#379 Hearing Loss, Tinnitus, and Meniere’s Disease

January 30, 2023 | By Kate Grant

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With Dr Steven Rauch

Demystify hearing loss, identify who will benefit from hearing aids and learn to evaluate hearing complaints in primary care including an overview of tinnitus and how to recognize and treat Meniere’s disease with guest expert, Dr. Steven Rauch (Harvard University).

Show Segments

• Intro, disclaimer, guest bio
• Case from Kashlak; 
• Definitions, etiology and risk factors for hearing loss 
• Initial workup for hearing loss
• What is an audiogram and what does it show 
• Hearing loss red flags 
• How to manage hearing loss
• Tinnitus: what can help patients 
• Diagnose, distinguish and treat Meniere’s disease 
• Outro

Hearing loss and Tinnitus Pearls

1. Hearing loss in adults is common with aging, and affects one-third of the population above age 65 years and more than two-thirds of adults over age 75 years.
2. Hearing loss in elderly patients can be mistaken for cognitive decline, and impairs effective communication. 
3. All patients complaining of hearing loss should be referred for audiology.
4. Sudden or rapidly progressive unilateral hearing loss needs an urgent referral to ENT.
5. Hearing aids are amplifiers.  They improve volume but not clarity.
6. Persistent tinnitus can cause significant morbidity.  Lifestyle measures and counseling helps.
7. Meniere’s disease is uncommon.  Consider vestibular migraine as a more common cause for dizziness.
8. Patients with Meniere’s disease are recommended to balance sodium intake throughout the day, avoiding large variations to minimize symptoms.

Hearing Loss, Tinnitus, and Meniere’s Disease Show Notes

Hearing Loss

Epidemiology and Etiology

• Age is one of the biggest risk factors for hearing loss, with about one-third of the US population above the age of 65 and over two-thirds above age 75 have hearing loss that would benefit from hearing aids (NIDCD).
• Presbycusis can start at any age and is a degenerative process. Genetic factors play a part (Tawfik 2020).
• The most important modifiable risk factor for hearing loss is avoiding occupational and recreational loud noise. Ototoxicity from drugs is important but less common.
• Many patients do not notice hearing impairment due to its slow progression; family are often more likely to identify hearing loss. 
• Hearing impairment carries a stigma that can make some patients defensive (Neiman 2020).
• Hearing loss can make patients appear to have memory loss, and there is increasing research that hearing loss may increase dementia risk (Brewster 2022).

Simple Hearing Assessment in the Office 

• Otoscopy: evaluate external ear for physical impairment (wax, objects) and tympanic membrane integrity
• Finger rub: a simple test to assess hearing impairment in the office. 
• “The Hum Test” described by Dr Rauch: Ask the patient to make a “humming” sound and block one of the ears with a finger.  If the sound lateralizes to the ear that is impaired, it probably is a conductive impairment. But if it lateralizes to the contralateral ear, this suggests a sensorineural hearing loss. It has the same logic as the Weber test, but you do not need to use a tuning fork. 
• All patients complaining of hearing loss should be referred to an audiologist and/or otolaryngologist for evaluation and management. 

Audiograms 

Audiograms are used to diagnose conductive or sensorineural hearing loss across audible frequencies.   Dr Rauch highlights two of audiograms’ key parts: 

• Hearing threshold (loudness): The lowest intensity of a sound a person can hear at different frequencies in a pure tone.  This is measured in decibels (dB).  Age-related hearing loss typically affects the high frequencies.  Hearing loss below -30dB may benefit from hearing aids. 
• Hearing clarity: This is the ability to recognise words when presented at an audible level.  A ‘Word Recognition Score’ assesses clarity and gives more insight into hearing ability.  Score can predict how successful hearing aids will be when volume is corrected.  Normal hearing will equate to a Word Recognition Score typically above 90%.  Once word recognition (clarity) declines it is typically permanent, and boosting the volume with hearing aids doesn’t always help.

Red Flag- Idiopathic Sudden Unilateral Hearing Loss

Unilateral hearing loss that develops over 72 hours or less (in patients for whom other etiologies such a stroke have been ruled out) is a hearing loss emergency that requires immediate referral to ENT to reduce potential permanent loss.  Patients can wake up deaf on one side, or experience rapidly progressive loss in a few hours.  It can be associated with clicking sounds or balance disturbance.  Patients might attribute a benign cause, such as earwax or congestion, and may delay seeking medical attention. Sudden sensorineural hearing loss should be evaluated with an audiogram, and the specialist may consider MRI to rule out acoustic neuroma (Fishman 2018, Ahsan 2015).  Patients are recommended to be evaluated by audiology and ENT within 24 hours of referral or within 14 days of hearing loss onset depending on the guideline makers respectively (NICE 2019, Chandrasekhar 2019).  There may be a benefit to urgent high dose oral or intratympanic steroid treatment to improve outcomes after sudden sensorineural hearing loss, but this is primarily based on expert opinion and data is not strong (Plontke 2022, Wei 2013, Chandrasekhar 2019). In his experience, Dr. Rauch estimates about 80% will have some continued hearing loss even with intensive treatment.

“Reddish Flag”- Asymmetric Sensorineural Hearing Loss

Asymmetric sensorineural hearing loss is concerning for intracranial pathology and referral to ENT and consideration for evaluation for acoustic neuroma/vestibular schwannoma or other brain pathology should be considered (Sweeney 2018).

Chronic Management of Hearing Loss

Following an audiogram, hearing aids are prescribed according to the loss and the type or fit of the device.  Frequencies and volume can be adjusted in the future if the hearing loss worsens. Dr. Rauch states that any stigma about wearing hearing aids may be overcome if you explain the benefits of improved communication. Companies typically allow money back guarantee for the devices and many states (but not all of them) mandate a return period.   Some patients find it hard to adjust to the new acoustics they hear with an aid.

Over the counter hearing aids have recently been approved in the US.  In Dr. Rauch’s opinion, these devices offer the significant benefit of costing around one-third of a traditional hearing aid prices, but at this point, it is difficult to guarantee the quality and durability of those alternative aids.  Patients might buy over the counter aids and benefit from scheduling audiologist appointments to learn how to use the aids. 

Some hearing deficits can not be improved by traditional hearing aids. The only alternative available treatment is a cochlear implant. But, according to Dr Rauch, the cochlear implants only significantly improves quality of hearing for those patients that have a word recognition score below 50% and they can still struggle in a noisy environment (Varadarajan 2021).

People with unilateral hearing loss often have difficulty localizing where sounds are coming from, and some research shows significant impact on hearing and comprehension (Snapp 2020).  In the US, unilateral hearing loss has not been regarded as a medical-legal disability (Mengel vs Reading Eagle Co).  After proper workup, if there are no actionable findings, in Dr. Rauch’s opinion, patients should be reassured that they usually adjust to the changes. 

Tinnitus

“Tinnitus is just a generic indication of an unhappy ear” says Dr. Rauch.  Tinnitus is often caused by hearing loss.  When there is damage to the auditory process, the neurological auditory centers in the brain that receive and process the sound signal increase the gain to attempt to hear and these amplified signals are perceived as tinnitus.  Distracting from the tinnitus can help lessen symptoms.

Pulsatile tinnitus should be investigated, as it can be a sign of vascular disease (Grierson 2018).

Tinnitus Management

Tinnitus is often accompanied by hearing loss, so hearing assessment is useful. 

Tinnitus is often exacerbated by stress, sickness, sleep deprivation, muscle tension.  Lifestyle modifications and improving quality of life can diminish the bothersome effect of tinnitus. 

Encourage patients to use self help resources:

The British Tinnitus Association

American Tinnitus Association

Meniere’s Disease 

Pathophysiology

Meniere’s Disease is a degenerative disease of the inner ear that impairs sensorineural hearing and balance.  Dr Rauch looks at this as there is a degenerative process that we don’t yet understand, and the ear’s response to this is the symptoms of Meniere’s, not that Meniere’s is a specific disease causing inner ear damage. The exact etiology is unknown.  It can be due to a trauma, a congenital abnormality, or ear infection.  One-third of Meniere’s disease patients have a congenital malformation of the vestibular aqueduct, which is a canal where inner ear fluid recycling happens.  Abnormal fluid recycling can be due to aqueduct hypoplasia, degenerative changes in the epithelial lining of the endolymphatic sac, leading to homeostatic failure of inner ear (Rauch 2016).  

Diagnosis

Onset is often in the fourth or fifth decade, and the degenerative process leads to loss of function of the inner ear.  Fluctuating and progressive episodes of vertigo last a few hours. Vertigo episodes commonly come in clusters and are associated with tinnitus, progressive unilateral hearing loss, and aural fullness (Paparella 2008).  

A clinical diagnosis of Meniere’s Disease is made based upon the following criteria (Lopez-Escamez 2015):

• Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours.
• Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear.
• Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear
• Other differentials have been excluded. 

Around 25% of Meniere’s disease patients will develop bilateral symptoms, but it typically occurs sequentially, not concurrently (Noij 2019). 

Migraine vs Meniere’s

There is a significant overlap with Meniere’s symptoms and migraine.  Vestibular Migraine may cause episodic vertigo, hearing impairment, and tinnitus. Dr. Rauch suggests assessing for migraine symptoms (headaches, nausea, visual disturbance, photophobia) especially in young female patients, as migraine is a more common diagnosis.  If clinically there are strong suggestions of migraine, focusing on migraine treatment is appropriate.

Imaging 

Meniere’s is a clinical/audiologic diagnosis.  MRI is optional according to AAOHNS guidelines. Dr Rausch’s practice is to get an MRI on new patients with Meniere’s to rule out structural causes, and he notes that with new technology, changes associated with Meniere’s can be seen on MRI scan (Conte 2018, Sang Cho 2021), but this is not yet widely available.

Treatment of Meniere’s 

Dr Rauch recommends a patient-centered, conservative approach, controlling their symptoms with minimum intervention if possible.  Patients should be counseled on the following:

• Diet and establishing a regular schedule should be first line treatments, including  minimizing stress, improving sleep, and regular mealtimes.
• Dietary salt intake should be balanced throughout the day, avoiding large variation.  Replace fluid and electrolytes before and during exercise.
• For those few patients that have severe symptoms other treatments are available, such as gentamicin (or methylprednisolone) intratympanic infusion (Guan 2021, Rausch 2016).
• Diuretics are commonly used to reduce vertigo crises, but the evidence is not strong. (Shavit 2019, AAOHNS guidelines).  

Take Home Points

1. Be mindful of patients with hearing loss, because doing so helps you better address their healthcare needs.
2. Hearing loss erodes patients’ quality of life.
3. A quick ‘finger rub’ test is an easy office test to assess if they need a referral to an  audiologist. 

Links

1. https://rnid.org.uk Royal National Institute for Deaf People
2. https://www.nad.org National Association of the Deaf
3. The World Health Organisation (WHO) launched a free smartphone app that allows you to
   check your hearing health and track it over time.

Goals
Listeners will gain confidence in assessing hearing loss in adults, spot the red flags that indicate
urgent referral to a specialist, and provide management advice for tinnitus and Meniere’s disease.
Learning objectives
After listening to this episode listeners will…

1. Assess and risk stratify adults with hearing loss
2. Recognize which patients will benefit most from hearing aids
3. Develop a narrative to discuss tinnitus with patients
4. Counsel patients to reduce Meniere’s disease vertigo crises.

Disclosures
Dr. Steven Rauch reports no relevant financial disclosures. The Curbsiders report no relevant
financial disclosures.
Citation
Grant K, Perdigão A, Rauch S, Williams PN, Watto MF. “#379 Hearing loss, Tinnitus and Meniere’s
disease”. The Curbsiders Internal Medicine Podcast. http://thecurbsiders.com/episode-list January
30, 2023