Understanding Pediatric Voice Disorders

Key Glossary Terms

Stridor
Noisy breathing

Larynx
Highly specialized structure atop the windpipe responsible for sound production, air passage during breathing and protecting the airway during swallowing

Glossary

This section contains information on the following:

• Voice disorders in newborns
• Voice disorders in children

Voice Disorders in Newborns

In a newborn, an abnormal cry and/or noisy breathing (stridor) indicate problems in the voice box (larynx). Stridor may accompany potentially life-threatening conditions.

Red Flag

• Noisy breathing (stridor) is a sign of obstruction or narrowing of the laryngeal or tracheal parts of the airway.
• Stridor is a sign of difficulty passing air.
• Any breathing difficulty needs immediate medical attention.

• Anything that creates a blockage in the breathing passages causes turbulence in the passage of air, which then makes breathing noisy (stridor).
• Stridor can occur while breathing in, while breathing out, or during both phases of the breathing cycle.

Relationship of Stridor to Breathing Cycle	Most Likely Site of Problem
Stridor occurs only during inhalation	Above chest cavity Outside chest cavity
Stridor occurs only during exhalation	Trachea and bronchi – within the chest cavity, e.g., wheezing heard in asthma
Stridor occurs during both inhalation and exhalation	Fixed narrowing in the trachea or voice box – most commonly below the voice box (subglottis)

• Stridor is most often seen when infants are awake and active or feeding.
• When the child is awake and active, increased rates of breathing often exacerbate the turbulent airflow and noisiness.

• Occasionally, children are noisy breathers when sleeping.
• This is a sign of a partial airway obstruction that worsens when the hypopharynx and larynx relax.
• Any obstruction(s) in the upper part of the airway should be investigated.

• Noisy breathing that only occurs during feeding or sleep and that resolves during crying is a sign of blockage in the nasal cavity.
• This is called paradoxical stridor because the infant breathes better when active or agitated rather than when quiet.

Key Information

Obligate Nose-Breathers

• Infants are obligate nasal breathers (breathe through the nose only; do not know how to breathe through the mouth) until about the age of three to four months.
• Any blockage in the nose and nose cavity can cause significant noisiness – even gasping – in a newborn.

Effects on Feeding

• Any child with persistent progressive noisy breathing should be evaluated by a practitioner familiar with breathing disorders in children. Often, no therapy is required. However, the child should be followed closely to be certain that he or she grows and gains weight properly.
• The very young must learn quickly to coordinate the suck-swallow-breathe cycle. Minor changes in this cycle due to airway obstruction may interrupt their ability to feed. Failure to thrive or grow is an important indicator of the severity of their breathing disorder.

• Laryngomalacia, or "floppy larynx," is a very common cause of noisy breathing in children.
• Typically, these children develop noisy breathing during inhalation within weeks of birth. The noise they make is a high-pitched squeak on inhalation that worsens with activity such as crying or feeding.
• Floppy larynx is caused by collapse of the epiglottis and other structures above the vocal folds during inspiration. The exact cause of the collapse is unknown. (For more information, see Anatomy & Physiology of Voice Production.)

The diagnosis of laryngomalacia is made by history and physical examination. The physical examination includes visualization of the upper airway during breathing to confirm the diagnosis.

In some cases further examinations, such as x-rays of the neck and chest, may be required. A more detailed viewing of the throat and voice box area (laryngoscopy), esophagus (esophagoscopy), and trachea and bronchi (bronchoscopy) might also be required.

The floppiness of the larynx is transient and typically resolves during the first year of life. If the child is able to gain weight and grow at expected rates, no treatment is required. Frequent assessment of weight gain should be taken for this purpose.

Although rare, severe cases of laryngomalacia may need surgical correction of the floppy parts of the larynx to provide an adequate airway.

• Microlaryngeal Surgery: Specialized surgery, performed with microsurgical tools and through a surgical microscope (microlaryngeal surgery), may be required in severe cases to relieve the obstruction and allow the infant to coordinate the breathing and swallowing cycles. The surgery aims to carefully remove the floppy parts of the larynx that collapse into the voice box inlet (laryngeal inlet) while making sure that voice function remains intact.

Often, treatment for laryngopharyngeal reflux is instituted in children with laryngomalacia. (For more information, see adult Reflux Laryngitis.) Laryngopharyngeal reflux is often seen in infants with laryngomalacia for several reasons.

• With the increase in the "work" of breathing, air is often swallowed during feeding, causing distention of the stomach. This then makes it easier for stomach fluids to backflow toward the food pipe (esophagus), eventually reaching the lower throat (hypopharynx) and voice box (larynx).
• With the floppy larynx collapsing during inhalation, efforts to breathe can result in a lower pressure in the chest. This makes it easier for stomach fluids to backflow toward the food pipe, lower throat, and voice box.
• Edema (swelling) from reflux creates a narrower breathing passage, which exacerbates the laryngomalacia.

An abnormal cry in newborns has many possible causes. They can be broadly grouped into congenital or neurologic causes.

• Description: The vocal folds are supposed to separate during development. Rarely, this separation does not occur and a web of tissue is left across the laryngeal inlet. This is called a laryngeal web or glottic web.
• Effects on breathing: Breathing is possible because the back portion of the larynx, where the arytenoids cartilage attaches to the vibrating part of the vocal fold, is usually not associated with the web. The child's cry may be characterized as weak. Occasionally, stridor also results from the mild narrowing of the glottic inlet.
• Diagnosis: Generally, children with laryngeal webs are not diagnosed until after the first or second year of life. The diagnosis is made by diagnostic endoscopy. Radiograms may also be helpful to determine the length of the web. A lateral neck radiograph may show the classic "sail" sign.
• Treatment: Surgical correction usually entails expansion of the subglottic cartilage as well as lysis of the web across the membranous vocal fold.

• Description: Congenital cysts are mucus-filled sacs that develop on the vocal folds. Cysts usually result in abnormal cry.
• Effects on breathing: Children with congenital cysts may also develop some problems with breathing.
• Diagnosis: Diagnosis is usually possible with diagnostic endoscopy.
• Treatment: Surgical excision of the cysts is necessary, as they will generally enlarge as the sacs fill with mucus. Endoscopic approaches through the mouth as well as external approaches through the neck are possible. The recommended technique depends on the situation of the individual child.

• Description: Hemangiomas are benign tumors made up of blood vessels (capillaries) that uncontrollably increase in number. When hemangiomas occur on the vocal folds or in the area underneath the voice box, hoarse cry and/or noisy breathing usually result.
• Typical Behavior of Hemangiomas:
  • Begins to grow in the first months of life: Generally, the tumor begins to grow within the first weeks to months of life and may be associated with other vascular birthmarks.
  • Enlarges, then shrinks: The tumor usually will begin to shrink or regress after the first year of life. The timetable for shrinking/regression is not predictable, however.
  • Even if it becomes large, it is not cancerous: Although they increase in size and can become large, hemangiomas are not cancerous (malignant).
• Treatment: Treatment varies depending on the size, location, and progression of the hemangioma. Physicians and patients must balance the need to intervene with waiting, since hemangiomas eventually shrink. In particular, treatment plans must be made cautiously to prevent any damage to structures in the voice box (larynx) or the area underneath the voice box (subglottis).

Red Flag

Very Large Hemangiomas Can Cause Problems Related to Breathing, Heart Function, and Bleeding Control

• Breathing difficulties: The size of the hemangioma can block the airway passage, making it difficult to breathe.
• Heart workload increased: The heart has to work harder in order to circulate the blood in the hemangioma and throughout the body.
• Imbalances in the control of bleeding: The hemangioma can cause an imbalance in the different cells and substances involved in the control of bleeding, to the point that a bleeding disorder can result.

Key Information

Airway problems can occur. When airway problems do occur, there are some surgical techniques that can be used to try to avoid a tracheostomy when airway obstruction and breathing problems become evident.

Neurological causes of hoarseness in newborns are generally related to paralysis of one or both of the vocal folds.

• Description: Bilateral vocal fold paralysis causes more breathing problems than voice problems. Although the cry may be a little weak and breathy, noisy breathing is more evident.
• Reason for the breathing problems: Normal laryngeal nerves and muscles move the vocal folds to the closed position during crying, sound production (newborn), or speech/singing (older children and adults), and move the vocal folds apart during breathing. Two-sided vocal fold paralysis makes this impossible. The vocal folds stay close together during inhalation, causing a high-pitched, squeaky noise.
• Diagnosis:
  • Two-sided paralysis may look similar to laryngomalacia. Since the vocal folds are paralyzed in approximation, the voice may sound relatively normal and can fool even the experienced practitioner. Diagnosis relies on diagnostic endoscopy.
  • The vocal folds may have the appearance of motion but it will be paradoxical, moving in during inhalation (instead of opening) and out during exhalation. This is due to the movement of the air in and out of the windpipe (trachea) during breathing.
  • Once diagnosed, the cause of the paralysis should be investigated. Often, no specific cause can be found. The cause may be related to some birth trauma that has pinched the nerves to the larynx. In some cases, the problem might be in the brain or in the base of the skull; these cases require specialized tests in consultation with a medical brain-nerve specialist (neurologist).
• Treatment: Regardless of the cause, a little more than half of newborns with vocal fold paralysis require a breathing tube placed directly in the windpipe (tracheostomy) below the voice box where the paralyzed vocal folds are located. When these children have grown and vocal fold motion has not returned in two or three years, other surgical procedures are possible to widen the airway and remove the tracheotomy tube.

• Description: Paralysis of only one vocal fold will result in a very weak and breathy cry. Generally, no breathing or swallowing problems will occur. Children are often capable of overcoming the lack of motion of one vocal fold, and their voice will perform very well in the long run. Infrequently, problems will occur with swallowing due to aspiration.
• Diagnosis: Determining the cause of the paralysis is important. As with bilateral vocal fold paralysis, the actual cause may never be known, but tumors along the path of the laryngeal nerve or impingement of the nerve by other structures should be ruled out.
• Treatment: Procedures to bring the vocal folds closer together, while keeping them far enough apart to allow good breathing, are possible. The larynx in children grows, however, and the correction may become inadequate over time.

Voice disorders in children are usually described as "hoarseness." Hoarseness is a rough character or quality of the voice that may make it more difficult to be heard and understood. Hoarseness may be associated with intermittent complete loss of voice, typically after extensive use (i.e., at the end of the school day).

Hoarseness is actually very common in children and is usually caused by abnormalities of the vocal folds. It can cause significant psychosocial and academic problems. Children are often teased and ostracized for things that make them different from their peers. Performance in school can also be hampered if students are unable to comfortably express themselves verbally.

Red Flag

• An estimated million-plus children nationwide suffer from voice disorders.
• Many of these children are not given the medical evaluation or attention they need to help them achieve improved voice function.

Fortunately, most hoarse children have benign and often easily treated laryngeal disorders causing their voice disturbance.

Hoarseness that persists for months or progresses with time needs to be evaluated to exclude the possibility of a life-threatening condition.

• Medical evaluation is best done in conjunction with a speech language pathologist.
• A complete history and physical examination is necessary.
• Visualization of the larynx is often required to determine the exact cause of the hoarseness.

Inflammation of the vocal folds is a very common cause of hoarseness in children. The two main causes of inflammation are:

• Viral infections that cause upper respiratory tract infections usually also cause swelling of the voice box or larynx. This is probably the most common cause of hoarseness in children.
• Typically, a viral upper respiratory tract infection is followed by hoarseness.
• It is unusual to have breathing or swallowing problems related to the infection, and the hoarseness is usually short-lived.

• Inflammation of the voice box due to the backflow of stomach fluids into the larynx is another common cause of hoarseness in children.
• LPR has been well recognized in adults but not in children. In children, the diagnosis of reflux-induced laryngitis is not easy to make with objective measures.
• As in adults, a trial of reflux therapy without an absolute diagnosis of gastroesophageal reflux is often necessary. If the hoarseness or inflammation seen on the larynx improves while the reflux is treated, reflux laryngitis can be inferred. However, it is difficult to confirm that adequate anti-reflux treatment has been achieved.
• In the studies that have been performed in adults, more aggressive treatment than typical for the treatment of heartburn-type reflux or gastroesophageal reflux (GERD) was required to resolve hoarseness.

Nodules on the vocal folds, also known as "screamer's nodules," are another very common cause of hoarseness in children. Typically, young boys who use their voices aggressively during play develop a thickening along the surface of each vocal fold at the junction of the middle one-third and front one-third. Children who are not necessarily aggressive or abusive with their voices may also develop these thickenings.

The actual cause of the nodules is unknown. The leading causes thought to induce the inflammation that leads to nodule formation are voice abuse or inflammation from reflux of gastric contents.

Diagnosis is made by visualizing the vocal folds and identifying benign thickening on the vocal folds.

The first lines of treatment are voice therapy and perhaps treatment for gastroesophageal reflux (with either behavioral management to decrease the likelihood of reflux or medications to decrease the caustic nature of the contents refluxed). Generally, nodules decrease in size and symptoms as the child ages. As the voice deepens and the vocal fold lengthens, the nodules generally improve. Again, the exact reason for this development is poorly understood but is most likely due to changes in the vibrating structures of the vocal fold as it lengthens with age.

• Correct Diagnosis Critical: Nodules respond to voice therapy, while cysts and polyps do not.
  • Lesions of the vocal folds can be misdiagnosed as vocal nodules and may in fact be a vocal fold polyp or cyst. Vocal fold polyps and cysts do not improve with voice therapy and thus may require surgical excision at the appropriate time in the child's maturity-development time line. If a patient is able to tolerate videostroboscopy, a confident diagnosis of vocal fold nodules versus other vocal fold lesions can be made.
• Decision for Voice Therapy Complex: Once the diagnosis of vocal nodules has been confidently made, the decision for proceeding with voice therapy is a complex decision based on a number of factors.
  • severity of the child's voice problem (frequency of voice loss, amount of negative attention due to hoarseness, negative social pressure in the classroom, etc.)
  • child's maturity
  • child's personal interest in improving voice
• Motivation of Child Critical to Success of Therapy:
  • Voice therapy is a treatment that needs to be taught and then carried over into the child's daily life. Concepts taught in voice therapy can be supplemented and reinforced by the parents, but the primary motivation for improving the voice must stem from the child or voice therapy will be unsuccessful. Thus, the maturity of the child is essential in deciding whether voice therapy should be used.
  • After the diagnosis of vocal fold nodules have been made in a young child, it is often best to wait before beginning voice therapy treatment until the child notes a functional limitation with the voice and is appropriately mature and motivated to comply with the recommendations and teachings that occur during voice therapy.

• Infrequently, a child may continue to have significant hoarseness despite mastering the voice therapy required for good voice use and receiving adequate treatment for reflux. These children may benefit from removal of the thickened portion of the vocal fold. This may make sound production easier by decreasing the mass of the vibrating vocal fold.
• Care must be taken, however, not to damage the underlying structures of the vocal fold, which could cause permanent scarring and perhaps lifelong hoarseness. New techniques for removing the superficial nodular thickening without damaging the underlying support structure of the vocal fold are being created.

• Cysts in the vocal fold are uncommon but may be mistaken for vocal nodules. It is important to try to discern between the two because cysts of the vocal fold (intracordal cysts) generally do not resolve without surgical intervention.
• Voice therapy may help to improve the voice but removal of the cyst is often required.
• At times the only way to correctly distinguish between the two is during direct laryngoscopy. Under a general anesthetic, the vocal folds can be palpated to determine if the thickening is from a nodule or a cyst.

• RRP is caused by a viral infection by the human papilloma virus (HPV). Viral infection with HPV results in small wart-like growths (papillomas) on the vocal folds.
• Vocal fold growths can cause hoarseness. They can also cause significant breathing problems if they become bulky and obstruct the breathing passage.
• There are currently no good medical treatments for the viral papilloma. Several anti-viral medications are being investigated, however.
• Typically, the growths require physical removal either with a laser, cold steel micro instruments, or with a laryngeal debrider instrument.
• Some immunological changes occur as a child ages, which may bring the viral infection under control. Until that time, the treatment standard is periodic removal of the papillomas to maintain the breathing passage without irreversibly scarring the larynx.

• Tumors of the larynx are extremely rare but should be considered in any child with persistent or progressive hoarseness.
• Visualization of the larynx is the best way to determine the presence or absence of a tumor of the larynx.
• If a tumor is present, biopsy is often required for diagnosis before a definitive treatment plan can be established.

• Placement of a tube through the larynx (endotracheal intubation) to help with breathing either during surgery or for other major illnesses may damage the voice box, causing hoarseness.
• Injuries may include:
  • Paresis of the vocal fold
  • Dislocation of the arytenoid cartilage of the vocal fold
  • Narrowing from scar formation in the area just under the vocal folds (subglottis)
• Hoarseness that persists after endotracheal tube placement should be evaluated and will likely require visualization of the larynx either in the office and/or operating room.
• Depending on the nature and cause of hoarseness, treatment is possible.

• Congenital abnormalities of the larynx can cause hoarseness in older children. These abnormalities may also cause breathing troubles and often present in the very young.
• Laryngeal web: Occasionally a web across the front portion of the larynx (laryngeal web) may not cause breathing problems but can cause hoarseness. The diagnosis of a laryngeal web relies on visualization of the voice box. The web at times may be divided through the mouth with endoscopic techniques but often requires dividing the subglottis as well with an incision through the neck. This procedure allows enlargement of the cricoid ring, if it is affected.