Diseases and Conditions
At Mass. Eye and Ear, we provide evaluation and treatment services for the complete spectrum of problems and disorders affecting the ears for both children and adults. To schedule an appointment, call 617-573-3954 today.
Our team of specialists work closely to provide a comprehensive, multidisciplinary approach to managing the following disorders:
Auditory Brainstem Implants
Balance and Vestibular Disorders
Canal Dehiscence Syndromes
Chronic Ear Infections
Eustachian Tube Problems
Facial Nerve Disorders
Hearing Testing and Rehabilitation
Neurofibromatosis Type 2
Pediatric Hearing and Balance
Skull Base Tumors
An auditory brainstem implant (ABI) is a special prosthesis used to provide or restore some hearing ability to deaf patients who cannot benefit from cochlear implantation. Candidates include children who were born without hearing and who do not have the inner ear structures (cochlea) or cochlear nerve (hearing nerve) necessary to utilize a cochlear implant, and adults who have had tumors (most commonly those with a syndrome called Neurofibromatosis Type II), surgery, or trauma (such as temporal bone fractures) that has disrupted the inner ears and/or cochlear nerves.
The ABI is surgically placed directly onto the brainstem. An external device, which is usually worn over the ear, contains a microphone and technology to translate sounds into electrical signals. The externally worn device communicates with the internal implanted device to directly stimulate neurons in the brainstem to generate auditory perceptions, enabling the patient to hear sounds and sometimes words.
Dizziness and imbalance symptoms may be attributed to a wide variety of underlying causes. A few of the more commonly associated diseases and conditions are:
- Ménière’s disease: A degenerative disorder of the inner ear characterized by fluctuating and progressive sensorineural hearing loss and attacks of whirling vertigo that last from 20 minutes to 24 hours.
- Benign Paroxysmal Positional Vertigo (BPPV): Caused by tiny crystals that come loose from their normal location in the inner ear to trigger sudden, brief attacks of whirling vertigo triggered by position change, especially rolling over in bed or pitching the head forward or back.
- Vestibular neuritis (also called vestibular neurolabyrinthitis): An inflammation of the inner ear or vestibular nerve fibers that causes a rapid onset of disabling vertigo that recovers slowly over days to months.
- Migraine-associated dizziness: Up to 40 percent of those suffering from migraines will experience symptoms of imbalance and/or vertigo.
Vestibular symptoms may also be caused by traumatic injury, tumors, degenerative nerve diseases (such as Parkinson’s disease and multiple sclerosis), stroke, muscular and skeletal issues, and cardiovascular disorders.
Canal dehiscence syndromes refer to conditions in which there is an incomplete bony covering of one or more semicircular canals (portion of the inner ear responsible for detecting motion). The incomplete bony covering can enable stimulation of the balance canal with movements that pressurize the ear or with loud sounds. Patients often notice that maneuvers such as heavy lifting, bearing down, or loud sounds can trigger a feeling of spinning or motion (vertigo).
The most common type of dehiscence is superior canal dehiscence. This condition is likely due to a congenitally thin skull base that may become thinner with age until a dehiscence develops. Posterior canal dehiscence most often occurs in the setting of a vascular structure impinging on posterior canal. Other causes of canal dehiscences include cholesteatoma (skin cysts), chronic otitis media, tumors, or surgery.
A canal dehiscence may be found when patients develop symptoms or by chance on imaging. Typically a high-resolution temporal bone computed tomography scan (CT) is needed to diagnose a dehiscence. Other types of balance tests (VEMP testing) may be used to determine whether the dehiscence leads to functional changes.
Management options include observation or surgical repair. The approach and extent for surgical repair depends on the location of the dehiscence and ranges from transmastoid surgery (behind the ear) to craniotomy surgery (above the ear).
Chronic ear infections of the middle ear (chronic otitis media) are caused by Eustachian tube dysfunction (blockage of air to the middle ear) that leads to chronic negative pressure and fluid sequestration in the middle ear, changes in the eardrum, and sometimes development of a cholesteatoma (skin cyst). Symptoms of chronic otitis media include hearing loss, ear blockage, drainage from the ear, and/or ear pain. Patients may also have trouble equalizing pressure on an airplane.
On examination, abnormalities of the eardrum can be identified, such as pockets in the eardrum (called retraction pockets) or perforations (holes). Sometimes erosion or destruction of the ossicles (bones necessary for hearing) is seen.
Regular ear exams and hearing tests are typically done to monitor any progression and determine the best course of action. Treatment includes medical therapies for treating ear infections, tympanostomy tube placement, and surgery for hearing loss, ear drum or ossicular (hearing bone) abnormalities, or cholesteatoma development.
Cochlear implants are widely considered revolutionary devices for hearing rehabilitation. They act to bypass a non-functioning cochlea (the organ of hearing) with an electrode that provides electronic signals directly to the hearing nerve found in the inner ear. The internally implanted portion of the device is placed during a 1–2 hour outpatient surgery, and communicates wirelessly across the skin with an externally placed speech processor that has a microphone. Sound picked up by the microphone is converted into an electrical signal, and that information is sent to the brain.
Who is a candidate for cochlear implants?
Cochlear implants may be considered for adults and children with severe problems detecting sound and understanding speech in both ears, to the point where hearing aids are no longer beneficial.
An evaluation for cochlear implants typically includes a CT and/or MRI scan of the ear. Additional tests may be ordered for children who were born deaf. Children born deaf and without the hearing nerve are not be candidates for cochlear implants. In these specific situations, the patient may instead be a candidate for auditory brainstem implantation.
Cochlear implant candidates are evaluated by an ear surgeon, an audiologist, and a social worker throughout their course of treatment.
Ear atresia is a congenital condition in which the external ear canal fails to open normally during development. This can result in hearing loss and, in some cases, the formation of a skin cyst (cholesteatoma) in the canal requiring surgical removal. The external ear may also appear different and/or smaller (microtia). These changes may be present in one ear or, less commonly, in both ears.
We offer a range of options for the management of ear atresia and microtia. Your child may be a candidate for surgery to widen the ear canal. In some cases, a bone-anchored hearing aid may be appropriate to improve the hearing in the ear with atresia. If microtia is also present, specialists from our Facial Plastic and Reconstructive Surgery Division may surgically repair the external ear.
In both ears, the Eustachian tube communicates between the spaces behind the eardrums (middle ear) and the common space behind the nasal cavities (nasopharynx). The Eustachian tube allows for pressure equalization of the middle ear. Opening of the Eustachian tube through swallowing, yawning, or nose-blowing causes “popping” of the ears, which equalizes the pressure within the middle ear.
Inability to open the Eustachian tube results in negative pressure within the middle ear. Patients with Eustachian tube dysfunction often experience an uncomfortable sensation of ear blockage and/or fluid within the middle ear, which may cause hearing loss. Long-standing Eustachian tube dysfunction can result in chronic middle ear infections, sometimes leading to formation of a skin cyst within the ear (cholesteatoma) that requires surgical removal.
Although there is no established cure for Eustachian tube dysfunction, the symptoms arising from the condition may be managed by one of our surgeons. Surgical treatment options range from placement of a ventilation tube in the eardrum to procedures to reconstruct the eardrum and middle ear.
Patients with problems involving the facial nerve may experience a range of symptoms, from twitching of the face to complete paralysis. There are many causes of facial nerve weakness or paralysis, including infections both within and around the nerve, benign and malignant tumors, congenital conditions, and skull fractures from head trauma. In many cases, prompt evaluation and treatment can improve long-term outcomes.
At Mass. Eye and Ear, we work closely with our facial plastic and reconstructive surgeons and oculoplastics surgeons in the Facial Nerve Center to outline an optimal course of management for facial nerve disorders. This multidisciplinary approach allows for efficient and expert patient evaluation. Treatments may be medical or surgical and are aimed at maximizing patient quality of life.
Hearing loss is a common medical condition that is often under-diagnosed. There are two major types of hearing loss: conductive hearing loss and sensorineural hearing loss. Both categories can occur gradually or suddenly. In some cases, patients can have both types in the same ear.
If hearing loss occurs suddenly, it is a medical emergency. Patients with sudden hearing loss should seek urgent evaluation in clinic or in an emergency room.
Conductive Hearing Loss: Occurs when sound cannot reach the inner ear due to a problem of the outer or middle ear.
- Common causes include ear wax, outer ear infection (otitis externa or swimmers ear), a hole in the eardrum, negative pressure of the middle ear (Eustachian tube dysfunction), fluid in the middle ear (otitis media), a tumor or cyst in the middle ear (cholesteatoma), damage of the hearing bones (ossicles), and stiffening of the joints of the ossicles (such as otosclerosis).
- Less common causes include abnormalities of the inner ear, such as superior semicircular canal dehiscence (SSCD), and large vestibular aqueduct syndrome (LVAS).
Many causes of conductive hearing loss may be surgically addressed.
Sensorineural Hearing Loss (SNHL): Occurs following a problem of the inner ear, when the hair cells and nerves of the inner ear, or cochlea, are damaged or lost. Sensorineural hearing loss is generally a permanent condition unless the hearing loss fluctuates or was sudden in onset.
- Common causes include aging (one third of patients over 60 years old and one half of patients over 85 have hearing loss), noise exposure (at work or recreationally), conditions of the inner ear that cause dizziness, like Meniere's disease, and a severe head injury or skull fracture.
- Genetic hearing loss is another common cause—many hearing loss cases are hereditary and caused by gene mutations.
- Less common causes include autoimmune factors and benign tumors (acoustic neuromas).
Our audiologists provide hearing testing and rehabilitation services for patients of all ages. In our state-of-the-art clinic, we perform a number of diagnostic tests, from basic hearing tests and evaluations to more advanced testing such as Auditory Brainstem Response (ABR). Our services also include aural rehabilitation and assistive devices including hearing aids and cochlear implants. Learn more »
Neurofibromatosis type 2 (NF2) is a genetic condition that affects 1 in 25,000 individuals, according to the National Institute of Neurological Disorders and Stroke (NINDS).
The hallmark of this rare condition is the presence of vestibular schwannomas, benign tumors of the eighth cranial nerve, near both ears. The eighth cranial nerve has a cochlear and vestibular portion that transmit the neural signals for the perception of hearing and balance, respectively. Patients with NF2 often first see a doctor about their condition at a young age with hearing loss, tinnitus (ringing in the ear), and problems with balance. Individuals with this condition are at risk of developing other tumors of the nervous system, including gliomas, meningiomas, schwannomas of other nerves, ependymomas, juvenile lenticular opacity, and cortical cataracts.
Our ear surgeons collaborate with the Neurofibromatosis Clinic at Mass General Hospital to provide multidisciplinary care for patients with NF2.
Management options for NF2 may include observation, radiation, and/or surgery for vestibular schwannomas. For any associated hearing loss, we offer all hearing rehabilitation strategies including hearing aids, cochlear implants, and auditory brainstem implants. NF2 patients may also choose to enroll in ongoing clinical trials available at Mass. Eye and Ear for their condition.
Otosclerosis is an abnormal, microscopic growth of bone in the walls of the inner ear, which causes the stapes bone (the smallest bone in the body—commonly called the "stirrup") to become frozen in place or "fixed." Normally the stapes vibrates freely to allow the transmission of sound into the inner ear. When it becomes fixed to the surrounding bone, hearing is impaired, as this prevents sound waves from reaching the inner ear.
Although many patients can wear a hearing aid successfully, natural hearing is often preferred. Surgery has been found to be the most effective method of improving hearing loss caused by otosclerosis.
Surgery for otosclerosis, called “stapedectomy,” involves replacement of the diseased bone with an artificial prosthesis. The surgery is typically performed in the outpatient setting and usually takes place entirely through the ear canal, meaning no outer incisions are made. The surgery has a very high rate of fully restoring natural conductive hearing in the affected ear.
Our team sees patients of all ages, including children. We care for children with a wide range of conditions, including minor and complex disorders of the ears, mild to profound hearing loss, and dizziness. We use leading-edge techniques and technology, combined with the kind of understanding and compassion children and their parents need while facing these medical issues. We also give careful consideration to a range of medical and surgical interventions to best help your child.
We treat all tumors of the cranial base including vestibular schwannomas (vestibular schwannomas), glomus tumors (paragangliomas), meningiomas, primary cholesteatomas (epidermoids), and cancers of the temporal bone and cranial base. We also treat traumatic injuries of the cranial base and cerebrospinal fluid leaks.
Since the treatment of tumors of the cranial base is complex and requires individualized care for each patient, our team includes physicians from both Mass. Eye and Ear and Massachusetts General Hospital. We work with each patient to develop a tailored treatment plan, which may include care from multiple specialties such as neurotology, neurosurgery, head and neck surgery, rhinology/endoscopic sinus surgery, medical and radiation oncology, and facial plastic surgery and rehabilitation specialists.
We also offer a range of services including consultation, evaluation and diagnosis, medical and surgical treatment, radiation, and rehabilitation services.
Exostoses are benign bony outgrowths of the external auditory canal. They are often called “surfer’s ear” as they are frequently seen in individuals who have been exposed to cold water over prolonged periods of time.
Most exostoses do not require treatment and can simply be observed. However, in some cases, they can significantly narrow the ear canal and lead to trapping of water and recurrent ear canal infections (otitis externa or “swimmer’s ear”). On rare occasion, they can cause hearing loss. Wearing earplugs can help to prevent infections for this condition. For individuals who still have infections or associated hearing loss, surgery can help to prevent and correct these problems.
Surgery for exostoses is called a canalplasty. It is performed under general anesthesia on an outpatient basis. The procedure can be performed using an operating microscope or endoscopes. The surgeon will remove the bony outgrowths from the ear canal with fine instruments and/or a microdrill. An examination by an ear specialist and a hearing test are necessary to determine if you are a candidate.
Tinnitus, or “ringing in the ears,” is a symptom afflicting more than 50 million Americans. It refers to the perception of sound when no external sound is present. Tinnitus can be high- or low-pitched and can manifest many different perceptions of sound including ringing, buzzing, hissing, whistling, swooshing, and clicking. It may be a temporary or chronic condition. The sudden and persistent onset of tinnitus in one ear may be a sign of sudden hearing loss and should be assessed with a hearing test within 48 hours.
While no medical intervention can cure tinnitus at this time, tinnitus may be associated with a treatable condition such as hearing loss or other problems in the heart and blood vessels, jaws, and neck. Treating the underlying cause may diminish the perception of tinnitus. It is therefore important to see your doctor or an ear, nose, and throat (ENT) specialist to determine the cause of the ringing. Our specialists work with audiologists to determine if there is hearing loss associated with the tinnitus.
A vestibular schwannoma (formerly known as an acoustic neuroma) is a rare, non-cancerous tumor in the inner ear that presses on the nerves of the inner ear and can cause hearing loss and balance problems. The most common symptoms are hearing loss, balance dysfunction, and tinnitus (ringing in the ear).
These tumors are benign, but can grow to a large, life-threatening size. In general, these tumors are very slow growing (1 to 2 millimeters per year) and some tumors do not grow at all or remain stable in size for years.
These tumors present in two different ways:
- Sporadic vestibular schwannoma: The most common type of vestibular schwannoma, typically presenting in adults, which affects only one ear.
- Bilateral vestibular schwannomas: The genetic condition neurofibromatosis type 2 (NF2) is associated with the presence of vestibular schwannomas in both ears, along with other brain and spinal cord tumors, including meningiomas and gliomas, and juvenile cataracts.
Vestibular schwannomas are diagnosed by assessing a patient’s history (symptom course), physical examination, audiograms (hearing tests), and obtaining an MRI. Treatment options include observation with repeated MRI imaging, surgery, or radiation therapy, depending on the size and severity of the tumor. As members of a high-volume vestibular schwannoma center, Mass Eye and Ear surgeons will work with you to tailor a treatment plan that meets your needs.