Cochlear Migraine: Exploring Migraine’s Impact on the Auditory System

Hamid R. Djalilian, MD*, Daniel Keum, BS, Yalda Yazdani, MD, Mehdi Abouzari, MD, PhD, and Euyhyun Park, MD, PhD

*Author disclosures follow at the end of this article.

Cochlear migraine, first described in 2018, represents the effects of migraine on the auditory system. This condition is distinct from the more widely recognized vestibular migraine, which primarily affects the vestibular system.^1,2 Cochlear migraine manifests as recurrent episodes of auditory symptoms associated with migraine features. These symptoms are most commonly recurrent or fluctuating unilateral low frequency hearing loss without vertigo or mild dizziness that does not meet the criteria for vestibular migraine or Ménière’s disease along with aural fullness in the affected ear.

Clinical Features and Presentation of Cochlear Migraine

Additional minor clinical features include aura before hearing loss, concomitant tinnitus, family history of migraine-related disorders, unilateral neck stiffness (most commonly ipsilateral to the ear with symptoms), photophobia or phonophobia, motion sickness, visual motion intolerance, and sensitivity to atmospheric pressure changes.^2,3

The hallmark of cochlear migraine is the presence of auditory symptoms that occur in association with migraine or migraine-like symptoms in the absence of headaches. Importantly, these auditory manifestations often occur without the typical migraine headache at the same time or in the past.⁴ As such, a diagnosis of classic migraine is not necessary for the diagnosis of cochlear migraine. We consider cochlear migraine a subcategory of otologic migraine, which includes all the manifestations of migraine in the ear (Figure 1).

Figure 1. Different manifestations of migraine in the ear (otologic migraine). Ménière’s disease is at the intersection of cochlear and vestibular migraine, sometimes termed “cochleovestibular migraine.”

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Pathophysiological Mechanisms

Trigeminal Nerve Involvement
The exact pathophysiological mechanisms underlying cochlear migraine are not fully known, but several theories have been proposed (Figure 2). What is  known is that the ophthalmic branch (V1) of the trigeminal nerve extensively innervates the spiral modiolar artery and the stria vascularis.^5,6 In addition, stimulation of V1 leads to fluid extravasation within the cochlea within one hour.⁷ Therefore, activation of V1, can lead to changes in the cochlear blood supply and resultant changes within the cochlea with more fluid in the cochlea (i.e., hydrops). Fluid dysregulation in the cochlea and a state of hydrops affects the trigeminal innervation of the cochlea.⁸

Cortical Spreading Depression and Neurogenic Inflammation
Migraine is caused by cortical spreading depression, a phenomenon typically associated with migraine aura, which might affect auditory processing areas in the brain.⁹ The cortical spreading depression causes activation of the trigeminal nerve that can lead to many downstream effects. The trigeminal nerve, in turn, secretes inflammatory neuropeptides, such as calcitonin gene-related peptide (CGRP) and substance P.¹⁰

The trigeminal nerve innervates all the intracranial blood vessels as well as the dura in addition to other structures of the head and neck. The secretion of CGRP and other compounds leads to neurogenic inflammation and increased blood flow in the affected blood vessels.¹¹ Finally, trigeminal nerve activation can lead to a state of central sensitivity, where the brain can amplify sensory stimuli, thus causing many other symptoms in patients.

Mechanisms of Auditory Symptoms

Hearing Loss Mechanisms
According to new understanding of the role of migraine on the ear, the most likely etiology of fluctuating hearing loss is recurrent changes to cochlear blood flow due to trigeminal nerve activation.¹² Increased tinnitus and hyperacusis are due to the central sensitization that occurs as a result of the trigeminal nerve activation and neuroinflammatory process it causes.¹²

Tinnitus, Hyperacusis, and Aural Fullness
Sometimes, hyperacusis presents with pulsatile tinnitus as patients tend to hear internal sounds at a louder level than external sounds. In some patients, sudden brief unilateral tapering tinnitus (tinnitus lasting less than one minute)¹³ likely occurs from a temporary decrease in hearing caused by change in cochlear blood flow. However, the fluctuation or change in baseline constant tinnitus is due to central sensitization.¹² Aural fullness in patients with cochlear migraine is most likely due to activation of the tensor tympani and sustained contraction.^14,15

^{Figure 2. Central and peripheral mechanisms involved in the pathophysiology of otologic migraine.}

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Proposed Diagnostic Criteria

Lai et al.¹⁶ outlined “hypothetical” diagnostic criteria for diagnosing cochlear migraine. These criteria are:

• at least two episodes of migraine with cochlear symptoms with or without recovery
• current or previous history of migraine with or without aura according to the International Classification of Headache Disorders-3 (ICHD-3)
• one or more migraine features with at least 50% of the cochlear episodes
• no current or previous history of vestibular symptoms
• not better accounted for by another cochlear or ICHD-3 diagnosis

Based on these criteria, they have defined four types of cochlear migraine:

Type 1: Unilateral fluctuating sensorineural hearing loss (SNHL) with or without tinnitus—generally low frequency SNHL

Type 2: Repeated idiopathic sudden SNHL with or without tinnitus, with good recovery

Type 3: Sudden SNHL with or without tinnitus, which deteriorates with time

Type 4: Unilateral fluctuating tinnitus with or without aural fullness, otalgia or mild SNHL¹⁶

Our Clinical Observations

The current authors have found that nearly half of our patients with cochlear symptoms (fluctuating hearing loss, sudden SNHL, or fluctuating tinnitus) do not have a history of migraine headaches. This can lead to an underdiagnosis of this condition. We have found that a vast majority of cases with fluctuating hearing loss, recurrent sudden hearing loss, fluctuating tinnitus, loud bothersome tinnitus, change in the baseline level of tinnitus (quiet tinnitus becoming louder after a certain event), intermittent tinnitus, and hyperacusis to all be part of the family of cochlear migraine. Such patients overwhelmingly respond to migraine preventative diet and lifestyle changes as well as prophylactic medical treatment.^17,18

Treatment of Cochlear Migraine

Acute Management
The management of cochlear migraine typically involves a multifaceted approach. Acute management of episodes may include migraine-specific medications such as oral steroids that are used as a migraine abortive.¹⁹

Combined Therapy for Sudden Hearing Loss
In the case of sudden hearing loss, short-term use of oral and intratympanic steroids has been shown to improve hearing outcome. It is possible that the effect of intratympanic steroids is due to its effect on cochlear blood flow.²⁰ When traditional therapy for sudden hearing loss (oral and intratympanic steroids) has been combined with migraine prophylactic medications, improved outcomes have been seen, especially in the low frequencies.²¹

In a previous work by our group, we found that even in patients with long-term sudden hearing loss (>3 months in duration), 33% of patients with non-usable hearing (<50% word discrimination) had a significant enough improvement with this combination of therapies to improve to usable hearing levels (>50% word discrimination).²² On average, posttreatment word discrimination and speech-recognition threshold (SRT) were significantly improved (45 ± 28% vs. 70 ± 28% and 57 ± 18 dB vs. 50 ± 16 dB, respectively, both p < 0.01).

Interestingly, we observed ≥15% improvement in Word Recognition Score (WRS) and ≥10 dB improvement in SRT in 13 (68%) and 8 (40%) patients, respectively. Of the 11 patients who presented with initial <50% word discrimination, eight (73%) had improved posttreatment >50% WRS with an average improvement of 39 ± 9%. We found that patients with active migraine-related symptoms, e.g., headaches, neck stiffness, or aural fullness, had better outcomes.²² We routinely use migraine prophylaxis for patients with fluctuating hearing loss and have had great success in controlling fluctuations with this regimen.

Diet and Lifestyle Modifications
Lifestyle modifications to avoid migraine triggers (Table 1) and stress management techniques are critical components in the long-term management of cochlear migraine.²³ We also recommend an in-person or internet-based cognitive behavioral therapy (iCBT) directed toward migraine and tinnitus for our tinnitus patients.²⁴

Therapeutic Outcomes for Tinnitus
We have found that the combination of sound therapy and iCBT for these patients led to significantly higher improvements than control patients in their Tinnitus Functional Index (16.7 ± 14.9 vs. 1.9 ± 10.8, p < 0.001), and generalized anxiety disorders-7 (GAD-7) score of 1.1 ± 3.6 vs. -0.9 ± 3.3, p = 0.009. For patients with persistent auditory symptoms, additional interventions such as generic or customized sound therapy for tinnitus²⁵ or hearing aids for significant hearing loss may be necessary. We have found that health coaches are valuable in keeping patients on task with improvement in stress reaction, sleep optimization, and dietary modifications.²⁶

Table 1. Common Migraine Triggers

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Future Directions and Clinical Implications

Research Priorities
Cochlear migraine represents a unique and often overlooked subtype of migraine that specifically affects the auditory system. Its recognition as a distinct entity is crucial for appropriate diagnosis and management as many patients with cochlear symptoms (e.g., tinnitus and hyperacusis) are untreated or undertreated and can be improved with medical therapy. As our understanding of cochlear migraine continues to evolve, several important research directions emerge.

There is a pressing need to develop specific diagnostic criteria for this condition, which would facilitate more accurate diagnosis and targeted treatment. In addition, a neurophysiologic or laboratory test can help facilitate easier diagnosis. Animal model development can help facilitate better understanding of and treatment development for this condition.

Implications for Otolaryngologists
For otolaryngologists-head and neck surgeons, awareness of cochlear migraine is essential when evaluating patients with recurrent auditory symptoms, particularly those with recurrent sudden hearing loss, fluctuating hearing loss, certain subtypes of tinnitus (Figure 1), and hyperacusis. There needs to be a paradigm shift away from considering these symptoms to be caused by an otogenic cause toward a neurovascular and central sensitivity-related disorder.

The idea of neurovascular changes affecting the inner ear was first described in 1948 and 1949 by Jerome Hilger.^27,28 It took nearly five decades for the basic science work to demonstrate the trigeminal innervation of the cochlear blood supply and stria vascularis,⁵ and that stimulation of cranial nerve V leads to fluid extravasation within the cochlea.⁷ It took another 10 to 20 years to better understand cochlear migraine and develop treatment algorithms that are effective in its treatment. We are in a new era of understanding many otologic disorders that were previously considered idiopathic.

Conclusion
Cochlear migraine, like migraine headaches and vestibular migraine, is a complex disorder that has multiple triggering factors such as stress, disrupted sleep, diet (dehydration, hunger, certain foods), hormonal changes, and overstimulation. Treatment of complex disorders generally requires a combination of trigger control (e.g., lifestyle and dietary changes) in addition to medical therapy.

Patient education on lifestyle changes, use of iCBT, health coaches, and becoming familiar with the treatment regimen will allow otolaryngologists to help these traditionally underserved patients. With better understanding and treatment of cochlear migraine, telling patients that nothing can be done for tinnitus and chronic oral steroid therapy for fluctuating hearing loss is no longer necessary.

The views expressed are solely those of the authors and may or may not be shared by the AAO-HNS. The AAO-HNS does not endorse specific products, services, or techniques.

Author Disclosures: Hamid Djalilian, MD, has equity interest in Eliava Technologies, Cactus Medical LLC, and NeuroMedCare. He is an inventor on intellectual property owned by the University of California related to hearing loss and tinnitus treatment. The other authors do not have relevant disclosures.

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