What are the treatment options for severe sensorineural hearing loss with no response at multiple frequencies?

Treatment Options for Severe Sensorineural Hearing Loss with No Response at Multiple Frequencies

For patients with severe sensorineural hearing loss showing no response at multiple frequencies, audiologic rehabilitation with amplification devices or cochlear implants is the most effective treatment approach to improve quality of life, as medical interventions are unlikely to restore hearing in this scenario. 1

Diagnostic Confirmation

• Audiometric evaluation is essential to confirm the diagnosis of sensorineural hearing loss (SNHL), distinguishing it from conductive hearing loss 1
• Complete audiometric assessment should include:
  • Air and bone conduction threshold measures with appropriate masking 1
  • Speech audiometry measures in quiet and noise 1
  • Otoacoustic emission (OAE) measures to determine cochlear function 1

Imaging and Further Workup

• MRI of the brain and internal auditory canals is recommended as the imaging study of choice to rule out retrocochlear pathology such as vestibular schwannoma 1
• CT scan is not recommended as routine imaging for SNHL but may be considered in specific situations (patients with pacemakers, severe claustrophobia, or known bone disease) 1
• Auditory brainstem response (ABR) testing may be considered as a less expensive alternative screening tool when MRI cannot be performed 1
• Routine laboratory tests are not recommended unless specific etiologies are suspected based on history and physical examination 1

Treatment Options

1. Amplification Devices

• Hearing aids should be considered as first-line management for residual hearing 1
• For unilateral severe hearing loss, options include:
  • CROS (contralateral routing of signal) hearing aids that transmit sound from the affected ear to the better ear 1
  • BICROS (bilateral contralateral routing of signals) for those with preexisting hearing loss in the better ear 1
  • Monaural hearing aid options for those who can benefit from amplification in the poorer ear 1

2. Implantable Devices

• Osseointegrated bone conductive devices can transfer sound from the affected side to the better hearing cochlea 1
  • Available with headband placement for non-surgical candidates 1
• Cochlear implantation may be considered for severe to profound bilateral hearing loss when hearing aids provide insufficient benefit 1

3. Medical Management

• For sudden sensorineural hearing loss (SSNHL), corticosteroids may be offered as initial therapy if within 2 weeks of symptom onset 1
• Intratympanic steroid therapy may be offered as salvage therapy for incomplete recovery 2-6 weeks following onset of SSNHL 1
• Hyperbaric oxygen therapy may be considered when combined with steroid therapy within 2 weeks of onset (initial treatment) or within 1 month (salvage therapy) 1

Counseling and Support

• Audiologic rehabilitation should be addressed as soon as the hearing loss is identified 1
• Counseling should address:
  • Impact on communication, safety, function, cognition, and quality of life 1
  • Emotional aspects (embarrassment, frustration, anxiety, insecurity, loneliness, depression) 1
  • Realistic expectations regarding treatment outcomes 1

Follow-up Care

• Follow-up audiometric evaluation should be obtained at the conclusion of any treatment and within 6 months of completion 1
• Regular monitoring of hearing status is recommended to assess for any changes and adjust management accordingly 1

Important Considerations and Pitfalls

• Severe SNHL with no response at multiple frequencies is typically permanent, and patients should be counseled about realistic expectations 2, 3
• Delay in audiologic rehabilitation can lead to auditory deprivation and poorer outcomes 1
• The majority of patients with severe SNHL will not regain significant hearing through medical interventions alone 4, 3
• For asymmetric SNHL, further evaluation is warranted to rule out retrocochlear pathology 1, 5