Issue #33 – Exploring Ears

Posted on Posted in All Articles, Down Syndrome and Congenital Heart Defects, ENT

Exploring Ears

Dr. Alison M. Maresh, MD


Winston’s Story

I walk into exam room 5 and Winston is waiting there with his mother. Winston is 5 years old and has Down syndrome. I am an Otolaryngologist, also known as an Ear, Nose, and Throat (ENT) specialist. I have been seeing Winston since he was an infant. He is here today for a follow up visit to check his ear and hearing health. Like many patients with DS, Winston has had ongoing problems with his ears and has had several surgeries to place small tubes in his ear drums to drain fluid and help his hearing.

I chat with Winston and his mom. He tells me he is feeling good today. No, his ears don’t hurt, and yes, can hear well. His mom fills in the details. Since I last saw him 6 months ago, he has been healthy. He is receiving services through school including speech therapy. His speech is improving, and his speech therapist is happy with his progress. His last general pediatrician visit was a month ago and his pediatrician was unable to see if his ears were healthy because of ear wax blocking his ear canals.

Winston sits on the exam chair by himself. He is now a pro at these visits. Like many kids, when he was younger he hated having his ears examined and cleaned and his mother had to hold him while he cried and squirmed through the visit. Fortunately, he has outgrown his fear of ear checks and now visits are quite easy for him (and me!). I look through a microscope and use small instruments to clean ear wax, or cerumen, out of his narrow ear canals.  I can now see the ear drums – they look healthy, and there are small plastic white tubes, about the size of the metal tip of a pen, sitting in place within the ear drums that I had placed surgically one year ago.

I tell Winston and his mom that his ears look healthy today and send them to the audiology team that works with me in my office to test his hearing. When they return I give them the thumbs up. He cooperated well with the test and his hearing is normal today. His mother is thrilled; he has had a long journey with his ears. He will come back in 6 months for his next assessment.

Winston has many of the common issues related to ear health seen in DS patients. Almost all patients with DS have very narrow ear canals, sometimes only 2-3mm in diameter. This makes it very hard for physicians to evaluate the health of the ear drum and diagnose common ear problems.

There are many different parts to the ear and DS children can have abnormalities with most of them. The second most common problem after narrow ear canals is having middle ear effusions. This is why Winston, along with so many other DS children, needed ear tubes. The middle ear is the space that sits behind the ear drum, also called the tympanic membrane (TM). When fluid sits in the middle ear it causes some degree of hearing loss as it prevents sound vibrations from traveling normally to the inner ear where they are converted to nerve signals. The normal mechanism for preventing fluid is  having a functional eustachian tube (ET), which is a drainage pathway that connects the middle ear to the throat in the back of the nose. Kids are prone to ET dysfunction because their ETs are short, flat, and the muscles around the ET are not fully developed. DS patients are especially prone to ET dysfunction due to an abnormal shape of the ETs and lower muscle tone around the ETs.

Some kids with DS also have an underlying sensorineural hearing loss (SNHL) which is where the inner ear does not work perfectly in converting sound vibrations into a nerve signal. While research is promising to someday find a cure for this type of hearing loss, currently the only treatment is to provide patients with SNHL with hearing aids. Hearing aids are programmed to an individual’s specific hearing loss and when used appropriately will prevent any limitations in development related to hearing loss.

Exposure to the full array of sounds around us is critical for the correct development of nerve connections in the hearing center of the brain. These nerve connections allow young infants and children to learn the meaning of sounds around them. Very importantly this includes the development of communication skills – both understanding and expressing speech. Subsequently, higher levels of communication and language depend on a strong basic speech foundation. The ability for neural networks to develop these abilities, known as brain plasticity, is very strong in infants and young children and fades with time. Very compelling studies have shown that even mild hearing loss has consequences on development, and any delays in diagnosing this hearing loss has long term consequences. This is why newborn hearing screening is now universal and the goal is to identify and correct hearing loss by the time infants are 6 months old. This is also why it is critical to identify young patients that develop ear effusions and treat with ear tubes if it doesn’t resolve – it is standard to recommend ear tubes if there are effusions for 3 months or longer.

All patients with DS should have regular ear checkups with an ENT specialist. We are able to clean the ear canal to make sure sound can get through to the middle ear. Clean ear canals also make it easier for other physicians to help monitor ear health. Along with our audiology colleagues we monitor hearing and treat any abnormalities in ear and hearing health.

While children with Down syndrome often have speech delay due to developmental causes, this means that it is even more important to make sure their hearing is normal to maximize their potential. Infants and children usually start to cooperate with office-based hearing tests when they are 7 to 9 months old. Unfortunately, some infants and children are not able or willing to cooperate with these evaluations; if office-based tests are not successful there are alternate ways of checking hearing.

I first met Winston when he was 15 months old. He didn’t cooperate with his hearing tests in the office and had effusions in both of his ears. At 18 months old we decided to put in his first set of ear tubes. This procedure was done under anesthesia which means it was painless for him and ensured that he was safe for the delicate procedure. His ear canals were so narrow that the standard instruments and tubes I usually use could not fit down the ear canal. I used smaller tubes that were able to fit down his ear canals and placed them endoscopically, using a tiny camera that fit down his ear canal and allowed me to see his ear drum while I operated. After the tubes were placed in the ear drum I was able to suction out the fluid that was sitting in Winston’s middle ear. The open tube then stays in the ear drum and prevents recurrence of the fluid until it falls out. Because he had not cooperated with hearing tests in the office we also did an auditory brainstem response hearing test, or ABR, while he was asleep. This involves putting small stickers on his head to measure the brainwaves through the hearing pathway. Fortunately, after the fluid was removed, Winston’s hearing was normal. His recovery after this surgery was easy – he was cranky when he first woke up but a few hours later it was like nothing happened!

Ear tubes are temporary, usually lasting about 1 ½ years before the growth of the ear drum pushes them out. The most common complications from this surgery are tubes that scar in place in the ear drum and don’t fall out requiring them to be pulled out under anesthesia when kids are older or ear drums that don’t heal after the tube falls out leaving a hole in the ear drum which can also be fixed surgically.  These are uncommon and both easily managed by the ENT specialist.

After his first set of ear tubes Winston was monitored in my office every 6 months. His tubes lasted for 2 years before they fell out when he was 3 ½ years old. Unfortunately, his effusions returned a few months after the tubes came out. At that time he was finally cooperating with office hearing tests and we could see that the effusions were causing a mild hearing loss. He had another surgery when he was 4 to put new tubes back in. I also removed his adenoids at the same time.  Adenoids are tissues that sit in the throat behind the nose, right where the eustachian tubes drain. They are part of the immune system but there is no negative effect on the immune system if they are removed. Adenoids affect eustachian tube function in several ways – they can block the opening of the ET, they can harbor bacteria, and as an organ of immune function they can trigger chronic inflammation of the ET. Many studies have shown that removing adenoids improves ET health so this is now standard for kids that need more than one set of ear tubes. His parents were initially worried that the adenoidectomy would make recovery after surgery harder, but, like most kids after adenoidectomy, Winston was fine later that day.

There are other advantages of ear tubes in addition to improving the hearing loss caused by effusions. Chronic fluid in the ears isn’t healthy for the middle ear. Years of inflammation can cause scarring of the ear bones that transmit sound causing permanent hearing loss. Abnormal pressures associated with fluid can also pull the ear drum inside the middle ear which can be dangerous. The outer layer of the ear drum is similar to skin, always growing new cells, which normally shed along with cerumen out through the ear canal.  If it starts growing inside the middle ear it becomes a cholesteatoma – basically a mass of skin debris that causes chronic inflammation and erodes through the bones and important structures of the middle ear. These problems can be fixed surgically but these surgeries can be complicated and there is often permanent hearing loss, which can be severe depending on how advanced the problem is before it is caught. This is one of many reasons it is so critical for DS patients to be monitored by an ENT specialist to catch any developing issues early on.

As I reviewed with his mother, Winston’s story is not over. His second set of ear tubes will fall out as well, probably within the next year. Hopefully the natural function of his ears will have matured by that time and he won’t need another set. It is rare for children to have ongoing issues into older childhood or adulthood, though as a patient with Down syndrome this is a possibility for Winston. If we need to put in a third set of tubes I will talk to his parents about putting in permanent tubes that don’t fall out. This will prevent the need for recurrent ear tube surgeries which isn’t healthy for the ear drum and obviously stressful for kids and families. The permanent ear tubes are very easily removed if or when the ENT doctor feels like it is appropriate.

Winston’s story is common. With regular checkups we’ve kept his ears healthy and his hearing normal so he and his family can focus on his therapy for language development, knowing his potential is maximized. Not all kids with Down syndrome have ear problems but given the difficulties in assessing these patients, all children with DS should have an ENT specialist that is comfortable managing younger and sometimes complex patients. Even my DS patients that have healthy ears see me yearly throughout their vulnerable stages of hearing and speech development. I am hopeful that Winston’s ear journey will be over soon but even if it isn’t I’ll be there to support him and his family throughout.


Dr. Alison Maresh is an Assistant Professor of Otolaryngology – Head & Neck Surgery of Otolaryngology at Weill Cornell Medicine. She is board certified in Otolaryngology and has completed advanced fellowship training in Pediatric Otolaryngology. Dr. Maresh treats the broad spectrum of pediatric ear, nose, and throat disorders. Her clinical interests include the endoscopic diagnosis and management of airway disorders, evaluation and treatment of complex pediatric ear and hearing pathology, surgical and medical management of congenital disorders of the head and neck, and the multidisciplinary care of complex patients with aerodigestive disorders.