eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Sudden Hearing Loss

Author: Neeraj N Mathur, MBBS, MS, Professor, Department of Ear, Nose and Throat, Lady Hardinge Medical College and Associated Smt SK and Kalawati, Saran Children's Hospital, University of Delhi, India; Professor, Department of Ear, Nose and Throat, BP Koirala Institute of Health Sciences, Nepal
Coauthor(s): Michele M Carr, DDS, MD, MEd, Associate Professor, Department of Otolaryngology, Hershey Medical Center
Contributor Information and Disclosures

Updated: Feb 6, 2009

Introduction

Background

Definitions of sudden hearing loss have been based on severity, time course, audiometric criteria, and frequency spectrum of the loss. Abrupt as well as rapidly progressive losses have been included under a single definition of sudden hearing loss. Awakening with a hearing loss, hearing loss noted over a few days, selective low- or high-frequency loss, and distortions in speech perception have all been classified as sudden hearing losses. A commonly used criterion to qualify for this diagnosis is a sensorineural hearing loss of greater than 30 dB over 3 contiguous pure-tone frequencies occurring within 3 days' period. Fortunately, the vast majority of cases of sudden hearing loss are unilateral, and the prognosis for some recovery of hearing is good. Usually it presents as unilateral loss of hearing; bilateral involvement is rare, and simultaneous bilateral involvement is very rare.

Sudden deafness or sudden sensorineural hearing loss (SNHL) has many possible etiologies.

Pathophysiology

The postulated pathophysiology for idiopathic sudden sensory hearing loss (ISSHL) has 4 theoretical pathways, as follows:

  • Labyrinthine viral infection
  • Labyrinthine vascular compromise
  • Intracochlear membrane ruptures
  • Immune-mediated inner ear disease.

A disease process involving any of these theoretical possibilities could have sudden hearing loss as a symptom. Each theory may explain a fraction of the episodes of sudden sensory hearing loss, but none of the existing theories individually could account for all episodes.

Viral infection

The evidence to implicate viral infection as one cause of sudden idiopathic sensory hearing loss is circumstantial. Studies of patients with ISSHL show a moderate prevalence of recent viral-type illness. Sometimes, evidence of recent viral seroconversion or inner ear histopathology consistent with viral infection is present.

The weakest of these links is the history of a recent viral illness. Noncontrolled studies report that 17-33% of patients recall a recent viral illness. Should those numbers seem significant, 25% of patients without hearing loss visiting an otolaryngology clinic had experienced a viral-like illness within a month.

Comparing patients experiencing ISSHL with control patients has produced some evidence of viral seroconversion. Rates of seroconversion for the herpesvirus family were significantly higher in the population of patients with sudden hearing loss.

Finally, temporal bone histopathologic studies of patients who experienced ISSHL found damage in the cochlea consistent with viral injuries. Loss of hair cells and supporting cells, atrophy of the tectorial membrane, atrophy of the stria vascularis, and neuronal loss were observed. These patterns were similar to findings in documented cases of hearing loss secondary to mumps, measles, and maternal rubella. Viral infection can be implicated as a cause of ISSHL, but this cannot, as yet, be proven. Infections with mumps virus provide the best model for a virally induced sensorineural hearing loss. In one study of ISSHL, subclinical mumps infections were documented in 9 of 130 patients by positive immunoglobulin M (IgM) mumps antibodies.1

Vascular compromise

The cochlea is an end organ with respect to its blood supply, with no collateral vasculature. Cochlear function is exquisitely sensitive to changes in blood supply. Vascular compromise of the cochlea due to thrombosis, embolus, reduced blood flow, or vasospasm seems to be a likely etiology for ISSHL. The time course correlates well with a vascular event, a sudden or abrupt loss. A reduction in oxygenation of the cochlea is the likely consequence of alterations in cochlear blood flow. Alterations in perilymph oxygen tension have been measured in response to changes in systemic blood pressure or intravascular carbon dioxide partial pressure (pCO2).

Histologic evidence of cochlear damage following occlusion of the labyrinthine vessels was documented in temporal bone studies in animals and humans. Intracochlear hemorrhage was noted as an early development; subsequently, fibrosis and ossification of the cochlea evolved.

In one study, a partial overlap was found between classical coronary risk factors and risk factors for sudden hearing loss. Hypercholesterolemia and hypoalphalipoproteinemia (low HDL cholesterol levels) were not found to be apparent major risk factors for sudden hearing loss, whereas the GPIa C807T polymorphism, elevated fibrinogen levels, and smoking were associated with an increased risk for ISSHL. Altogether these findings suggested a vascular involvement in the pathogenesis of ISSHL. This may have important implications for the development of therapeutic and preventive strategies for ISSHL.2

Intracochlear membrane rupture

Thin membranes separate the inner ear from the middle ear, and within the cochlea, delicate membranes separate the perilymphatic and endolymphatic spaces. Rupture of either or both sets of membranes theoretically could produce a sensory hearing loss. A leak of perilymph fluid into the middle ear via the round window or oval window has been postulated to produce hearing loss by creating a state of relative endolymphatic hydrops or by producing intracochlear membrane breaks. Rupture of intracochlear membranes would allow mixing of perilymph and endolymph, effectively altering the endocochlear potential. The theory of intracochlear membrane rupture was favored by Simmons and Goodhill, and histologic evidence has been documented by Gussen.3,4,5

Immune-mediated inner ear disease

Sensorineural hearing loss induced by an immune process has gained greater and greater notoriety since the concept was introduced in 1979. Progressive sensorineural loss is observed with this condition. Whether or not sudden hearing loss occurs with immune-mediated inner ear disease is unclear, but immunologic activity in the cochlea is supported by greater and greater evidence. The association of hearing loss in Cogan syndrome, systemic lupus erythematosus, and other autoimmune rheumatologic disorders has been well documented. With better markers for inner ear autoimmunity, perhaps a greater linkage with ISSNHL will be found. A recent prospective study on 51 patients with ISSNHL supported the existence of multiple immune-mediated disorders in these patients.6

Frequency

United States

Estimates of the annual incidence of sudden sensory hearing loss range from 5-20 cases per 100,000 persons. Many cases likely go unreported, and the incidence may be higher. A sudden hearing loss may resolve before the patient can be evaluated medically, making it unlikely for that individual to seek care.

Sex

The female-to-male distribution appears to be equal. Combined data from several studies show a slight male preponderance, at 53%. However, a single large study of 1220 patients had slightly more females.7 Sex does not seem to be a risk factor. An equal distribution of right- and left-sided cases should be expected. As with sex, no greater risk for right-sided losses compared with left-sided losses seems to exist. Bilateral sudden hearing loss occurs in approximately 1-2% of cases.

Age

People of all age groups are affected by sudden hearing loss, but fewer cases are reported in children and the elderly. The peak incidence appears to be in the sixth decade of life. Young adults have incidence rates similar to those of middle-aged adults. The median age at presentation ranges from 40-54 years. The occurrence of sudden hearing loss across all age groups is an indication of the multifactorial nature of this clinical problem.

Clinical

History

Sudden sensorineural hearing loss has been called an otologic emergency. Patient evaluation should proceed promptly and expeditiously. Early presentation to a physician and early institution of treatment improves the prognosis for hearing recovery. The immediate goal is discovering a treatable or defined cause of the sudden hearing loss.

  • Information about the onset, time course, associated symptoms, and recent activities may be helpful.
  • Past medical history may reveal risk factors for hearing loss.
    • All medications, including over-the-counter products, must be described.
    • Aspirin can cause a reversible sensorineural hearing loss, and the list of aspirin-containing products is extensive.

Physical

Perform a careful head and neck examination, with special attention to the otologic and neurologic examination.

Tuning fork tests and a fistula test using pneumatic speculum must be performed.

Causes

  • The term sudden hearing loss encompasses defined causes and ISSHL. In the evaluation of a sudden sensorineural hearing loss, a definite etiology may be uncovered. The following have been associated with sudden hearing loss:
  • The greater number of cases, however, fits into the idiopathic category. ISSHL is the frustrating endpoint for most patients with sudden hearing loss.
Despite the detailed history taking, physical examination, and laboratory investigations, ascertaining the pathophysiology of the events may not be possible; therefore, the cause and treatment of these cases is more presumptive than factual.

More on Inner Ear, Sudden Hearing Loss

Overview: Inner Ear, Sudden Hearing Loss
Differential Diagnoses & Workup: Inner Ear, Sudden Hearing Loss
Treatment & Medication: Inner Ear, Sudden Hearing Loss
Follow-up: Inner Ear, Sudden Hearing Loss
References
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Further Reading

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Keywords

hearing loss, sudden hearing loss, sudden deafness, sudden sensorineural hearing loss, idiopathic sudden sensory hearing loss, ISSHL, hearing problems, hearing

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Contributor Information and Disclosures

Author

Neeraj N Mathur, MBBS, MS, Professor, Department of Ear, Nose and Throat, Lady Hardinge Medical College and Associated Smt SK and Kalawati, Saran Children's Hospital, University of Delhi, India; Professor, Department of Ear, Nose and Throat, BP Koirala Institute of Health Sciences, Nepal
Neeraj N Mathur, MBBS, MS is a member of the following medical societies: Association of Otolaryngologists of India, Cochlear Implant Group of India, Indian Medical Association, National Academy of Medical Sciences, India, Neuro-Otologic and Equlibriometric Society of India, and Royal Society of Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Michele M Carr, DDS, MD, MEd, Associate Professor, Department of Otolaryngology, Hershey Medical Center
Michele M Carr, DDS, MD, MEd is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

Cliff A Megerian, MD, FACS, Medical Director of Adult and Pediatric Cochlear Implant Program, Vice-Chairman and Director of Otology and Neurotology, University Hospitals of Cleveland; Professor, Department of Otolaryngology-Head and Neck Surgery and Neurological Surgery, Case Western Reserve University School of Medicine
Cliff A Megerian, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Association for Research in Otolaryngology, Massachusetts Medical Society, Society for Neuroscience, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, School of Human Development.
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Alcon labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear corp Honoraria Board membership; Med El corp travel grants Speaking and teaching; Insight vision Consulting fee Consulting

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
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