Otosclerosis book

NYOG proudly announces the publication of a new textbook by Dr. Sperling.

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QUESTION: Would a tympanogram always reveal if someone has otosclerosis? Or is a CT scan a more conclusive way to determine if someone has otosclerosis?

ANSWER: Although not mandatory, a CT scan done properly will reveal evidence of otosclerosis in the majority of cases.  A tympanogram is far less specific.

Thanks for your question!

Neil Sperling, MD

NYOG proudly announces the publication a new textbook co-edited by Dr. Neil Sperling.

Available from Plural Publishers in January 2014

 Otosclerosis book

Otosclerosis
by De Souza, Goycoolea & Sperling

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Question:

Recently semi-diagnosed with otosclerosis? History of swimmers ear rt ear and over lady 3 years recurrent ear infections and one time small staff infection at the beach. It all cleared up. I was fine until oct 29th of this year, took a mountain trip with sinus trouble, ear popped and now having clogged ear feeling rt ear only with buzzing and screeching sound now. For about 6 weeks, spot with ENT in Charlotte after several hearing evaluations at visit, he is pretty sure it is otosclerosis ?  Can this come on that sudden and he says in not ready for surgery yet… He will recheck ear in 4 weeks and then maybe consider a small hearing device ? No pain or balance problem. I sometimes hearing my bones or something in the back if my ear/head make a sound ..  Help !

Answer :

Thank you for your inquiry.  Its difficult to be sure whether you have otosclerosis or the consequences of prior ear infections.  Of course there is more than 1 condition that can present with ear stuffiness and hearing loss. It is possible for it start rather suddenly, or you become suddenly aware of something that has been slowly developing.

Otosclerosis is a problem that causes stiffening of the joints between the little hearing bones (ossicles) in the middle ear.  This serves to muffle sound as it goes from the ear drum, through the ossicles to the inner ear.  It usually develops when you’re in the 20s and can cause a significant hearing loss.  Otosclerosis is readily treated with surgery to replace these bones (stapedectomy) or with hearing aids.

Usually the diagnosis becomes clearer as time passes, and with careful examinations.  If it worsens, surgery or hearing aids are considered.  If you send us a copy of your hearing test, we may be able to give you a better idea.

I hope this helps clear things up.

Best of Luck.

Neil M Sperling, MD

New York Otolaryngology Group

36A East 36th Street

New York, NY 10016

 

Associate Professor

SUNY Downstate

Brooklyn, NY

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What Is Otosclerosis?

The term otosclerosis is derived from the Greek words for “hard” (scler-o) and “ear” (oto). It describes a condition of abnormal bone growth around the stapes bone, one of  the tiny bones of the middle ear.  This leads to a fixation of the stapes bone. The stapes bone must move freely for the ear to work properly and hear well.

Hearing is a complex process. In a normal ear, sound vibrations are funneled by the outer ear into the ear canal where they hit the tympanic membrane (ear drum). These vibrations cause movement of the ear drum, which  transfers the vibrations to the three small bones of the middle ear, the malleus (hammer), incus (anvil), and stapes (stirrup). When the stapes bone moves, it sets the inner ear fluids in motion, which, in turn, start the process to stimulate the tiny sensory hair cells in the inner ear, which connect with the auditory (hearing) nerve. The hearing nerve then carries sound information to the brain, resulting in hearing of sound. When any part of this process is compromised, hearing is impaired.

Who Gets Otosclerosis and Why?

It is estimated that ten percent of the adult Caucasian population is affected by otosclerosis. The condition is less common in people of Japanese and South American decent and is rare in African Americans. Overall, Caucasian, middle-aged women are most at risk.

The hallmark symptom of otosclerosis, slowly progressing hearing loss, can begin anytime between the ages of 15 and 45, but it usually starts in the early 20’s. The disease can develop in both women and men, but is particularly troublesome for pregnant women who, for unknown reasons,  can experience a rapid decrease in hearing ability.

Approximately 60 percent of otosclerosis cases have a genetic predisposition. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.

Symptoms of Otosclerosis

Gradual hearing loss is the most frequent symptom of otosclerosis. Often, individuals with otosclerosis will first notice that they cannot hear low-pitched sounds or whispers. Other symptoms of the disorder can include dizziness, balance problems, or a sensation of ringing, roaring, buzzing, or hissing in the ears or head known as tinnitus.

How Is Otosclerosis Diagnosed?

Because many of the symptoms typical of otosclerosis can be caused by other medical conditions, it is important to be examined by an otolaryngologist (ear, nose and throat doctor) to eliminate these other causes. After an examination, the otolaryngologist may order a hearing test. The typical finding on the hearing test is a conductive hearing loss in the low frequency tones.  This means that the loss of hearing is due to an inability of the sound vibrations to get transferred into the inner ear. Based on the results of this test and the exam findings, the diagnosis of otosclerosis can be made. The otolaryngologist will suggest treatment options.

Treatment for Otosclerosis

If the hearing loss is mild, the otolaryngologist may suggest continued observation or  a hearing aid to amplify the sound reaching the ear drum. Sodium fluoride has been found to slow the progression of the disease and  is sometimes prescribed. In some cases of otosclerosis, a surgical procedure called stapedectomy can restore or improve hearing.

What Is a Stapedectomy?

A stapedectomy is an outpatient surgical procedure done under local or general anesthesia. The surgeon performs the surgery through the ear canal with an operating microscope. It involves removing part or all of the immobilized stapes bone and replacing it with a prosthetic device. The prosthetic device allows the bones of the middle ear to resume movement, which stimulates fluid in the inner ear and improves or restores hearing.

Modern-day stapedectomy has been performed since 1956 with a success rate of approximately 90 percent. In rare cases (about one percent of surgeries), the procedure may worsen hearing.

Otosclerosis affects both ears in eight out of ten patients. For these patients, ears are operated on one at a time; the worst hearing ear first. The surgeon usually waits a minimum of six months before performing surgery on the second ear.

What Should I Expect after a Stapedectomy?

Most patients return home the evening after surgery and are told to lie quietly on the un-operated ear. Oral antibiotics may be prescribed by the otolaryngologist. Some patients experience dizziness the first few days after surgery. Taste sensation may also be altered for several weeks or months following surgery, but usually returns to normal.

Following surgery, patients may be asked to refrain from nose blowing, swimming, or other activities that may get water in the operated ear. Normal activities (including air travel) are usually resumed two to four weeks after surgery.

Notify your otolaryngologist immediately if any of the following occurs:

  • Sudden hearing loss
  • Intense pain
  • Prolonged or intense dizziness
  • Any new symptom related to the operated ear

Since packing is placed in the ear at the time of surgery, hearing improvement may not be noticed until it is removed about one to three weeks after surgery.  The ear drum will heal quickly, generally reaching the maximum level of improvement within two weeks.

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Otosclerosis is a condition of the ear caused when an abnormal growth surrounds the tiny vibrating bones of the middle ear, resulting in conductive hearing loss. It typically affects the Stapes bone which, when immobilized, cannot properly conduct sound energy toward the inner ear.

Approximately 60 percent of otosclerosis cases are hereditary. While it is seen across ethnic groups, it is most common in Caucasians between 20 and 45 years of age. Women are twice as likely to be affected as men and, once affected, often experience accelerated hearing loss during pregnancy.

Symptoms

Hearing loss is the primary symptom of otosclerosis. The hearing loss may be in one or both ears. It usually begins with mild loss, but often increases over time. Patients with otosclerosis may also suffer tinnitus, a ringing or rushing sound in the affected ear. Balance problems such as dizziness, vertigo, and unsteadiness may also occur, but are far less common.

Diagnosis

Patients with possible otosclerosis are first given a hearing test. However, as hearing loss can be caused by a number of disorders, a CT scan may sometimes be recommended.

Treatment

The good news is that conductive hearing loss due to otosclerosis is reversible, in most cases, with a surgical procedure known as a laser stapedotomy. Part of the Stapes is removed and replaced with a prosthesis that, once in place, allows the bones of the middle ear to move properly, thus restoring conductive function to the ear.

At NYOG, Dr. Sperling uses a specialized laser stapedotomy technique that includes vein grafting in order to eliminate post-operative vertigo, which is common after more traditional stapedectomy procedures. This technique is 90 percent to 95 percent effective at restoring the ability to hear, often immediately after surgery.

As an alternative to stapedectomy (surgery), traditional hearing aids can correct the hearing loss associated with otosclerosis. In some cases, a sodium fluoride treatment may also be prescribed to slow the progression of the disease.

Download: Surgery for Hearing Restoration, by Dr. Neil Sperling

Testimonials

Life Changing! Due to Otosclerosis I have worn hearing aids over 15 years. During that time I went from a mild loss to a severe loss. In June, 2015 Dr. Sperling performed a stapedoctomy on my left ear. The results are amazing having gotten back about 90% of my hearing. Dr, Sperling’s skill as a surgeon are matched by his compassion and reassuring manner. Although he’s done this procedure countless times, I never felt like just a number. The initial consultation, sitting with me a few minutes before the operation, and personally calling me the day after all proved his dedication and care for his patients. I highly recommend Dr, Sperling and his team and look forward to having the same experience with my right ear in September.

Dr. Sperling performed a Stapedoctomy to treat my Otosclerosis. He made me feel at ease since my first consultation with him. He explained my condition thoroughly and even showed me a power point presentation. On the day of the surgery he met with me right before the surgery to see if I had any questions and just sat by my side for a few minutes, which was very comforting. Once the Surgery was completed he took the time to go to a different floor to find my wife to let her know that everything went well. The day after the surgery he called me himself to see how I was feeling. On top of this great experience I was able to hear again!!!

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Question:

Hi,
I am 51 years old with hereditary otosclerosis, diagnosed right after the birth  of my 1st son when I was 36. In 2009, scared by the surgery (even though successful for my mom who had the procedure done by Dr Freich, you might know since you seem to give lectures in France) I opted for a hearing aid. It has now reached it’s full capacity and I need to buy a new one. But working as a School Counselor (for the French American Pre-School), listening to kids with multicultural backgrounds is a BIG challenge! And I think I still compensate with lip reading. Is it still possible to get a stapedotomy? Do you speak some French so I could consult you and understand better the pros and cons of such a procedure?

Thank you so much for your opinion/advice.

Answer:

Hi Floriane,
I would highly recommend a consultation with an experienced stapes surgeon to discuss the pros and cons.  In experienced hands, the surgery is highly successful with a recognized complication rate less than 1%.  In France, the Clinque Causse near Beziers is a world class facility with some of the most experienced surgeons in the world.

Best of luck.

Neil Sperling, MD
New York Otolaryngology Group
36A East 36th Street
New York, New York

Associate Professor, Dept of Otolaryngology
SUNY Downstate
Brooklyn, New York

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Question:

Many years ago I was diagnosed at Mass Eye and Ear with otosclerosis. I was operated on by Dr. Joseph Nadol. Over the past few years, my hearing has once again diminished and I wonder if its possible to do a revision? Also, my other ear was never been operated on. I am 68 years old, female, and a medical librarian, and my loss of hearing is obviously impinging on my work and life. I would prefer to not have to travel back to Boston…is this something you can help me with?

Answer:

Thank you for your inquiry regarding your hearing loss. It is often possible to regain excellent hearing with revision stapes surgery. It all depends on the nature of the hearing loss that you have. The first step would be to re-test your hearing. I would be happy to help you if you’d like. You can make an appointment online or by calling our office. Best of luck.

Neil M Sperling, MD

Associate Professor
SUNY Downstate
Brooklyn, NY

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Sensorineural Hearing Loss or Nerve Damage Hearing Loss

Most common causes include:

  • Presbycusis: age-related hearing loss
  • Noise induced hearing loss (more on this below)
  • Sudden Hearing Loss
  • Auto-Immune hearing loss
  • Medical conditions associated with hearing loss: stroke, diabetes, ototoxic medications

Conductive Hearing Loss

There are some health related causes of hearing loss, like:

  • a virus or bacteria
  • heart conditions or stroke
  • head injuries
  • tumor
  • certain medicines
  • diabetes
  • and hypertension

Then there are the factors of which we have little control, like:

Noise-induced Hearing Loss

Taking care of your hearing is important, especially since we now live in such a noisy world. Whether you live in a big city, like New York City, the suburbs or a rural area, technology can provide many opportunities for damaging your hearing. Here at NYOG we are often asked how you can prevent hearing loss.

There are many things you can do, like wearing earplugs when you know you are going to be in a situation where noise levels are extreme, or avoiding these situations altogether, as well as being responsible with the volume levels of your music devices and television.

Here are some examples of situations to be on the look out for where noise can be an issue to your hearing health:

Extreme volume at:

  • music concerts
  • sporting events
  • surround-sound stereo systems
  • car alarms
  • motorcycles, trucks, airplanes and other vehicles
  • construction noise (jackhammers, drills, saws, etc…)
  • portable music players (i.e. ipods)
  • anytime you are using headphones

Continued exposure to these and other excessive environmental noise is a major contributor to hearing loss.

Some professions that are often associated with noise-induced hearing loss are:

  • construction workers
  • farm workers
  • musicians, concert stagehands or roadies
  • airport workers
  • tree cutters and lumber workers
  • employees and soldiers in the armed forces

Click here to read about Hearing Preservation for Musicians. This protocol can be applied to any profession in which extreme noise is an issue.

If you are experiencing hearing loss in one or both ears, or if you have a constant ringing [also known as tinnitus (tin-NY-tus)] or any pain or ache, come in to see us. Whether environmental, health-related or genetic, our docs will get to the root of the problem and provide solutions. We are here to answer any and all of your questions because at NYOG…we are here to clear things up.

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Sometimes surgery is the best way to restore health and hearing, and you can be confident that at NYOG you are in the hands of the best surgeons in the field.

Our success rates with surgical interventions are among the highest in the country. Our doctors are consistently ranked as the finest physicians available by Castle Connolly, New York Magazine’s Best Doctors, Best Doctors in America (a rating by peer physicians), Super Doctors, and New York’s Top Doctors.

As always, we are here to answer any and all of your questions regarding your ear and hearing health and all options for your treatment. Below are some descriptions of the surgeries associated with the ear and hearing. We want our patients to be as informed as possible, because at NYOG our goal is to…clear things up.

Types of ear surgery include:

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NYOG’s Hearing & Balance Center provides specialized care for the evaluation, prevention and treatment of hearing loss and related conditions in the greater New York region. Dr. Sperling and his team have expertise is in treating and diagnosing Otitis MediaOtosclerosis, Conductive Hearing LossCholesteatoma, Meniere’s Disease, Sudden Hearing Loss and Autoimmune Inner Ear Disease.

OTITIS MEDIA (EAR INFECTIONS)

The most common childhood illness, ear infections are painful and can cause permanent hearing loss. Often, antibiotics to fight the infection will make the earache go away rapidly, but recurrent infections may need other treatments. Learn more about the treatment for Ear Infections (Otitis Media)

OTOSCLEROSIS

One cause of conductive hearing loss is when an abnormal growth surrounds the tiny vibrating bones of the middle ear. Hearing loss due to otosclerosis is reversible, in most cases, with a surgical procedure known as a laser stapedotomy.  Learn more about the treatment for Otosclerosis

CHOLESTEATOMA

Cholesteatoma is a benign tumor caused by the build-up of skin cells and protein from the ear canal behind the eardrum. If the cholesteatoma is small and hearing loss remains at acceptable levels, then microscopic cleaning may be performed. For a more advanced cholesteatoma, a mastoidectomy may be performed. Mastoidectomy is a surgical procedure designed to remove cholesteatoma and infections from within the mastoid bone. Learn more about the treatment for Cholesteatoma

EAR DRUM PERFORATION (RUPTURED EAR DRUM)

Perforations or ruptures of the ear drum are usually caused by an injury or repeated infections. By repairing the tympanic membrane and/or the ossicular bones, the conductive mechanism can be re-established and hearing restored. Learn more about the treatment for Ear Drum Perforation

OSSICULAR CHAIN DISCONTINUITY

One cause of conductive hearing loss is ossicular chain discontinuity or malformation, the result of which is the loss of transmission of sound from the ear drum (tympanic membrane) to the inner ear. In a surgical procedure known as ossicular chain reconstruction, the ossicluar chain is re-established.  Read more about Ossicular Chain Reconstruction.

AUTOIMMUNE INNER EAR DISEASE

Autoimmune inner ear disease is a rare disease and most doctors are unfamiliar with this disorder. Dr. Sperling has extensive experience treating this uncommon condition. A steroid called prednisone is most often prescribed to suppress the immune system and slow the attack on inner ear structures. Intratympanic steriod injections offer a potent addition to treating this condition. Learn more about the treatment of this condition

MENIERE’S DISEASE

While there is presently no known cure for Meniere’s Disease, which occurs when there are excess fluids and pressure in the inner ear, Dr. Sperling and his team at NYOG specialize in alleviating the symptoms of the disease, with treatment tailored to your specific case. Most cases can be greatly improved with treatment. Learn more about the treatment for Meniere’s Disease

TINNITUS

An otolaryngologist will work with you to find the specific cause of your tinnitus, your doctor may be able to offer a specific treatment to eliminate the noise. Some other treatments are: mindful meditation, hearing aids, cochlear implants or electrical stimulation, cognitive therapy, drug therapy, sound therapy and TMJ treatment. Learn more about the treatment for Tinnitus

Download: Surgery for Hearing Restoration, by Dr. Neil Sperling

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