Sensorineural hearing loss and tinnitus are gradually receiving more attention in medicine due to advances in diagnosis and treatment. Acoustic neuromas can now be detected when they are small, and early microsurgical removal results in the lowest overall morbidity. We examine the historical development of acoustic neuroma management, discuss current diagnosis and treatment, and present illustrative cases from our recent experience. Complaints of tinnitus and hearing loss, especially when unilateral, require appropriate medical evaluation

Monsell, Edwin M.

Rock, Jack P.

English

Henry Ford Health System Scholarly Commons

Henry Ford Hospital Medical Journal Volume 38 Number 1 Article 3 3-1990 Sensorineural Hearing Loss and the Diagnosis of Acoustic Neuroma Edwin M. Monsell Jack P. Rock Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Monsell, Edwin M. and Rock, Jack P. (1990) "Sensorineural Hearing Loss and the Diagnosis of Acoustic Neuroma," Henry Ford Hospital Medical Journal : Vol. 38 : No. 1 , 9-12. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol38/iss1/3 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Sensorineural Hearing Loss and the Diagnosis of Acoustic Neuroma Edwin M. Monsell, MD, PhD,* and Jack P. Rock, MD' Sensorineural hearing loss and tinnitus are gradually receiving more attention in medicine due to advances in diagnosis and treatment. Acoustic neuromas can now be detected when they are small, and early microsurgical removal results in the lowest overall morbidity. We examine the historical development of acoustic neuroma management, discuss current diagnosis and treaiment, and present illustrative cases from our receni experience. Complaints of tinnitus and hearing loss, especially when unilateral, require appropriate medical evaluation. (Henry Ford Hosp Med J 1990:38:9-12) I n the past hearing loss and tinnitus were often regarded as mi-nor nuisances not merifing medical attention. This neglectful attitude originated from the once common view that hearing toss was a hopeless or comical condition and was indicafive of soci-ety's prejudice against the aged and the hearing impaired. More-over, littie could be done for hearing-impaired individuals unfil the introduction of Lempert's one-stage fenestration procedure just prior to World War It and the development and refinement of stapes surgery, tympanoplasty, and hearing aids in the first two decades after the war. Today hearing toss, tike the loss of any sensory function, is more widely recognized as a medical symptom which requires evaluation and which does not in itself consfitute a diagnosis. Historical Perspective on Tumor Treatment At the tum ofthe century, intracranial surgery uniformly re-sulted in fatality. Patients who did not die from hemorrhage suc-cumbed to .sepsis. Despite this grim prognosis, surgical treat-ment of acoustic neuromas was sometimes attempted when pa-tients became bedridden by severe cerebellar ataxia, lapsing in to and out of obtundation from brain stem compression and in-tracranial hypertension. If a patient presented with hearing loss, headache, and papilledema, the diagnosis of intracranial tumor was considered to be established. If the patient were atso ataxic, the tumor was presumed to be in the posterior cranial fossa (1). Boston neurosurgeon Harvey Gushing made significant eariy contributions to the diagnosis and treatment of acoustic neuro-mas, Cushing's first successful treatment consisted of removing the suboccipital bone bilaterally, allowing for expansion of the intracranial contents. This procedure allowed most pafients to become ambulatory and protonged their life by a few months. Later, Gushing succeeded with the subtotal removal of tumors. Operative mortality was reduced to 25%, a great achievement at that time, Cushing's careful accumularion of patient histories and physical findings led to the first detailed account of the clin-ical course and natural history of acousfic tumors. He described several surgical approaches to the cerebellopontine angle that are still in use today (2,3). Cushing's pupil, Walter Dandy, not as keen a clinical observ-er as Gushing though he had remarkable technical skill, was the first to carry out complete removal of tumors consistently. His lifetime operative mortality was 22.1%, and in one remarkable series toward the end of his career, operative mortality was 2.4% (4). In 1949 the importance of the anterior inferior cerebellar ar-tery as a vital stmcture was recognized (5). Sparing this stmc-ture reduced operative mortality further. In 1964 William House, an otologist working with neurosur-geon William Hitselberger, published his series of 53 tumors re-moved through a transtemporal bone microsurgical approach, called the translabyrinthine approach (6). House and coworkers have since removed more than 2,700 tumors with operative mortality below 0.5%. Moreover, facial nerve function was pre-served in the majority of cases and other neurologic deficits were rare (6,7). These results were achieved because the facial nerve can be identified lateral to the tumor with the surgical microscope us-ing secure bony landmarks. The nerve can then be followed me-dially to the tumor white the plane of separation between nerve and tumor is maintained. Manipulation of the brain stem and cerebellum is minimized, resulting in fewer intracranial compli-cations. Pafients are operated upon in the supine position, which avoids the rare complication of quadriplegia and the more com-mon, potentially fatal, venous air emboli sm that can occur when the pafient is operated upon in a sitting position (8). Other surgical approaches continue to be useful, including the middle fossa approach described by House, which permits the removal of small tumors and preserves hearing. The combined approach, described by Gushing and popularized in the contem-porary era by Glasscock (8), combines the advantages of the translabyrinthine and suboccipital approaches. Surgical ap-Submitted for publication: October 18, 1989. Accepted for publication: June 14, 1990. *Division of Otology and Neurotology, Henry Ford Hospilal. tDepartment of Neurological Surgery, Henry Ford Hospilal. Address cortespondence lo Dr. Monsell, Division of Otology and Neurotology, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202. Henry Ford Hosp Med J—Vol 38, No I , 1990 Acoustic Neuroma—Monsell & Rock 9 proaches and techniques confinue to evolve. Most centers offer a team approach combining the skills of an otologist, neurosur-geon, neuroanesthesiotogist, and neurophysiologist. The use of perioperative antibiotics, continuous arterial pressure monitor-ing, pulse oximetry, neuroanesthesia, intensive care units, and intraoperative facial nerve monitoring have improved the out-come of acoustic tumor surgery. Current operative mortality is in the range of 0.5% in most series. Ttimor Diagnosis Improvements in surgical techniques have been matched by improvements in diagnostic techniques. Audiology has devel-oped rapidly as a clinical discipline since the 1940s. It is now possible to measure hearing pure tone air and bone conduction thresholds, speech reception thresholds, and speech discrimina-tion. The introduction of the measurement of acoustic imped-ance has added the ability to determine the threshold and decay properties of the acoustic reflex (9). Auditory brain stem re-sponse (ABR) testing determines the integrity of the auditory nerve. The ABR is an auditory-evoked response and the most cost-effecfive screening test available for acoustic neuroma and other disorders of the auditory nerve (10). Its main limitafion is its dependence on intact hearing. Test subjects must have hear-ing at the 60 to 70 dB tevel or better in the high frequency range of 3,000 to 4,000 Hz; otherwise the recording may be uninter-pretable. The sensitivity of the test depends on the magnitude of the normal range, which is defined statistically and is generally in the range of 98%. For this reason, ABR is best used in the con-text of the ful l neurotologic evaluation. Imaging techniques were once limited to skull radiography that showed the outiine of the intemal auditory canals (lAGs). The IAG is usually widened in medium to large acoustic neuro-mas. Complex motion tomography combined with the introduc-tion of radiographic contrast material into the subarachnoid space allowed tumor detecrion and estimation of tumor size and boundaries. Computed tomography (CT) with intravenous radiographic contrast and air contrast in the cerebellopontine an-gle represented an advance that made early routine detection of even small tumors possible for the first time. Magnetic reso-nance imaging (MRI) with gadolinium contrast has become the "gold standard" diagnostic test for acoustic neuroma. This method is able to detect tumors as small as 2 to 3 mm and is the most accurate in determining tumor boundaries at any size. The history of acoustic neuroma diagnosis and the history of treat-ment are linked; as treatment has improved, the imperative for early diagnosis has grown. Clinical Characteristics Histologically, acoustic neuromas are benign tumors of the Schwann cefi sheath of the eighth cranial nerve. They grow slowly but continuously and, i f untreated, are eventually fatal in most cases. Regardless of the surgical technique used, risks and complications are fewer i f tumors are removed when small. Now that early tumor detection is possibte and the advantages of eariy diagnosis are well appreciated, there is a compelling need to pursue this diagnosis. Gushing showed that the earliest sign of acoustic neuroma is hearing loss. Today asymmetric hearing loss is still recognized as the most prevalent sign on presentarion, although patients may present with other symptoms (11). Hearing Loss Sensorineural hearing loss is prevalent in approximately 10% ofthe population, with the risk increasing to 20% by age 65 years. While the hearing loss rate of 2 to 3 dB/year after age 65 years may be a part of the normal aging process (presbycusis), an asymmetric sensorineural hearing loss at any age should be considered the result of an acoustic neuroma until proved oth-erwise. Moreover, tumors may present with dysequUibrium, numbness ofthe face, aural fullness, tinnitus, or aural pain. They may be detected as incidental findings in asymptomatic pa-tients. The incidence of acoustic neuroma has been estimated to be 0.8 to 1.0 per 100,000 population per year. Case Presentations Casel This healthy 16-year-old boy was sitting on his front porch when he noticed a ringing sound in his left ear and suddenly lost all hearing in the ear. He had no vestibular symptoms. He sought medical attention and was referred lo an otolaryngologist. Physical examination revealed the ears, head, and neck to be normal. Audiometric evaluation revealed a profound sensorineural hearing loss on the left. CT demonstrated a soft tissue mass filling the intemal auditory canal and extending 1 cm medial to the porous acusticus. He was referred to Henry Ford Hospital. A 1 cm tumor was removed via the translabyrinthine route in a com-bined neurotologic and neurosurgical procedure. Facial nerve function was normal postoperatively. Histopathologic examination ofthe tumor specimen established the diagnosis of schwannoma (acoustic neuro-ma). An organized hematoma was found in the tumor specimen. The patient's recovery was uneventful. Comments This case illustrates the occurrence of acoustic neuroma presenting as sudden sensorineural hearing loss. The presence of an old hematoma suggests that the sudden loss of hearing may have been caused by sud-den bleeding into the tumor. It has been established that acoustic neuro-ma may present as sudden hearing loss and that hearing may recover even in the presence of a tumor (12). Case 2 This 38-year-old woman presented to her otolaryngologist with a three-year history of intermittent mild dysequilibrium and a nine-month history of mild left hearing loss. Physical examination was un-remarkable. Audiometric evaluation revealed mild left sensorineural hearing loss and normal acoustic reflexes. An auditory-evoked poten-tial study revealed abnormality on the left side. CT showed mild wid-ening of the left intemal auditory canal. A faint shadow suggested the possibility of a mass in the cerebellopontine angle. The patient was re-ferred to Henry Ford Hospital. MRI with gadolinium enhancement demonstrated an enhancing mass extending from the fundus of the intemal auditory canal to the brain stem. The tumor was removed via the translabyrinthine route in a combined neurotologic and neurosurgical procedure. Facial nerve function was normal postoperatively. Histopathologic examinalion confirmed the diagnosis of acoustic neuroma. The patient's recovery was uneventful. 10 Henry Ford Hosp Med J—Vol 38, No 1, 1990 Acoustic Neuroma—Monsell & Rock Figure—Small left intracanaticular acoustic neuroma similar to that of case 3; left) axial and right) coronal section by MRI. Comments This case illustrates the presentalion of acoustic neuroma with mild hearing loss. Hearing preservation was not attempted because the tu-mor filled the internal auditory canal and extended to its fundus. CT only suggested the presence of a tumor, which was confirmed by MRI with gadolinium enhancement. Case 3 This 26-year-old man presenied wilh a one-year hislory of fluctuat-ing hearing loss and aural fullness without vestibular symptoms. Audiometric evaluation showed a mild righl sensorineural hearing loss. Subsequent evaluations demonstrated normal hearing. MRI with gado-linium enhancement showed a tumor in the intemal auditory canal, ex-tending 0.5 cm loward the brain stem. The acoustic neuroma was re-moved through the suboccipital approach with electrophysiologic monitoring of both auditory and facial nerves. Intraoperatively, the tu-mor was noted to be tightly adherent to the auditory nerve withoul a clear plane of separation. Postoperative hearing remained normal through 1,000 Hz but dropped precipitously at higher frequencies (Fig-ure). Postoperative speech discrimination was 60% and facial nerve function was normal. Comments This case illustrates that mild hearing loss usually associated with relatively small tumors may fluctuate and could even presenl as nor-mal. Even a hisloiy of hearing loss should be persued, despite initially negative findings. Hearing preservation is possible in some cases. Case 4 This 60-year-old woman was followed for several years because of hearing loss and episodic vertigo which were thought to be caused by Meniere's disease. She soughl a second opinion al Henry Ford Hospi-tal. Audiometric evaluation showed severe bilateral sensorineural hear-ing loss, with hearing better on the righl side. CT showed a 3 cm tumor in the right cerebellopontine angle. The acoustic neuroma was removed through the translabyrinthine approach. Postoperatively facial nerve function was normal, andthe patient's recovery was uneventful. She was referred for auditory rehabilitation. Comments This case illustrates that acoustic neuromas can mimic classical Meniere's disea.se. Whereas this patient had episodic vertigo of the Me-niere type, case 3 showed fluctuating hearing in the manner of early Meniere's disease. Acoustic neuroma should be excluded in patients with episodic vertigo, tinnitus, and hearing loss before that clinical syn-drome is accepted as diagnostic of Meniere's disease. Discussion We are in a new era in tumor management and in the man-agement of sensorineural hearing loss. Tumors can be diag-nosed when small, and operative complications are fewer if sur-gery is performed when tumors are small (13). Tumors present with common, seemingly minor symptoms, such as sensorineu-ral hearing loss. Whereas tumors are rare, sensorineural hearing loss is common. While it might be desirable for everyone with mild ear symp-toms to undergo M R I , health care resources are limited and scans are expensive. Unfortunately, we have no uniformly ac-cepted standard method to detect the rare acoustic tumors among the common cases of sensorineural hearing loss. Devel-opment of such a standard would need to take into account a monetary estimate of the "cost" of the morbidity added by delay in diagnosis. The costs of tests vary between institutions as does the rate of surgical complications. Tumor growth rates are un-predictably variable across individuals and over time for the same individual. Henry Ford Hosp Med J—Vol 38. No I , 1990 Acoustic Neuroma—Monsell & Rock 11 We recommend that patients be screened with air, bone, speech, and impedance audiometry. Patients with asymmetric hearing, asymmetric speech discrimination, or afferent limb acoustic reflex abnormalities should be evaluated further (9,14). We believe that the possibility of acoustic neuroma should be considered in atl patients with asymmetric sensorineural hear-ing toss or with other persistent unilateral or asymmetric ear symptoms. A tess aggressive approach can be taken in asympto-matic patients who are permanently debilitated or over age 65 years. References 1. House WF. A history of acoustic tumor surgery 1800-1900, early history. In: House WF, Luetje CM, eds. Acoustic tumors. Vol I . Diagnosis. Baltimore: University Park Press, 1979:4-5. 2. Gushing H. Tumors of the nervus acusticus and the syndrome of the cere-bellopontile angle. 2nd ed. New York: Hafner Publishing Co, 1963. 3. House WF. A history of acoustic tumor surgery 1900-1917, the Gushing era. In: House WF, Luetje CM, eds. Acoustic tumors. Vol 1. Diagnosis. Balti-more: University Park Press, 1979:9-23, 4. House WF. A history of acoustic tumor surgery 1917-1961, the Dandy era. In: Hou.se WF, Luetje CM, eds. Acoustic tumors. Vol 1. Diagnosis. Baltimore: University Park Press, 1979:25-32. 5. Atkinson WJ. Anterior-inferior cerebellar artery: Its variations, pontine dis-tribution, and significance in surgery of cerebello-pontine angle tumours. J Neu-rol Neurosurg Psychiatry 1949; 12:137-51. 6. House WF. Transtemporal bone microsurgical removal of acoustic neuro-mas: Report of cases. Arch Otolaryngol 1964;80:617-67. 7. House WF. Hitselberger WE. Surgical complications of acoustic tumor sur-gery. Arch Otolaryngol 1968;88:659-67. 8. Glasscock ME. Surgical techniques for the removal of acoustic tumors: A system of operations. Arch Otolaryngol 1968:88:618-27. 9. Clemis JD, Mastricola PG. Special audiometric test battery in 121 proved acoustic tumors. Arch Otolaryngol 1976:102:654-6, 10. Sellers WA, Brackmann DE. Acoustic tumor detection with brain stem electric response audiometry. Arch Otolaryngol 1977:103:181-7. 11. Bebin J. Pathophysiology of acoustic tumors. In: House WF, Luetje CM, eds. Acoustic tumors. Vol 1. Diagnosis. Baltimore: University Park Press, 1979:70, 12. Roland PS, Glasscock ME III, Bojrab DI, Josie AF. Normal hearing in pa-tients with acoustic neuroma. South MedJ 1987;80:166-9. 13. Brackmann DE. A review of acoustic tumors: 1979-1982. Am J Otol 1984;5:233-44. 14. Windle-Taylor PG, Dhillon RS, Kenyon GS, Morrison AW, Acoustic neuroma suspicion index: An aid to investigation and diagnosis. Laryngoscope 1984;94:1464-7. 12 Henry Ford Hosp Med J—Vol 38, No 1, 1990 Acoustic Neuroma—Monsell & Rock 

Sensorineural Hearing Loss and the Diagnosis of Acoustic Neuroma

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Sensorineural Hearing Loss and the Diagnosis of Acoustic Neuroma

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