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Allow your Audiologist to professionally evaluate the type and severity of your hearing loss. The acoustic neuroma, also known as vestibular schwannoma,or an acoustic neuroma, is a nonmalignant tumor of the 8th cranial nerve (see Figure 1). In spite of the usual origin of acoustics in the vestibular nerve (Roosli et al 2012), vertigo (spinning) is not common, occurring in only about 20% of persons with acoustic neuroma. Facial sensory disturbances occur only in large tumors (about 50% of those greater than 2 cm in size). Acoustic neuroma occurs in two forms: a sporadic form and a form associated with an inherited syndrome. Figure 2: MRI scan of the brain showing an acoustic neuroma (the white spot on the left side of the picture).
Electronystagmography (ENG testing) is frequently abnormal, and about 50% of all tumors are associated with unilateral loss of calorics. Although it is relatively costly compared to audiometry or ABR, the optimal test for excluding an acoustic neuroma is a gadolinium enhanced T1 MRI (Figure 3). Acoustic neuromas range in size up to 4 cm.A The smallest, the intracanalicular acoustic, is measured in millimeters. If surgery is eventually required, surgical complications in this situation, such as severe facial nerve weakness, are nearly 100%. In January 2008, a visit to the National Library of Medicine’s search engine, PubMed, revealed more than 5409 research articles referring to acoustic neuroma published since 1949 with 150 published in the last year.
The American Hearing Research Foundation is a non-profit foundation that funds research into hearing loss and balance disorders related to the inner ear and is also committed to educating the public about these health issues. A hearing disorder presenting with grossly abnormal or absent neural responses, as measured by evoked potentials, in the presence of normal outer hair cell function evidenced by present otoacoustic emissions or cochlear microphonics. An in-vitro comparison of the disintegration of human ear wax by five cerumenolytics commonly used in general practice. Methotrexate in the management of immune mediated cochleovestibular disorders: clinical experience with 53 patients. This presentation will focus on cochlear implant candidacy criteria and how it has changed over the years to current standards. In this webinar, several unusual causes of conductive hearing loss will be examined including enlarged vestibular aqueduct, superior semicircular canal dehiscence, malleus fixation and cerebrospinal fluid leak. Most commonly, it arises from the covering cells (Schwann cells) of the inferior vestibular nerve (Roosli et al 2012).
NF2 is rare; there are only several thousand affected individuals in the entire United States, corresponding to about 1 in 40,000 individuals.
On MRI, acoustic neuromas are frequently uniformly enhanced, dense, and expand the internal auditory meatus. Acoustic neuroma caused by neurofibromatosis type II (NF 2) should be suspected in young patients and those with a family history of neural tumors.
Gamma knife stereotactic radiosurgery has become more prevalent recently as it has been demonstrated to be safe and effective in the control of acoustic neuromas (Likhterov, 2007). This occurs because the facial nerve often becomes “fused” to the tumor after the gamma knife procedure. This might occur if an acoustic tumor is present in the only hearing ear, or after surgery to remove bilateral acoustic neuromas. In spite of this concentration of effort by the medical community, acoustic neuroma remains a disorder that cannot be prevented and is often diagnosed after it is too late to save hearing. Ruckenstein, MJ Bigelow DC, The efficacy of corticosteriods in restoring hearing in patients undergoing conservative management of acoustic neuromas. Comparison between intraoperative observations and electromyographic monitoting data for facial nerve outcome after vestibular schwannoma surgery. Causes of unilateral sensorineural hearing loss screened by high-resolution fast spin echo magnetic resonance imaging: review of 1070 consecutive cases.
Outcome analysis of acoustic neuroma management: a comparison of microsurgery and stereotactic radiosurgery. Pain subsequent to resection of acoustic neuromas via suboccipital and translabyrinthine approaches. Retrospective study of postcraniotomy headaches in suboccipital approach: diagnosis and management. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference.
Jill Messina has experience with audiological and vestibular diagnostic assessment, electrophysiological testing, hearing aids, aural rehabilitation, and supervision of audiology Doctoral students. The presentation will include brief videos of the mechanics of ossicular reconstruction and surgical reconstruction of the ossicles including stapedectomy. The differentiating features of these unusual causes of conductive hearing loss will be discussed relative to more common causes of conductive hearing loss. Topics to be discussed include the symptoms and signs, audiologic profile and the latest treatments for these two conditions. Acoustic neuromas comprise about 6% of all intracranial tumors, about 30% of brainstem tumors, and about 85% of tumors in the region of the cerebellopontine angle. A high-frequency sensorineural pattern is the most common type, occurring in approximately two-thirds of patients. Unsteadiness is much more prevalent than vertigo, andA approximately 70% of patients with large tumors have this symptom.
A fast spin-echo T2 variant of MRI is very sensitive to acoustics, and in some clinical settings, can be done fairly inexpensively.
Tumors are staged by a combination of their location and size: An intracanalicular tumor is small and in the internal auditory canal (IAC). There are several other tumors that can occur in the same region of the brain [the cerebellopontine angle (CPA)] as acoustic neuromas.
Gamma knife does not generally make tumors go away — Figure 3 is actually that of a patient who had gamma knife surgery several years prior. Belal (2001) reported that cochlear implantation is possible only if there is an intact cochlear nerve (as shown by a positive response to promontory stimulation), and if the implantation is done at the time of acoustic tumor removal, before the cochlea ossifies (turns to bone). At the American Hearing Research Foundation (AHRF), we have funded basic research on acoustic neuroma in the past, and are very interested in funding additional research on acoustic neuroma in the future.
A comparison of direct eighth nerve monitoring and auditory brainstem response in hearing preservation surgery for vestibular schwannoma.
Intraoperative facial motor evoked potentials monitoring with transcranial electrical stimulation for preservation of facial nerve function in patients with large acoustic neuroma. Irradiation of cochlear structures during vestibular schwannoma radiosurgery and associated hearing outcome. Facial nerve monitoring paramaters as a predictor of postoperative facial nerve outcomes after vestibular schwannoma resection.
Medical history, cigarette smoking and risk of acoustic neuroma: an international case-control study.
Hearing preservation following fractionated stereotactic radiotherapy for vestibular schwannomas: prognostic implications of cochlear dose.
This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. He is in private practice at the Ear Institute of Illinois (formerly called the Ear Institute of Chicago) in Hinsdale, Illinois. In the remaining third, the next most common observation is hearing loss at low frequency (which would be more typical of Meniere’s disease). Facial twitching, also known as facial synkinesis or hemifacial spasm, occurs in about 10% of patients. Some small studies have found an association of acoustic neuromas with cellular phone use or prolonged exposure to loud noises, but other studies do not find this link (Christensen et al 2004, Edwards et al 2006, Edwards et al 2007, Hardell et al 2003, Lonn et al 2004, Myung et al 2009, Schlehofer et al 2007, Schoemaker et al 2007).

It has been estimated that 5% of persons with sensorineural hearing loss have acoustics (Daniels et al 2000), but this estimate is suspect as it would imply a much higher prevalence of acoustic neuromas than are commonly accepted. If an MRI cannot be done, an air-CT scan should be obtained in high-risk individuals, particularly if the ABR is suggestive of an acoustic neuroma.
Instead, gamma knife radiation shrinks the tumor and prevents future growth in most patients. The risk of hearing loss correlates with the radiation dose to the cochlea (Thomas, 2007; Massager, 2007). Those who opt not to have surgery will likely need to have periodic imaging studies to determine if it is still safe to leave the tumor without treatment.
We are particularly interested in projects that might lead to early detection (prior to onset of hearing loss).
Effects of vestibulo-ocular reflex exercises on vestibular compensation after vestibular schwannoma surgery.
Environmental risk factors for sporadic acoustic neuroma (Interphone Study Group, Germany). She is working in conjunction with the physicians of the Ear Institute of Chicago, LLC on a research study examining the Vibrant Soundbridge as a method for treating conductive and mixed hearing losses. Only about 10 tumors are newly diagnosed each year per million persons in the United States, corresponding to between 2,000 and 3,000 new cases each year. There is not hard evidence supporting a link between environmental factors and acoustic neuromas.
Less conservative incidence estimates for acoustic neuroma in asymmetrical hearing loss are reported at 2.5% (Suzuki et al 2010). Delayed facial weakness, and facial numbness also occur in roughly one-third of patients after gamma knife. Management options for cerebrospinal fluid leak after vestibular schwannoma surgery and introduction of an innovative treatment. Conservative management, gamma-knife radiosurgery, and microsurgery for acoustic neurinomas: a systematic review of outcome and risk of three therapeutic options.
The ear is divided anatomically into three sections (external, middle, and inner), and pathology contributing to hearing loss may strike one or more sections. View PDFSigns and SymptomsUnilateral sensorineural hearing loss is most common presenting symptom of a patient with an acoustic neuroma. In patients with hearing asymmetry, it is believed that only about 1 in 1,000 has acoustic neuroma (source: National Institutes of Health). When abnormal with a progressively worsening pattern, audiometry usually leads to further testing such as ABR (auditory brainstem response) and gadolinium enhanced MRI (magnetic resonance imaging), which establishes the diagnosis. Some tumors cause hydrocephalus by obstructing cerebrospinal fluid (CSF) drainage pathways in the 4th ventricle.
The recurrence rate of the tumor is about 3% after surgery, and 14% after gamma knife, but of course, this figure will vary with the surgeon and the gamma knife protocol. Hydrocephalus has been reported to occur in between 3 and 12.8% (Noren et al, Pollock et al). Train time as quantitative electromyographic paramater for facial nerve function in patients undergoing surgery for vestibular schwannoma. Preservation of facial nerve function after postoperative vasoactive treatment in vestibular schwannoma surgery.
Acoustic neuromas are sometimes identified in asymptomatic patients on radiological exams for other reasons, and may be identified in up to 0.02% incidentally (Lin et al, 2005).
However, because acoustic neuroma is a rare condition, sudden hearing loss attributable to an acoustic tumor occurs in only 1 to 5% of patients with sudden hearing loss as there are many more common causes (Nosrati-Zarenoe et al 2010, Suzuki et al 2010). Dysequilibrium is reported 8 to 31% of the time, a figure analogous to surgical management. Leading causes of conductive hearing loss include cerumen impaction, otitis media, and otosclerosis. A new technique, called summated ABR, which is essentially several ABRs compared over time, may provide better sensitivity. Leading causes of sensorineural hearing loss include inherited disorders, noise exposure, and presbycusis.
Tinnitus is very common in acoustic neuroma, and is usually unilateral and confined to the affected ear.
Radiation to the head increases the risk of future tumors of the brain and skull, but this risk is thought to be small. An understanding of the indications for medical management, surgical treatment, and amplification can help the family physician provide more effective care for these patients.
Radiation damage can also occur in the surrounding tissues, including nerves that control the face and tongue.
A five year follow-up study of 317 patients who underwent gamma knife radiosurgery found a five- and ten-year progression-free survival rates of 93% and 92% with minimal complications. The differential diagnosis of hearing loss can be simplified by considering the three major categories of loss. Conductive hearing loss occurs when sound conduction is impeded through the external ear, the middle ear, or both. Sensorineural hearing loss occurs when there is a problem within the cochlea or the neural pathway to the auditory cortex. Mixed hearing loss is concomitant conductive and sensorineural loss.EvaluationA thorough history and a careful physical examination are essential to the diagnosis and treatment of hearing loss. An otoscope should be used to examine the external auditory canal for cerumen, foreign bodies, and abnormalities of the canal skin. The mobility, color, and surface anatomy of the tympanic membrane should be determined (Figure 1). The sound remains midline in patients with normal hearing.The Rinne test compares air conduction with bone conduction.
When the patient no longer can hear the sound, the tuning fork is placed adjacent to the ear canal (air conduction). In the presence of normal hearing or sensorineural hearing loss, air conduction is better than bone conduction. Audiograms objectively measure hearing levels and compare them with standards adopted by the American National Standards Institute in 1969.1 Normal hearing levels are 20 dB or better across all frequencies. The audiogram measures air conduction and bone conduction and presents them graphically across the hearing frequencies.
Audiographically demonstrated conductive hearing loss results in the air line falling below the bone line, creating an air-bone gap.Speech testing should be performed using standard word lists. The speech reception threshold is the sound level at which 50 percent of presented words are understood.
The speech recognition score is the percentage of words understood at 40 dB above the speech reception threshold.Conductive Hearing LossEXTERNAL EARComplete occlusion of the ear canal by cerumen is a frequent cause of conductive hearing loss. The sensitivity of auditory brainstem response testing for the diagnosis of acoustic neuromas. Warm water (body temperature) irrigation is a safe method of removing cerumen in patients who have no history of otitis media, perforation of the tympanic membrane, or otologic surgery. The distance to the tympanic membrane must be kept in mind, because otoscopes do not allow for depth perception.
Aqueous-based preparations, including docusate sodium, sodium bicarbonate, and hydrogen peroxide, are effective cerumenolytics.2,3Foreign bodies in the external auditory canal also can cause unilateral conductive hearing loss. If the object is not impacted or hygrostatic, warm water irrigation probably should be attempted first. If this approach is not effective, the foreign body can be removed with an instrument if the patient is cooperative. If the patient is uncooperative, removal in an operating room may be necessary.Otitis externa is an infection of the skin of the external auditory canal.

Patients with otitis externa experience pain on manipulation of the pinna or tragus, and their ear canal is edematous and filled with infectious debris. The most common pathogens in otitis externa are Pseudomonas aeruginosa and Staphylococcus aureus.4 Treatment involves debridement of the canal, followed by the application of ototopical drops.
In patients with severe otitis externa, a wick is placed in the ear for two to three days to ensure delivery of the medication.
The conductive hearing loss resolves after the inflammation subsides.Exostoses and osteomas are benign bony growths of the external auditory canal that interfere with normal cerumen migration, leading to occlusion and conductive hearing loss.
Osteomas are single and unilateral, and are found at the bony-cartilaginous junction (Figure 2). Sebaceous cysts, fibromas, papillomas, adenomas, sarcomas, carcinomas, and melanomas also have been reported. If a malignancy is suspected, prompt biopsy is indicated.MIDDLE EARMiddle ear pathology may lead to conductive hearing loss. Perforations of the tympanic membrane cause hearing loss by reducing the surface area available for sound transmission to the ossicular chain (Figure 3). In patients who have had chronic otitis media with tympanic membrane perforation, otoscopic examination and debridement are essential.
Ototopical antibiotics (ofloxacin [Floxin]) are necessary, and oral antibiotics may be helpful. Small perforations (less than 2 mm) often heal spontaneously.5 In the acute setting, blood may obstruct the ear canal and prevent visualization of the membrane. If the perforation or hearing loss persists beyond two months, the patient should be referred for consideration of surgical correction. The diagnosis of otitis media can be confirmed by tympanometry and audiometry, and resolution of the effusion restores hearing. Congenital cholesteatoma presents as a pearly white mass located behind an intact tympanic membrane in a patient with unilateral conductive hearing loss. Acquired cholesteatoma results from a retracted or perforated tympanic membrane with an ingrowth of epithelium. Conductive hearing loss caused by ossicular erosion is present in 90 percent of patients with cholesteatomas.8 Longstanding cholesteatomas expand to involve the mastoid, inner ear, and facial nerve.
Suspicion of cholesteatoma warrants surgical consultation.Myringosclerosis of the tympanic membrane develops in response to infection or inflammation (Figure 3). Irregular white patches consisting of calcium are visible on the membrane.9 Isolated myringosclerosis of the tympanic membrane rarely causes significant conductive hearing loss. However, extensive myringosclerosis, referred to as tympanosclerosis, involves the tympanic membrane, ossicular chain, and middle ear mucosa, and causes significant conductive hearing loss by stiffening the entire system.Otosclerosis is characterized by abnormal bone deposition at the footplate (base of stapes). This bone deposition leads to fixation of the stapes at the oval window, preventing normal vibration.
Otosclerosis typically presents as progressive bilateral conductive hearing loss in middle-aged white women. It is the leading cause of conductive hearing loss in adults who do not have a middle ear effusion or a history of otitis media.10 There is usually a positive family history. These neuroendocrine tumors arise from the adventitia of the jugular bulb or the neural plexus within the middle ear space. Characteristically, patients presenting with glomus tumors are women 40 to 50 years of age who report pulsatile tinnitus and hearing loss. On examination, a pulsating reddish-blue mass may be seen behind an intact tympanic membrane. However, diagnosis of these tumors is difficult, and computed tomography of the temporal bones is required. Although most patients with this type of hearing loss are adults, children also can be affected. Hereditary and non-hereditary congenital hearing loss are the two major pediatric classifications. The majority of hereditary losses are autosomal recessive and are frequently associated with other systemic findings. More than 100 congenital syndromes are associated with sensorineural hearing loss.The consequences of delayed detection can be significant. Neonates considered at high risk for congenital hearing loss (Table 3)11 traditionally have been screened, and 30 states now require universal newborn auditory screening.12 The American Academy of Pediatrics and several other organizations endorse universal auditory screening.
Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs.
The etiology is a combination of inherited and environmental factors, including lifetime noise exposure and tobacco use. High frequencies are affected first, typically at 4,000 Hz, followed by middle and lower frequencies. The use of foam-insert earplugs decreases noise exposure by 30 dB.A less common cause of hearing loss is ototoxin exposure, typically from diuretics, salicylates, aminoglycosides, and many chemotheraupetic agents. These medications must be administered carefully in patients who are elderly, have poor renal function, require a prolonged course of medication, or require simultaneous administration of multiple ototoxic agents.
Patients with ototoxin exposure may experience hearing loss or dizziness.Autoimmune hearing loss has been diagnosed with increasing frequency since the 1980s.
Patients present with rapidly progressive bilateral sensorineural hearing loss and poor speech discrimination scores, and they also may have vertigo or disequilibrium.
Hearing loss progresses over three to four months, and an associated autoimmune disorder may be present. Symptoms usually improve with the administration of oral prednisone, and response to this steroid is currently the best way to make the diagnosis.
Low-dose methotrexate therapy is becoming an accepted alternative to long-term prednisone therapy.14UNILATERAL HEARING LOSSTemporal bone fractures can cause unilateral sensorineural and conductive hearing loss. When the fracture line involves the bony labyrinth (cochlea or vestibule), sensorineural hearing loss occurs. Temporal bone injuries are associated with facial nerve paralysis, cerebrospinal fluid leakage, and other intracranial injuries.
Early consultation is essential, and prompt surgical intervention may be required.Trauma may cause rupture of the round or oval window membranes, with perilymph leaking into the middle ear (fistula). Perilymph fistulas also may occur after straining, lifting, coughing, or sneezing, and are managed with three to six weeks of bed rest, followed by surgical repair if symptoms do not improve.15Meniere's disease is another cause of sensorineural hearing loss.
Patients report unilateral fluctuating hearing loss with aural fullness, tinnitus, and episodic vertigo. Initially, the hearing loss is in the low frequencies, but higher frequencies are affected as the disease progresses. The etiology of Meniere's disease remains unclear, but endolymphatic hydrops (increased fluid pressure within the inner ear) has been identified. The work-up consists of serial audiometry to document a fluctuating loss, vestibular testing to verify the diseased ear, and radiographic imaging to rule out an acoustic tumor. Hearing aids are often ineffective because patients suffer from poor speech discrimination, as well as diminished tolerance to amplified sound. A history of upper respiratory infection within a month of the hearing loss is often described in patients who have a viral etiology. The work-up includes audiometry, followed by radiographic imaging to rule out an acoustic tumor.
One study found that patients with minimal hearing loss, no vestibular symptoms, and early treatment have better outcomes.18Patients with acoustic neuromas present with unilateral sensorineural hearing loss approximately 10 to 22 percent of the time19 (Figure 6). Patients with asymmetric sensorineural hearing loss require evaluation for a retrocochlear tumor.

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