Designed and created by Guideline Central in participation with the American Academy of Otolaryngology - Head and Neck Surgery Foundation
Sudden Hearing Loss
Objective
Objective
The purpose of this patient summary is to provide guidance on the diagnosis, management, and follow-up of sudden hearing loss. It focuses on sudden idiopathic sensorineural hearing loss in adult patients aged 18 years and older, and mainly on sudden sensorineural hearing loss with no obvious known cause.
Background and Definitions
Background and Definitions
- This patient summary focuses on idiopathic sudden sensorineural hearing loss. Idiopathic means that there is no clear cause.
- Definitions of types of hearing loss covered in this patient summary:
- Sudden Hearing Loss: A rapid-onset subjective sensation of hearing impairment in one or both ears.
- Sensorineural Hearing Loss: Hearing loss resulting from problems in the inner ear and/or the auditory nerve that connects the inner ear to the brain.
- Sudden Sensorineural Hearing Loss: A subset of sudden hearing loss that:
- Is sensorineural in nature, occurs due to a problem with the sound signals not reaching the brain properly.
- occurs within a 72-hour window
- meets certain audiometric criteria (referring to hearing test results).
- Other types of hearing loss, including conductive hearing loss and mixed hearing loss, are not covered in this patient summary.
- The cause of sudden sensorineural hearing loss is often unknown. If it is not recognized and treated promptly, it can result in long-lasting hearing loss and tinnitus (ringing in the ears), and reduced quality of life.
- Sudden sensorineural hearing loss is usually treated with one or more of the following:
- Steroid therapy with corticosteroids
- Medications taken by mouth to reduce inflammation
- Steroid therapy with intratympanic steroids
- Medications injected into the inner ear to reduce inflammation
- Hyperbaric oxygen therapy (in combination with steroid therapy)
- Hyperbaric oxygen uses oxygen to reduce inflammation
- Steroid therapy with corticosteroids
- The treatment options for sudden sensorineural hearing loss usually depend on the following:
- How long it has been since any initial symptoms started
- Whether previous therapies had been tried, and if so, if they were successful.
- Salvage therapy is any treatment given 2 weeks after the start of sudden hearing loss even if that initial treatment was just observation.
- Procedures used to diagnose and assess sudden sensorineural hearing loss include:
- Audiometry (hearing test)
- MRI or Auditory Brainstem Response
- MRI: gives a detailed image
- Auditory brainstem response: measures how sound travels to the brain
Evaluation and Diagnosis
Evaluation and Diagnosis
- The first step in evaluating sudden hearing loss is to identify the type of hearing loss that is happening. Specifically, sensorineural hearing loss should be identified, and other causes of hearing loss, including conductive loss, should be ruled out.
- When sudden sensorineural hearing loss is suspected, a history and physical examination is recommended to check for sudden hearing loss in both ears, recurrent episodes of sudden hearing loss, and/or focal neurologic findings.
- The following should NOT be used in the initial evaluation of sudden sensorineural hearing loss:
- Computed tomography (CT scan) of the head
- Laboratory testing
- In all cases of sudden hearing loss, audiometry should be performed within the first 14 days of the first symptoms. The goal is to confirm a diagnosis of sudden sensorineural hearing loss.
- If a diagnosis of sudden sensorineural hearing loss is confirmed, MRI or Auditory Brainstem Response can be used to help determine the best treatment plan.
Treatment
Treatment
- Healthcare providers should provide background information and education about sudden sensorineural hearing loss, including the typical course of the disorder, the benefits and risks of medical treatments and the limitations of existing evidence regarding the effectiveness of treatment.
- Corticosteroids (steroids) may be offered as the initial treatment for sudden sensorineural hearing loss. They can be used within the first 2 weeks after any initial symptoms.
- Hyperbaric oxygen therapy, combined with steroid therapy, is an option for treating sudden sensorineural hearing loss:
- within the first 2 weeks after any initial symptoms
- as salvage therapy within one month after any initial symptoms
- Intratympanic steroid therapy is recommended for sudden sensorineural hearing loss when other therapies have failed or when there is an incomplete recovery 2–6 weeks after the initial onset of symptoms.
- Routine use of antivirals, thrombolytics, vasodilators, or vasoactive substances is NOT recommended for sudden sensorineural hearing loss.
Monitoring and Follow Up
Monitoring and Follow Up
- Within 6 months of completion of treatment for sudden sensorineural hearing loss, a follow-up audiometric evaluation is recommended.
- For lasting hearing loss or tinnitus (ringing in the ears), it is recommended that education be provided about the possible benefits of audiological rehabilitation and other supportive measures.
Additional Information
Additional Information
Some Of the Symptoms and Signs of Non-Idiopathic Sudden Sensorineural Hearing Loss
- Sudden onset of bilateral hearing loss
- Antecedent fluctuating hearing loss on one or both sides
- Concurrent severe bilateral vestibular loss with oscillopsia
- Gaze evoked or downbeat nystagmus
- Concurrent eye pain, redness, lacrimation, and photophobia
- Focal neurological symptoms or signs such as headache, confusion, diplopia, dysarthria, focal weakness, focal numbness, ataxia, facial weakness
- Recent head trauma
- Recent acoustic trauma
- Recent barotrauma
Selected Conditions That May Be Associated With Bilateral Sudden Sensorineural Hearing Loss
Cause: Infection (viral including herpes simplex virus [HSV], varicella zoster virus [VZV], human immunodeficiency virus [HIV] and others — bacteria, mycoplasma, Lyme disease, tuberculosis, syphilis, fungal)
Other Features: Headache, fever, other cranial nerve palsies, abnormal cerebrospinal fluid (CSF) commonly seen in meningitis. pinna or ear canal vesicles and facial weakness are often seen in VZV (Ramsay Hunt Syndrome/Herpes Zoster Oticus).
Cause: Autoimmune inner ear disease
Other Features: Hearing fluctuation, vertigo
Cause: Ototoxic Medication
Other Features: Vestibular loss, oscillopsia
Cause: Trauma
Other Features: Temporal bone fracture with possible Battle’s sign, cochlear concussion without visible fracture, barotrauma
Cause: Lead poisoning
Other Features: Learning disabilities, other stigmata of lead poisoning
Cause: Genetic Disorders
Other Features: May be syndromic or non-syndromic and may present later in life
Cause: Mitochondrial Disorders including MELAS (Metabolic Encephalopathy, Lactic Acidosis and Stroke-like episodes) and others
Other Features: Confusion, stroke like spells, elevated lactate, MRI white matter changes, others with variable phenotypes
Cause: Stroke
Other Features: Vertigo, dysarthria, facial weakness, ataxia, nystagmus, unilateral numbness, abnormal CT or MRI or MR angiogram of the vertebro-basilar vasculature
Cause: Cogan’s syndrome
Other Features: Non-syphilitic interstitial keratitis of the cornea, hearing loss, vertigo
Cause: Neoplastic (neurofibromatosis II, bilateral vestibular schwannomas, carcinomatous meningitis, intravascular lymphomatosis, others)
Other Features: Abnormal brain MRI, cerebrovascular imaging study, or CSF
Cause: Sarcoidosis
Other Features: Pulmonary symptoms, bilateral vestibular loss, elevated angiotensin converting enzyme (ACE) level, abnormal gallium scan
Cause: Hyperviscosity syndrome
Other Features: Mucous membrane bleeding, neurologic and pulmonary symptoms, associated retinopathy
General Guidelines for Corticosteroid Therapy for Sudden Sensorineural Hearing Loss
Systemic Corticosteroids
Timing of Treatment: Immediate, ideally within the first 14 days. Benefit has been reported up to six weeks post onset of symptoms.
Dose: Prednisone 1 mg/kg/day (usual maximal dose is 60 mg/day) or Methylprednisolone 48 mg/day or Dexamethasone 10 mg/day
Duration/Frequency: Full dose for seven to 14 days, then taper over a similar time period.
Technique: Do not divide doses
Monitoring: Audiogram at completion of treatment course and at delayed intervals
Modifications: Medically treat significant adverse drug reactions, such as insomnia
Monitor for hyperglycemia, hypertension in susceptible patients.
Intratympanic Corticosteroids
Timing of Treatment: Immediate; Salvage (rescue) after initial treatment fails or after 2 weeks from symptom onset
Dose: Dexamethasone 24 mg/mL (compounded), or 10 mg/mL (stock) if compounded concentration unavailable or Methylprednisolone 40 mg/mL or 30 mg/mL
Duration/Frequency: Inject 0.4 to 0.8 mL into middle ear space up to four injections over a two-week period
Technique: Fill the middle ear with steroid solution. Keep head in otologic position (one side down, affected ear up) for 15–30 minutes.
Monitoring: Audiogram at completion of treatment course and at delayed intervals. Interval audiograms between injections may help direct early termination of therapy if hearing loss resolves. Inspect tympanic membrane to ensure healing at completion of treatment course, and at a delayed interval.
Modifications: May insert pressure-equalizing tube if planning multiple injections, but this increases risk of tympanic membrane perforation.
Frequently Asked Questions
Frequently Asked Questions
- What is causing the problem?
- The cause of sudden sensorineural hearing loss (SSNHL) is often not readily apparent and thus called idiopathic. It rarely affects both ears and can be associated with other symptoms such as ringing (tinnitus), dizziness (vertigo) and fullness in the ear.
- How is sudden hearing loss diagnosed?
- The sudden change in hearing is obvious to you and may be accompanied by loud ringing, vertigo (spinning sensation or balance problems), and/or pressure in the ear and should be evaluated as quickly as possible. Your health care provider will take a comprehensive history and complete a physical exam. Routine labs and x-rays are not recommended, but a hearing test (audiogram) should be done.
- Will my hearing come back?
- Approximately 1/3 to 2/3 of patients with SSNHL may recover some percentage of their hearing within two weeks. Those who recover half of their hearing in the first two weeks have a better prognosis. Patients with minimal change within the first two weeks are unlikely to show significant recovery. Additionally, patients with dizziness at the time of onset of SSNHL have a poorer prognosis.
- Is there additional testing needed with SSNHL?
- SSNHL can rarely be associated with benign tumors of the vestibular nerve. These tumors are called vestibular schwannomas and can lead to progressive hearing loss, balance problems and in some cases compression of the brainstem with severe neurologic symptoms. Your provider may order an MRI to screen for these tumors. While an MRI of the brain and internal auditory canals is the most sensitive test, some patients opt for an Auditory Brainstem Response (ABR. This is a less sensitive screening tool but is less expensive and does not require being in the confined space of the MRI machine. If the ABR is abnormal, good practice requires an MRI.
- How is sudden hearing loss treated?
- Many treatments have been proposed for SSNHL. Watchful waiting is an alternative to active treatment since between 1/3 and 2/3 of patients may recover hearing on their own and can be monitored with repeat hearing tests. Based on current research, clinicians may offer corticosteroids as initial therapy. This is most commonly given in pill form but can be done with an injection through the eardrum (intratympanic) for those patients for whom oral steroids are contraindicated. Although antivirals are commonly prescribed, there is insufficient evidence to support their effectiveness in treating sudden hearing loss. Hyperbaric oxygen may also be offered within 2 weeks of the initial diagnosis of SSNHL or up to 1 month in conjunction with steroids. Clinicians should offer salvage therapy (usually intratympanic steroids) for incomplete recovery after initial therapy. The benefits of therapy may include more prompt and complete recovery of hearing, but there are also side effects that must be considered when choosing among the available options. If the ABR is abnormal, good practice requires an MRI.
- What are the side effects of each treatment?
- Side effects vary with each treatment modality but may include such things as increased anxiety, pain, dizziness, elevated blood sugar, elevated blood pressure, depression or insomnia. You should have a conversation with your provider regarding the specific side effects associated with your treatment.
- What else can I expect?
- Sudden hearing loss can be frightening and may result in embarrassment, frustration, anxiety, insecurity, loneliness, depression and social isolation. Individual or group counseling can be helpful in supporting patients with SSNHL. Audiologic rehabilitation needs to be addressed as soon as the hearing loss is identified. This includes counseling and discussion of nonsurgical and surgical amplification and hearing restoration options. Clinicians should obtain follow up audiometry within 6 months of initial diagnosis of SSNHL.