On The Matter Of Monsters

Angelika Scudamore - monsters under bed scene

http://www.angelikasillustrations.com: “monsters under bed scene”


Zane’s mother looked exhausted. I asked her if all was well.

“He won’t go to sleep unless I’m with him, he is taking forever to fall asleep and waking me up several times every night,” she sighed. “It is exhausting.”

“How come?” I asked, looking from the preschooler to his mother.

“It’s the monsters,” he chirped to clarify.

“For the hundredth time, Zane,” his mom exasperated, “there is no such things as monsters and there are certainly none in your room!”

“Is too!” his lower lip tightened in determination then began to tremble.

Zane’s mom took in a long breath and mouthed a silent “help.”

I smiled gently. The matter of monsters comes up often. Many young children–especially between four and six years of age–go through a period where they fear monsters. Under the bed, in the closet, behind the curtains/desk/wardrobe/chair, camouflaged among the stuffed animals on the top shelf … At the age where imagination and reality can merge and the veil between what’s real and what could be is thin, many children find the dark ominous and fear the parting with parents for the night and being left to their own thoughts and imagination. They are often too young to verbalize what it is they fear, exactly, but the feelings are still there: scary, dark, sneaky; the territory and making of monsters.

Scared or fearing to become so, they plead, coerce, and cry for their parents to stay and make sure they are okay.

Some are reassured by the adult checking under bed or dressers. For others, securing the closet door closed can suffice. However, for many, the fear remains in the ‘what if’ category: “what if the monster comes later?”, “what if the monster opens the door?”, “what if it is invisible and you can’t see it?”, “what if it just pretending to be my shoes but it will scare me later?”

Perception, reality, and belief make a sticky trio; and declaring monsters nonexistent rarely helps. To many children–as with Zane–this only makes the fear grow further and adds frustrated loneliness onto it, making nighttime doubly scary.

Zane’s mother needed her sleep. Zane needed his to feel safe. It was time to bring out the ‘big guns.’

I looked at the boy. A messy head of curls, brown piercing eyes under thick brows, a smattering of freckles on a button nose, wide lips, and a tongue that likes to slip out during speech and activity regardless of whether its presence is required (the tongue thrust being the main reason he sees me for speech-therapy).

The little boy regarded me. He needed to ascertain whose side I was on. “I have monsters,” he announced, “under my bed.”

“Yikes,” I replied. “This sounds scary.”

He smiled and turned to glare victoriously at his mommy.

She looked at me with uncertainty.

“You also see monsters!?” he checked, suddenly a bit wary of the possibility. Monsters being real is one thing. Monsters being REAL is quite another.

“Nah,” I shook my head. “But you say you do, so maybe they are there.”

He nodded quickly.

“What do they look like?” I wondered aloud.

“I don’t know!” he exclaimed. “They are hiding under my bed and it’s dark.” He followed that obvious fact with an ‘adults-can-be-so-thick’ look.

“Oh.” I demurred. “What if you turn on the light?”

“You can’t see them in the light. They do magic.”


“If I go to sleep by myself they will come and get me,” he warned. “Mommy says they not there but they are.”

“Well then,” I breathed. “I’m not in your house and I haven’t seen them, but just in case they are there, have you tried telling them you don’t want them there?”

“They don’t know English,” he responded.

“They don’t?” I let my voice rise some.

“No!” he explained, “they only speak Monster.”


He nodded sagely.

“…and they eat children,” he added for emphasis, then his eyes grew big with fright at the possibility of his own words and he backpedaled, “…um, maybe … if they really hungry.”

“We can’t let that happen,” I said.

He nodded again, reached for my hand.

I squeezed his little palm in reassurance. Children may be small but their fears can still be big, and their imaginations; bigger.

“Good thing we know what to do,” I stated.

He looked at me hopefully.

I pursed my lips in contemplation. “Have you tried Monster Spray?”

“Monster Spray?” This sounded intriguing.

“Yeah. They hate the stuff. Makes their noses itch.”

His eyes grew again, this time with wonder. He looked at his mom, clearly expecting her to know everything there is to know about sprays and all manner of remedies.

She raised her palms up in bewilderment and gave me an ‘I hope you know what you are doing’ glare.

“It works every time,” I reassured both of them.

“What’s Monster Spray?” Zane asked. “Mommy, you have to listen, too,” he ordered. “Because you didn’t learn it yet.”

I swallowed a chuckle. I was waiting to see how he would get back at her for not believing him that monsters waited under his bed waiting to eat children (maybe … if they really hungry…).

“It’s a spray and it makes monsters go away. It smells the same as an air freshener or perfume. The monsters don’t know the difference,” I said meaningfully. Mom’s eyebrows lifted and the corner of her month twitched a bit. Good. One aboard.

“Like in the bathroom?” Zane’s eyes narrowed suspiciously.

“Sort of. Doesn’t have to be the same one, though. You can pick any scent you like. They hate all of them. Makes their noses itch. Here is what you have to do. You listening?

He was.

“First, you find a spray that smells good to you. Mommy can help you choose. Next you make a sign that says “Monster Spray” and you tape it on the bottle …”

He nodded in approval. It was important to label things. Especially when it came to monsters.

“…and before you go to sleep you spray a bit under your bed, and if you want you can spray a little in the air, and that’s it. If the monsters are there they will say: ‘Oh, no, Monster Spray, we better come another day!’ and they’ll go away.”

Zane’s jaw hung open in delight. “For really?”

“Yep,” I nodded. “Works every time. If there are monsters there, they’ll run away from the monster spray.”

“What if they come tomorrow?”

“If they come another day, they’ll have to deal with more monster spray … and they’ll say: ‘Oh, no, Monster spray …”

“… better come another day!” he completed, his eyes shining.

“So we’ll have to do this forever?” Zane’s mom. I could sense her wariness about committing to nightly spray-bottle battles till Zane was in college.

“Oh, no,” I clarified. “You see, once you do it a few times, if the monsters come again they will say: ‘Oh no, more Monster Spray; we better go another way.’ They hate this stuff so much, they will tell all their monster friends to go another way!”

“Better go another way!” Zane clapped his hands, intoning, “Oh, no, Monster Spray; better go another way! Hey!” he paused, “Spray-way!” he lisped. “It rhyme!”

“It does indeed!”

“Spray, spray, go away,” Zane sang to himself and doodled as I explained the ‘anti-monster process’ to his mother.

Any scented spray would work. Body mist or freshener or even bottled water with some essential oils, vanilla extract, or lavender for scent. The scent will help Zane remember that the ‘Monster Spray’ is working, and can make associations to feeling safe and in control. I recommended keeping the spray bottle within reach, in case he woke at night and needed a ‘booster squeeze.’

As we returned to speech-sound practice, we spent part of the session making a label with the words “Monster Spray” on it, complete with a drawing of a dark-green/red/black blob (“that’s the monster, but you can’t see it because it is under”) and a figure in a cape holding a spray bottle like a sword (“that’s me, because I am super-Zane”).

The progress report the following week was that the monsters had such itchy noses the first time Zane used the newly minted spray on them, that they declared right away: “Oh, no, Monster Spray; Better go another way.” When a few monsters did not get the memo and tried their luck a few nights later, Zane spritzed them and they reportedly scuttled away to warn all others that: “Zane has Monster Spray, better go another way!”

monster Spray1

Pendred Syndrome–a Query

A query from a parent:

“My son was just diagnosed with Pendred syndrome. He already has hearing aids from when we discovered he has hearing loss when he was 8 months old, but he needs stronger ones now because his hearing got much worse. The doctor said that he may end up deaf and that we need to check his thyroid, too. My son is already getting PT with early intervention because he is not walking yet at 18 months but we’re going to put him in speech therapy, too. What is Pendred syndrome and is it true my husband and I gave it to him? Will the therapy help with his hearing? There’s so much technical stuff and I’m really worried.”

Dear Worried Mom,

Of course you are worried. It would be overwhelming for any parent to have their child diagnosed with a problem, let alone one that can continue to affect them and that may change with time. A good step is to seek more information–as you are doing now, and hopefully will continue to do.

I’m glad that your son is already getting amplification (i.e. hearing aids) and that they are going to be adjusted/changed to reflect the change in his hearing–this is very important, and is a big step of helping him develop the best way possible. Am glad that he’s getting PT, too, if his motor skills can use some help; and that you are going to begin Speech-Language-Therapy with him, to ensure he makes the most out of his hearing and that his speech and language can develop as well as possible.

I hope that your pediatrician and the clinicians you’ll be working with (in Early Intervention, the ENT, the Audiologist, etc) will refer you to more information about Pendred syndrome, hearing loss, and rehabilitation of hearing loss in young children. Do let them know you are concerned and discuss your worries with them–I am quite confident that they will have resources, support, and more specific answers for you.

In the meanwhile, here’s a summary of some of the main features and issues in Pendred syndrome. This is not in any way a comprehensive review of Pendred or what the course of it would be in your son’s case. Nor is it a specific recommendation as to what to do in your son’s case, but I hope this overview will nonetheless give you some information to start with.

Pendred syndrome is a genetic disorder that causes early hearing loss in children. The syndrome is named after Vaughan Pendred, the physician who first described people with the disorder. It is actually the most common syndromal form of deafness in children–it is estimated that Pendred syndrome accounts for 5-10% of hereditary deafness cases! It is an autosomal recessive disorder (which basically means that both parents need to be carriers of the recessive gene–but the parents often have no symptoms and often don’t know that they carry the gene), and is associated with abnormalities of the cochlea, sensory-neural hearing loss, and diffuse thyroid enlargement (goiter). It can also sometimes create problems with balance.

Children who are born with Pendred syndrome may begin to lose their hearing at birth or soon after birth (like in your son’s case), and often lose hearing by the time they are three years old. The hearing loss is sensory-neural in type. When hearing is present at birth, it will usually worsen over time, with some children with Pendred syndrome becoming totally deaf (but not all, some children do have residual hearing or a lesser hearing loss). The hearing loss in Pendred syndrome is usually bilateral (in both ears), although one ear may have more hearing loss than the other.

Childhood hearing loss has many causes. Researchers believe that 50 to 60 percent of cases are due to genetic causes, and 40 to 50 percent of cases are from environmental causes (e.g. substances that damage the inner ear, such as certain antibiotics or chemotherapy medications).

The first part of a diagnosis of Pendred syndrome often includes the discovery of a hearing loss via a child failing a screening test or the presence of a hearing loss being found. Not all hearing loss in childhood is related to Pendred Syndrome, so the clinicians evaluate many things before considering the diagnosis and it can indeed take a bit of time before things are known for sure. Things like the kind, timing, amount, and pattern of hearing loss; the anatomy of the inner ear structures; the child’s balance and thyroid, etc. All these can help the health care professional determine whether to seek genetic testing and imaging tests for the inner ear, and to conclude whether a child has Pendred syndrome or some other type of progressive deafness.

Pendred syndrome can make the thyroid gland grow larger (when a thyroid gland grows too large and can visually bulge on the lower neck, it is called a goiter). The thyroid is a small, butterfly-shaped gland in the front of the neck, just under the ‘Adam’s apple’ and just above the collarbones. It plays an important role in the way the body uses energy from food, as well as in normal growth and development. Some thyroid issues in childhood (e.g. Grave’s Disease) can cause growth problems. However, in Pendred syndrome children often develop properly even if their thyroid is affected and their levels of thyroid hormones are usually normal. Not everyone with Pendred syndrome gets a goiter, and it typically develops in adolescence or early adulthood. Not all goiters are problematic, but if a goiter becomes large, it can impact breathing and swallowing and then treatment may be necessary. So an endocrinologist (doctor trained with diseases and disorders that involve the hormonal/endocrine system) is probably a good person to get involved even if only for followup.

By the way, it is still not clear to scientists why some people with Pendred have more issues than others (e.g. some have goiter and others don’t).

Alongside hearing loss, Pendred syndrome also can affect the vestibular system, which is the system which controls balance. The good news is that most children and adults with Pendred learn to adapt to whatever vestibular dysfunction they have and can manage routine tasks and daily balance requirements well. That said, because of the vestibular issues, some babies with Pendred syndrome may start walking later than other babies.

To get a bit technical: It is believed that Pendred syndrome is caused by mutations (i.e. changes) in a gene called SLC26A4(formerly known as the PDS gene) on chromosome 7. To be born with Pendred syndrome, a child would need to inherit two mutated SLC26A4 genes—one from each parent. Because it is a recessive gene, the parents are only carriers of the mutation (i.e. they don’t have the syndrome themselves). In a family where both parents are carriers, a child has a chance of 1:4 to be born with Pendred, and a 1:2 chance to be born a carrier. It is often recommended that if people are concerned about the possibility of Pendred (e.g. in a child or future children) that they seek genetic testing and counseling.

The anatomy of the inner ear is often viewed through imaging techniques such as magnetic resonance imaging (MRI) or computed tomography (CT), which allows viewing the cochlea and the vestibular system. The cochlea is the spiral-shaped part of the inner ear that converts sound into electrical signals that are then sent along to the brain through the auditory nerve. A healthy cochlea has two-and-a-half turns, but some persons with Pendred syndrome may have a cochlea with only one-and-a-half turns. The vestibular system includes a part called vestibular aqueduct. It is a kind of a bony canal that runs from the vestibule (the part of the inner ear between the cochlea and the semicircular canals) and the inside of the skull. Inside the vestibular aqueduct there is a fluid-filled tube called the endolymphatic duct, which ends with a balloon-shaped sac. People with Pendred syndrome may have an enlarged vestibular aqueduct, and the endolympatic duct and sack may also be enlarged.

Below is an image of the inner ear in some people with Pendred syndrome, taken from: http://www.nidcd.nih.gov/health/hearing/pages/pendred.aspx


When it comes to helping children with Pendred: First of all, to minimize the risk of worsening hearing loss, children with Pendred syndrome may be advised to wear head protection when engaged in activities such as bicycle riding and skiing (to avoid trauma to their head). They may be advised to avoid contact sports or situations that can lead to extreme, rapid changes in pressure such as scuba diving or hyperbaric oxygen treatment.

Like with your son, the treatment of children with Pendred syndrome indeed often involves a team of specialists. These could include an audiologist, speech-language-pathologist, otolaryngologist, endocrinologist, geneticist, physical therapist, and primary care physician. Not all need to see the child all the time, and not necessarily throughout childhood, but it is always good to have those who are working with the child communicate with each other as need be so that they can coordinate goals and needs and work as a team. Don’t be shy about asking professionals to collaborate–they can’t and won’t do so without your consent.

Hearing amplification (hearing aids, and for those who need them: cochlear implants) can greatly minimize the impact of hearing loss on language and development. Children with Pendred syndrome should start early treatment with a speech-language-pathologist, to help gain communication skills and learn how to maximize their use of their hearing aids or cochlear implant. So I think it is probably an excellent thing for your son to begin with an SLP now, when any gaps or difficulties, if there are any, hadn’t had a chance to grow wide. In addition to hearing rehabilitation through working on discriminating, identifying, understanding, and processing sounds and language better, speech-language-pathologists can also help children with Pendred syndrome with speech, voice, and language development. The hearing itself may not be made better, but how the child used the hearing they have can be get a lot better with treatment. Treatment can help minimize the impacts of hearing loss on development, language, communication, and learning. Physical therapy can help with balance issues, and help aid with motor-goals (such as walking, and later running, climbing, and so on).

It seems that your son is already up to a good start, by having a parent who is interested in getting more informed about his condition and who is seeking to follow through on the best treatment plan for him. I wish you and your son all the best, and a lovely, joyful, satisfying healthy childhood.