II. Definitions

  1. Sudden Sensorineural Hearing Loss
    1. Rapid loss of Hearing within a 72 hour period
    2. Hearing Loss of 30 DB in at least 3 connected frequencies

III. Epidemiology

  1. Incidence: 5-20 per 100,000 people annually aged 40-60 years old

IV. Causes

  1. See Sensorineural Hearing Loss
  2. Background
    1. Idiopathic in 80-90% of cases
    2. Significant underlying pathology identified in 16% of cases
  3. Most common causes
    1. Autoimmune inner ear process
    2. Viral Infection
      1. Herpes Zoster Oticus (Ramsay Hunt Syndrome)
        1. Presents with severe Otalgia, ear vessicles, Bell's Palsy
      2. HIV Otitis
        1. Presents with Mastoiditis and other Cranial Nerve neuropathies
    3. Vascular event
      1. Vertebrobasilar Vascular Disease
        1. Presents with bilateral internal auditory artery Occlusion
        2. Brainstem infarct could also cause acute Hearing Loss
      2. Hyperviscosity Syndrome
        1. Presents with Retinopathy, mucous membrane bleeding, neurologic signs, pulmonary signs
  4. Other causes
    1. Neoplasms
      1. Neurofibromatosis II
      2. Bilateral Vestibular Schwannomas
      3. Intravascular Lymphomatosis
      4. Meningioma
      5. Hyperviscosity Syndrome
    2. Toxins
      1. Lead Poisoning
      2. Ototoxic Medications
    3. Infections
      1. Mononucleosis (Epstein-Barr Virus)
      2. Group A Streptococcus
      3. Herpes Simple Virus
      4. Herpes ZosterVirus
      5. HIV Infection
      6. Lyme Disease
      7. Meningitis
      8. Syphilis
    4. Trauma
      1. Head Injury
      2. Barotrauma
      3. Temporal BoneFracture
      4. Acute exposure to sudden loud noise >130 dB noise
    5. Miscellaneous
      1. See Ototoxic Medications
      2. Genetic disorders
      3. Sarcoidosis
      4. Meniere Disease
      5. Pregnancy
      6. Cerebrovascular Accident
      7. Cogan Syndrome
        1. Presents with interstitial Keratitis, Hearing Loss and Vertigo
      8. Mitochondrial disorders (MELAS)
        1. Presents with Mitochondrial Encephalopathy, Lactic Acidosis, Stroke-Like episodes

V. Symptoms

  1. Sudden onset Hearing Loss
  2. Typically unilateral (bilateral is rare)
  3. Sounds are harsh and distorted
  4. Associated with aural fullness
  5. Vertigo and Ataxia may be present

VII. Differential Diagnosis

VIII. Evaluation

  1. Labs
    1. Not indicated unless dictated by history (e.g. Lyme Titer)
  2. Urgent Audiogram
    1. Perform same day of presentation
    2. Confirms Sensorineural Hearing Loss
    3. Repeat at 6 months from initial Audiogram

IX. Diagnosis: Audiogram

  1. Rapid loss of Hearing within a 72 hour period
  2. Hearing Loss of 30 DB in at least 3 connected frequencies

X. Imaging

  1. Indicated if SSHNL confirmed by Audiogram
  2. MRI with gadolinium
    1. Evaluates retrocochlear lesion (e.g. Schwannoma)
    2. CT Head is not recommended as alternative

XI. Management

  1. Sudden Sensorineural Hearing Loss requires immediate evaluation
    1. Otolaryngology evaluation is recommended within 24 hours
  2. Consider antiviral medications
  3. Corticosteroids
    1. Targets inflammatory cell death cascade in Sudden SNHL
    2. Standard of care treatment, but does not appear to significantly improve outcomes
      1. Labus (2010) Laryngoscope 120(9): 1863-71 [PubMed]
    3. Systemic Corticosteroids orally
      1. Typical adult dose: 60 mg for 5 days and then taper off over next 5 days
    4. Intratympanic Corticosteroids by Otolaryngology
      1. Indicated for patients not recovering spotaneously or after initial treatment
    5. Hyperbaric oxygen
      1. May be beneficial in first 3 months
  4. Other measures used
    1. Diuretics
    2. Plasma expanders
    3. Immunosuppressants (in addition to Corticosteroids as above)
  5. Measures without evidence of benefit
    1. Antiviral agents
      1. Consider only if suspected underlying viral etiology (e.g. HSV, VZV)
      2. Previously 10 days of Valacyclovir, Famciclovir, or Acyclovir was considered
    2. Thrombolytics
    3. Vasodilators
    4. Vasoactive substances
    5. Antioxidants

XII. Prognosis

  1. Spontaneous recovery in up to 70% of idiopathic cases

XIII. References

  1. Browning (2008) Park Nicollet Primary Care Update, Lecture, St. Louis Park, MN
  2. Orman and Finley (2018) EM:Rap 18(2): 15-6
  3. Michels (2019) Am Fam Physician 100(2): 98-108 [PubMed]
  4. Rauch (2008) N Engl J Med 359(8): 833-40 [PubMed]
  5. Stachler (2012) Otolaryngol Head Neck Surg 146(3 Suppl): S1-35 [PubMed]

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Related Studies

Ontology: Hearing Loss, Sudden (C0011057)

Definition (MSH) Sensorineural hearing loss which develops suddenly over a period of hours or a few days. It varies in severity from mild to total deafness. Sudden deafness can be due to head trauma, vascular diseases, infections, or can appear without obvious cause or warning.
Concepts Finding (T033)
MSH D003639
ICD9 388.2
ICD10 H91.2
SnomedCT 155247008, 194391007, 79471008
English Sudden hearing loss, unspecif., Sudden hearing loss, unspecified, Unspecified sudden hearing loss, Sudden idiopathic hearing loss, sudden loss of hearing, sudden loss of hearing (symptom), sudden hearing loss, Hearing Loss, Sudden [Disease/Finding], sudden hear loss, hearing loss sudden, Sudden hearing loss NOS, Unspecified sudden hearing loss (disorder), Unspecified sudden hearing loss (finding), Hearing Loss, Sudden, Sudden Hearing Loss, Sudden hearing loss, Sudden hearing loss, NOS, Sudden hearing loss (finding), Sudden hearing loss (disorder)
Dutch niet-gespecificeerd plotseling gehoorverlies, plots gehoorverlies NAO, Plotseling gehoorverlies zonder bekende oorzaak, plots gehoorverlies, Gehoorverlies, plotseling, Plotselinge doofheid, Plotseling gehoorverlies
French Perte auditive subite, non précisée, Perte soudaine de l'audition SAI, Perte soudaine de l'audition, Perte d'audition subite, Perte d'audition brusque, Baisse soudaine de l'acuité auditive, Déficience auditive soudaine, Perte auditive soudaine
German Gehoersturz, unspezifisch, Hoersturz NNB, Idiopathischer Hoersturz, Hoersturz, Hörverlust, plötzlicher, Plötzlicher Hörverlust
Italian Perdita improvvisa dell'udito NAS, Perdita improvvisa dell'udito, non specificata, Perdita improvvisa dell'udito
Portuguese Perda de audição repentina NE, Défice auditivo súbito NE, Perda de audição repentina, Perda Auditiva Súbita
Spanish Pérdida súbita del oído, de origen indeterminado, Pérdida súbita del oido NEOM, pérdida auditiva súbita (hallazgo), pérdida de la audición súbita (trastorno), hipoacusia súbita, pérdida de la audición súbita, pérdida auditiva súbita, pérdida auditiva súbita (trastorno), pérdida auditiva súbita no especificada (trastorno), Unspecified sudden hearing loss, pérdida auditiva súbita no especificada, pérdida auditiva súbita no especificada (hallazgo), Pérdida súbita del oído, Pérdida Auditiva Súbita
Japanese 詳細不明の突発難聴, 突発難聴NOS, ショウサイフメイノトッパツナンチョウ, トッパツナンチョウNOS, トッパツナンチョウ, 突発性聾, 突発難聴, 聾-突発性, 突発性難聴, 難聴-突発性
Swedish Plötslig dövhet
Czech nedoslýchavost náhlá, Náhlá ztráta sluchu NOS, Náhlá ztráta sluchu, blíže neurčená, Náhlá ztráta sluchu
Finnish Kuulon äkillinen heikkenimen
Korean 돌발성 특발성 난청
Polish Utrata słuchu nagła, Ogłuchnięcie nagłe, Głuchota nagła
Hungarian Nem meghatározott hirtelen hallásvesztés, hirtelen hallásvesztés k.m.n., hirtelen hallásvesztés
Norwegian Plutselig hørselstap, Hørselstap, plutselig