced 

    IR. 

    The 

    3

    other  patients  that   underwent  VR   initially  presented  to  us  at  54,  90,

    and 

    180 

    days 

    after 

    symptom 

    onset, 

    respectively, 

    the 

    latter 

    of  

    which

    continued 

    to 

    have 

    some 

    persistent 

    symptoms 

    even 

    after 

    completing

    VR.  Two patients  received  a  course of   oral prednisone  starting  at 6   and

    7  days  after  symptom  onset,  respectively,  neither  of   whom  had  IR,though

     

    both 

    of  

    these 

    patients 

    also 

    underwent 

    VR.

    4. 

    Discussion

    Adult  studies  consistently  demonstrate  VN  to  be  the  third  most

    common   cause  of   adult  vertigo,  making  up  approximately  7%  of 

    adult 

    vertigo 

    cases 

    [4]. 

    General 

    epidemiological 

    studies 

    of  

    pediatric

     Table  1

    Patient  demographics.

    Age,  years

    Mean  SD (range) 13.1 5.34 (4–19)

    Sex,  n

    Male  6

    Female  5

    Preceding  URI

    Yes  6

    No  5

    Secondary  diagnoses,  n

    BPPV  4

    Migraine  2

    Epilepsy  1

    BPPV,  benign  paroxysmal  positioning  vertigo;  URI,  upper

    respiratory 

    tract 

    infection.

     J.R.  Brodsky  et   al.  /   International   Journal  of   Pediatric   Otorhinolaryngology  83  (2016)  104–108  105

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    vertigo 

    describe 

    VN 

    as 

    representing 

    between 

    and 

    5% 

    of  

    cases 

    of 

    pediatric 

    vertigo 

    [11–15]. Our 

    study 

    found 

    VN 

    to 

    make 

    up 

    3.6% 

    of 

    cases  of   pediatric  dizziness  or  vertigo  at  our  program.   These

    patients  were  identified  from  a  group  of   301  pediatric  patients  at

    our 

    program, 

    which 

    is 

    much 

    larger 

    group 

    than 

    that 

    described 

    in

    the 

    above-mentioned 

    epidemiologic 

    studies 

    of  

    pediatric 

    vertigo,

    with  the  exception  of   Wiener–Vacher’s  2008  study  of   2000children  with  vertigo,  which  described  a  rate  of   VN  of   5%  of 

    pediatric 

    vertigo 

    cases 

    [11]. No 

    patients 

    in 

    our 

    study 

    initially 

    came

    to 

    our 

    clinic 

    with 

    diagnosis 

    of  

    VN 

    made 

    prior 

    to 

    their 

    visit, 

    despite

    the  fact  that  patients  initially  presented  to  our  clinic  an  average  of 

    nearly  2  months  after  their  symptoms  began,  and  despite  the  fact

    that 

    the 

    majority 

    of  

    patients 

    had 

    previously 

    been 

    evaluated 

    by

    multiple 

    other 

    physicians. 

    This 

    may 

    have 

    resulted 

    from 

    lack 

    of 

    awareness  of   the  existence  of   VN  among  pediatric  healthcare

    providers, 

    an 

    assumption 

    that 

    VN 

    does 

    not 

    occur 

    in 

    pediatric

    patients, 

    or 

    lack 

    of  

    knowledge 

    about 

    how 

    to 

    clinically 

    assess 

    a

    patient  for  VN.  This  supports   the  need  for  increased  awareness  of 

    the  features,  assessment,  and  management  of   VN  among  pediatric

    healthcare 

    providers.

    Vestibular 

    testing 

    results 

    were 

    all 

    consistent 

    with 

    partial,

    unilateral,  peripheral  vestibular  loss  affecting  only  the  function  of organs  supplied  by  the  superior  vestibular  nerve.  In  adults  VN  has

    been 

    shown 

    to 

    typically 

    affect 

    only 

    the 

    superior 

    vestibular 

    nerve

    [5], 

    with 

    only 

    rare 

    cases 

    of  

    inferior 

    vestibular 

    nerve 

    involvement

    being  described  [23].  In  the  current  study  normal  posterior  canal

    function  on  all  subjects  that  underwent  VHIT   (Fig.  1) and

    consistently 

    normal 

    cVEMP 

    findings 

    indicate 

    the 

    absence 

    of  

    any

    cases 

    with 

    objective 

    evidence 

    of  

    inferior 

    vestibular 

    nerve 

    involve-

    ment.  An  association  between  VN  and  upper  respiratory  tract

     Table  2

    Vestibular  testing  results.

    Patient  Rotation  Calorics  (RVR)  VHIT  (canals  affected)  SVV  (deviation)  cVEMP

    1 (+)  Right  (20%)  (+)  Right  (2.58)

    2 (+)  (+)  Left  (LC)  (–)  (–)

    3 (+)  Right  (23%)  (+)  Right  (AC)  (–)

    4 (+)  (–)  (+)  Right  (3.18) (–)

    5 (+)  (–)  (–)

    6 (+)  (+)  Left  (LC  &  AC)

    (–) 

    (+) 

    Right 

    (AC) 

    (–) 

    (–)8 (+)  (+)  Right  (2.08) (–)

    9 (+)  (+)  Right  (AC  &  LC)  (+)  Right  (4.88) (–)

    10  (+)  (–)

    11  (+)

    (+),   abnormal  result;  (–),   normal  result;  RVR,  reduced  vestibular  response;  VHIT,  video  head  impulse  test;  SVV,  subjective  visual  vertical  test;  cVEMP,   cervical  vestibular

    evoked  myogenic  potential  test;  LC,  lateral  canal;  AC,  anterior  canal.

    Fig. 1.  Video  head  impulse  test  result  in  a  patient  with  left  vestibular  neuritis  demonstrating  reduced  vestibulo-ocular  reflex  gain  (blue  X’s,   left  column,  first   and  second  rows)

    and  the  presence  of   overt  corrective  saccades  (middle  column,  first   and  second  rows)  in  the  left  anterior  and  lateral  semicircular  canals  with  sparing  of   the  posterior  canal,

    representing 

    the 

    typical 

    isolated 

    involvement 

    of  

    the 

    superior 

    vestibular 

    nerve.

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