Voice Disorders in Medically Complex Children

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Voice Disorders in Medically Complex Children

• Roger C. Nuss, MD, FACS
• Geralyn Harvey Woodnorth, M.A., CCC-SLP
• Department of Otolaryngology and Communication
  Enhancement
• Childrens Hospital Boston
• Harvard Medical School

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The Medically Complex Child

• Can at first be a bit overwhelming
• Underlying medical problems may have similar
  effects on larynx and ability to phonate

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Themes in medically complex child

• Laryngeal / vocal fold scarring
• Vocal fold immobility

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Themes in medically complex child

• Compensatory techniques, vocal hyperfunction are
  common
• Overall weakness / debilitation may limit
  respiratory support
• Compromise of airway may limit ultimate vocal
  outcomes

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Case Example

• Teenager with
• Dev. Delay
• Hypotonia
• Subglottic stenosis
• Prior LTR
• Replacement of trach
• Cricoarytenoid fixation

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What range of medical issues are seen in the
complex patient ?

• Usually combination of problems related to
• Prematurity
• Complex congenital heart disease
• Genetic disorder
• Autoimmune disease
• Iatrogenic

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Congenital Laryngeal Disease

• Laryngeal web
• Laryngeal stenosis
• Laryngeal cleft

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Complex Congenital Heart Disease

• Tetralogy of Fallot
• Right sided arch
• Double arch
• Single ventricle

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Pulmonary Disease

• Bronchopulmonary dysplasia
• Chronic lung disease of prematurity
• Congenital lobar emphysema
• Restrictive lung disease
• Asthma

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Case Example

• School age boy with
• Asthma
• Obesity
• Severe OSA
• GERD
• Polypoid corditis

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Cystic fibrosis

• Pulmozyme related vocal fold polyposis
• Poor pulmonary reserve, breath support
• Hemoptysis

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Vocal Fold Palsy / Paresis

• Idiopathic - congenital
• Neurologic
• Chiari malformation
• Hydrocephalus
• CNS injury
• Stroke

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Vocal Fold Immobility - Iatrogenic

• Cardiac surgery
• PDA ligation
• TOF repair
• VSD repair
• Tracheo-esophageal fistula repair
• Vagal nerve stimulator

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Cricoarytenoid ankylosis

• Prolonged intubation
• Prolonged immobility ? fixation

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Autoimmune related disease

• Crohns disease
• Juvenile rheumatoid arthritis
• Wegeners disease

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Case Example Lipoid Proteinosis

• Teenager with longstanding coarse low pitched
  voice quality
• Poor intelligibility on telephone
• No OSA

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How do Complex Medical Problems affect the Voice ?

• Pulmonary reserve
• Breath support
• Incompetent glottis
• Inability to adduct vocal folds
• Decreased phonation time
• Supraglottic compensation / hyperfunction

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How do Complex Medical Problems affect the Voice ?

• Inflammatory changes of glottis
• GERD / LPR

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How do Complex Medical Problems affect the Voice ?

• Scarring of glottis
• Prior surgery
• Recurrent procedures for laryngeal papillomas

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What are reasonable surgical interventions ?

• Improve the Airway
• Subglottal stenosis repair
• Repair glottic web
• Management of RRP
• Hemangioma management
• Vascular malformations of larynx

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Surgical Interventions

• Improve glottal closure
• Reduce risk of aspiration
• Improve subglottal pressure
• Improve vocal fold mucosal wave entrapment
• Techniques
• Laryngeal framework surgery
• Injection medialization

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Injection Medialization Laryngoplasty
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Surgical Interventions

• Improve vocal fold surfaces
• Prevention
• Treatment of scarring
• Vibratory characteristics

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Medical Interventions

• Control of asthma
• Recognition treatment of GERD
• Allergy management
• Autoimmune diseases

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Why does surgery alone not correct the voice
disturbance ?

• A childs internal set point for their vocal
  output may not be the desired goal.
• Children with congenital laryngeal pathology may
  never had produced a normal voice
• Vocal compensatory techniques may need to be
  unlearned

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Effective voice treatment with medically complex
children requires a team approach

• SLP
• ORL
• Related disciplines
• Pulmonary
• GI
• Cardiology
• Etc.

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Voice Evaluation

• History
• Hearing
• Medical evaluation
• Laryngeal structure and function
• Instrumental measurements
• Acoustic
• Aerodynamic
• Videostroboscopy

• Speech mechanism
• Respiration
• Phonation
• Resonance
• Articulation
• Perceptual evaluation
• CAPE-V
• Quality of life index
• Parent/child perspective

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CAPE V MI MO SE SCORE     Overall
C I /100 Severity Roughness
C I /100 Breathiness C I
/100 Strain C I
/100   Pitch C I
/100   Loudness C I /100
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Recurring findings around voice disorders in
medically complex children
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Primary findings.

• Vocal hyperfunction
• Incomplete glottic closure
• Dyscoordination respiration/phonation

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Post Surgery

• Surgery by itself is not a fix for longstanding
  voice difficulties

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Post Surgery

• Residual limitations
• Improved capacity yet insignificant adaptation
• Learned behavior is retained,
• muscle memory

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Behavioral Voice Treatment

• Education and support
• Eliminate maladaptive behaviors
• Coordination respiration/phonation
• Vocal improvement
• Environmental enhancements

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Compensatory Behaviors

• Eliminate maladaptive behaviors
• Buccal speech
• Phonation on inspiration
• Ventricular phonation
• Non-compulsory vs necessary compensation

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Respiration

• Coordination respiration / phonation
• Phonation on exhalation
• Replenishing breaths
• Phrasing

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Voice Enhancement

• Improved quality
• Reduce vocal hyperfunction as possible
• Optimum, most efficient vocal function
• Increased vocal range and flexibility

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Voice Therapy Techniques

• Chant talk, singing
• Chewing
• Forward focus/resonant voice therapy
• Lip, tongue trills
• Humming, nasal consonants
• Kazoo-like productions
• Phonation on inhalation
• Vocal function exercises
• Sustained, smooth production of vowels
• Pitch glides
• Yawn-Sigh

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Comfortable Therapy Environment

• Exploration
• Try new things
• Follow the leader
• Encouragement / Support
• Gradually shape improved production

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Feedback

• Specific feedback
• Describe what you feel / hear
• Visual and/or auditory feedback

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Goal Directed Voice Therapy

• Identify / define target voice
• Establish goals across task hierarchy
• Train self-evaluation
• Plan for generalization
• Goals for increased vocal range and flexibility

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ConsiderationPrinciples of Motor Learning

• High number responses
• Intense repetitive practice
• Move from blocked training to randomized trials
• Progress through a hierarchy
• Give specific feedback
• Build in success

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Treatment A Dynamic Process

• Target voice may change over time
• Surgery
• Refinement of best voice
• Maturity

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Environment Enhancement

• Accommodations
• Amplification
• Positioning / Seating
• Control environmental noise

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EG

• History of L-transposition of the great arteries,
  status post double switch procedure at 18 years
  of age
• Weak breathy strained voice quality after
  surgery
• Left vocal fold palsy - wide paramedian position.
  

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EG

• Voice pre-surgery
• CAPE-V
• Overall 56
• MPT 9 seconds
• Voice post-surgery
• CAPE-V
• Overall 15
• MPT 18 seconds

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EG

• Aerodynamic measurements post surgery
• Mean airflow .16 lit/sec (lt.20)
• Mean peak air pressure 7.54 cm H20 (4-8)
• Aerodynamic Resistance 45 (31-45)

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DW

• Former 28 week preemie
• Left vocal fold palsy dx at 11 years
• Suspect longstanding hyperfunction observed 7
  years before
• Weak, breathy voice quality
• Vocal hyperfunction

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DW

• Left vocal fold fat injection medialization
• Pre-post perceptual and acoustic measures
  essentially unchanged
• Voice therapy undertaken

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CASE ZR

• History of tracheoesophageal fistula, repaired as
  a newborn infant.
• Status post prior LTR, anterior graft
• Bilateral VF immobility, only limited movement R
  arytenoid
• Complete glottic closure and a fairly good voice
  quality for a brief duration
• One month post decannulation

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CASE ZR

• Sustained /a/
• Speech

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JF

• Prematurity 24 weeks
• Subglottic stenosis
• Tracheostomy at 81 days
• s/p LTR, anterior and posterior grafts
• s/p cordotomy R TVC
• Long term plan for decannulation

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JF

• Oral communicator using buccal speech

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JF

• Communication options discussed
• Immediate therapy
• Reduce excessive strain
• lip synch
• Progressed well with artificial larynx training
  and articulation therapy

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JF

• Decannulation accomplished
• Initially aphonic
• continued with artificial larynx
• Gradually established functional voice

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GC

• Congenital glottic and subglottic stenosis
• Status post LTR
• ADHD

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GC

• Before voice therapy
• CAPE-V Overall Rating 72
• Low pitched, harsh voice
• Variable loudness
• Visible neck tension
• After short course of therapy
• CAPE-V 53
• Reduced jitter