Sudden Hearing Loss Guidelines

Updated: Apr 26, 2017
  • Author: Neeraj N Mathur, MBBS, MS, DNB(ENT), MNAMS, FAMS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Guidelines Summary

AAO-HNSF guidelines

The 2012 American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) clinical practice guidelines for sudden hearing loss strongly recommend that clinicians distinguish sensorineural hearing loss (SNHL) from conductive hearing loss (CHL) in patients presenting with sudden hearing loss. Patients should also be assessed for bilateral sudden hearing loss, recurrent episodes of sudden hearing loss, and focal neurologic findings, to determine whether the hearing loss is associated with a definable underlying disease. Under the AAO-HNSF guidelines, presumptive idiopathic sudden sensorineural hearing loss ( ISSNHL) should be diagnosed if audiometry confirms a 30-decibel (dB) hearing loss at three consecutive frequencies and no underlying condition has been identified. [13]

The AAO-HNSF guidelines recommend against computed tomography (CT) scanning of the head/brain when initially assessing presumptive SSNHL, as well as routine laboratory testing in patients with ISSNHL, but recommend that ISSNHL patients undergo magnetic resonance imaging (MRI), auditory brainstem response (ABR), or audiometric follow-up to assess for retrocochlear pathology. [13] .

​Even though most studies drew varying conclusions about the efficacy of steroid treatment for ISSNHL, the AAO-HNSF guidelines give clinicians the option of offering corticosteroids as initial therapy to patients with the condition. Additional treatment recommendations include the following [13] :

  • Hyperbaric oxygen therapy (HBOT) within 3 months of diagnosis of ISSNHL
  • Intratympanic (IT) steroid perfusion for patients with incomplete recovery after failure of initial treatment for ISSNHL
  • Antivirals, thrombolytics, vasodilators, vasoactive substances, or antioxidants should not be routinely prescribed in cases of ISSNHL
  • Follow-up audiometric evaluation within 6 months of diagnosis of ISSNHL
  • Patients with incomplete hearing recovery should be advised of the possible benefits of amplification and hearing-assistive technology (HAT) and other supportive measures

ACMG guidelines

The 2014 American College of Medical Genetics and Genomics (ACMG) guidelines for clinical evaluation and etiologic diagnosis of hearing loss are summarized below. Medical histories may be helpful in differentiating between acquired versus inherited causes of hearing loss. According to the ACMG, histories aimed at assessing hearing loss should include the following [22] :

  • Prenatal history of maternal infections (eg, cytomegalovirus, rubella, syphilis) or drug exposures (eg, thalidomide, retinoic acid)
  • Neonatal history, including premature birth, low birth weight, birth hypoxia, hyperbilirubinemia, sepsis, and exposure to ototoxic medications
  • Postnatal history, including viral illnesses, bacterial meningitis, head trauma, noise exposure, and exposure to ototoxic medications
  • Audiometric assessment of the hearing loss; age of onset; progressive, nonprogressive, or fluctuating nature of the hearing loss; laterality, symmetry, severity, and configuration of the hearing loss; and the presence or absence of vestibular dysfunction or auditory neuropathy

The family medical history should concentrate on the following [22] :

  • First- and second-degree relatives with hearing loss or with features commonly associated with hearing loss (such as pigmentary, branchial, or renal anomalies) or sudden cardiac death
  • A pattern of inheritance
  • Ethnicity and country of origin
  • A common origin from ethnically or geographically isolated areas
  • Consanguinity

The physical examination should focus on the following [22] :

  • Unusual facial appearance, with attention to asymmetry
  • Pigmentary anomalies
  • Neck, skin, facial, or ear anomalies
  • Neurologic abnormalities
  • Balance disturbances
  • Skeletal abnormalities
  • Other unusual physical findings

For individuals with physical findings and histories that do not suggest a known syndrome or environmental cause of hearing loss, a tiered diagnostic approach is needed that includes the following [22] :

  • Genetic counseling and, after informed consent, genetic testing
  • Single-gene testing in cases in which a specific etiology is suspected from the history and physical findings
  • In the absence of any specific clinical indications and for apparent autosomal recessive inheritance, testing for DFNB1-related hearing loss (due to mutations in  GJB2 and adjacent deletions in  GJB6)
  • If initial genetic testing is negative, consider genetic testing using gene panel tests and next-generation sequencing (NGS) technologies; however, the clinician must be aware of the genes included in the test (panel) and the performance characteristics of the platform chosen, including coverage, analytic sensitivity, and what types of mutations will be detected
  • Test results should be communicated through genetic counseling regardless of whether they are positive, negative, or inconclusive
  • If genetic testing reveals mutation(s) in a hearing loss–related gene, mutation-specific genetic counseling should be provided, followed by medical evaluations and referral to a multidisciplinary care center, when available
  • Consider temporal bone imaging by CT scanning or MRI, particularly if the diagnosis remains unclear, if cochlear implantation is being considered, if auditory neuropathy is noted, in cases of progressive hearing loss, or if other clinical concerns exist
  • If genetic testing fails to identify an etiology for a patient's hearing loss, the possibility of a genetic or acquired etiology remains and further genetic testing may be pursued in a research setting; periodic follow-up care every 3 years with a geneticist may be appropriate to identify syndromic forms of hearing loss that may appear as individuals age and to discuss new genetic tests that may have become available