Symptom: Vertigo After Labyrinthectomy

Abstract

A 61-year-old man with a history of left-sided Meniere’s disease first diagnosed 10 years ago presented with severe headaches, vertigo, aural pressure, otalgia, tinnitus, and nighttime discharge. He was treated with a pressure equalization tube followed by an endolymphatic sac surgery and shunt placement. Three years later he underwent a labyrinthectomy with initial resolution of his symptoms, but his vertigo later returned. Two years later, a head injury worsened his vertigo. He suffered a spinal cerebrospinal fluid (CSF) leak that was patched using a blood patch but severe headaches and dizziness remained. The dizziness is described as vertigo, which comes in episodes. CT of his left temporal bone is to the right.Figure 1: Axial (horizontal) CT left temporal bone showing the intact posterior semicircular canal ampulla. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Figure 2: CT of the left temporal bone showing the vestibule is mostly unopened from the labyrinthectomy. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Figure 3: Axial (horizontal) CT of brain showing defect of the endolymphatic sac surgery. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Figure 4: Coronal (parallel to the face) CT of the left temporal bone showing the vestibule is mostly untouched. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Figure 5: Coronal (parallel to the face) T2-weighted MRI showing intact fluid compartment of the cochlea indicating the patient is a cochlear implant candidate if he desires. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Figure 6: Axial (horizontal) T2-weighted image showing T2 white matter changes which are a sign of chronic migraine. Clinical Consultation, vertigo, Labyrinthectomy, migraine-related Meniere's syndrome.Diagnosis: Migraine-Related Meniere’s Syndrome This patient’s history is interesting in that he had a labyrinthectomy for Meniere’s disease, which resulted in the temporary suspension of his vertigo. His vertigo then returned a few years later without contralateral symptoms of fullness, hearing loss, or tinnitus. The vertigo became worse after a head trauma, which was severe enough to cause a CSF leak in the spine. When evaluating a patient with vertigo, the question in the mind of many clinicians is whether the vertigo is peripheral or central. We have found that many forms of what used to be thought of as purely peripheral disorders (i.e., Meniere’s disease and benign positional vertigo) are likely dependent on or caused by central mechanisms. Recurrent benign positional vertigo has been found linked to migraine. 1 Additionally, Meniere’s disease has been strongly associated with a history of migraine, and 92% of patients with Meniere’s disease have been found to respond to migraine therapy. 2–5 Even Prosper Meniere mentioned in his original papers that his patients all suffered from migraine. 6 While in the past, Meniere’s disease was thought to be autoimmune 7 or purely peripheral in etiology 8, current evidence points to migraine as the underlying etiology, which manifests with peripheral symptoms via change in the blood flow within the cochlea and vestibule as well as fluid extravasation in the tissues of the inner ear caused by trigeminal nerve activation, the nerve intimately involved with the symptoms of migraine. 3 Currently, criteria for the clinical diagnosis of Meniere’s disease as outlined by the AAO-HNS include: two or more spontaneous vertigo attacks for 20 minutes to 12 hours; fluctuating low- to mid-frequency sensorineural hearing loss of affected ear that is audiometrically documented; and fluctuating aural symptoms including hearing loss, tinnitus, or fullness. Endolymphatic hydrops, which is thought to be the histopathologic feature of Meniere’s disease, is found in asymptomatic ears as well. As Meniere’s disease is episodic with long periods of remission, the syndrome can be difficult to diagnose and conclusively treat. Lifestyle modifications are often first-line with restriction of salt, caffeine, and alcohol. Medical management often consists of diuretics and betahistine with intratympanic steroid injections. Surgical intervention has been proposed since the 1890s with preservation of hearing as a major concern. Currently, at our Center, we treat patients with Meniere’s disease with the migraine dietary and lifestyle changes. Surgical decompression of the endolymphatic sac was first described in 1926 by Georges Portmann. Endolymphatic sac surgery has a questionable efficacy rate and was found to be equivalent to a sham operation in a double-blind controlled study finding no significant difference between mastoidectomy and endolymphatic shunt. 9 The most destructive surgical intervention that is used for Meniere’s disease is the labyrinthectomy. Patients with intractable symptoms from Meniere’s disease and those that have no residual hearing may potentially benefit from this procedure if all other treatments have been exhausted. Labyrinthectomy is done with either the transcanal or transmastoid approach with similarly high success rate in relieving severe attacks of vertigo. Simultaneous cochlear implant is often performed due to the consequent total hearing loss. There may be impaired balance or disequilibrium postoperatively if there is not adequate central vestibular compensation or the other vestibule is not normal. In this patient, the CT scans of the left temporal bone showed an intact posterior semicircular canal ampulla, as well as an unopened vestibule (Figures 1, 2, 3, 4). These findings suggest that the patient underwent a partial labyrinthectomy which can explain the persistent dizziness. On the other hand, his T2-weighted MRI showed a cochlea filled with fluid indicating that the patient is a cochlear implant candidate if he desires (Figure 5). In the case of this patient, manifestations of Meniere’s disease recurred following a traumatic brain injury despite partial remission status post labyrinthectomy. The persistence of vertigo after labyrinthectomy points to the central etiology with the persistence of some vestibular function remaining after a partial labyrinthectomy. Severe headache was the most functionally impairing in combination with aural fullness and tinnitus. Post-traumatic exacerbation of the symptoms is due to the post-traumatic activation of migraine. The CSF leak caused a significant decrease in intracranial pressure which leads to significant irritation of the dura and thus trigeminal nerve activation. Activation of the trigeminal nerve leads to the symptomatology of migraine, which included headaches, vertigo, aural fullness, and exacerbation of tinnitus. Persistent post-traumatic headache (PTHA) is a secondary headache that lasts longer than three months by definition. PTHA can be diagnosed clinically, with CT and MRI used to rule out hemorrhage, ischemic infarction, and masses. Persistent PTHAs are associated with migraine and respond well to migraine treatment. The suggested pathophysiology of persistent PTHA including trigeminal sensory system activation is shared with migraine headaches. Migraine treatment includes maintenance of a regular sleep schedule, eating at regular intervals throughout the day, staying hydrated with 2 liters of water daily, following the migraine diet, and minimizing stress. Evaluation for obstructive sleep apnea needs to be performed in patients with the body habitus or examination or history consistent with apnea. The migraine diet consists of avoidance of fermented products, pickled or preserved fruits, and vegetables, yeast products, nuts, eggs, alcohol, and fruit high in histamine. The Meniere’s diet (low caffeine, alcohol, and salt) is slightly different from the migraine diet. The migraine diet involves elimination of caffeine, histamine (found in nuts and citrus fruit), tyramine (found in fermented products like alcohol, cheese, or processed or aged proteins), and glutamate (found in ready to eat packaged foods, e.g., chips, salad dressings, frozen/canned foods). The elimination of salt inadvertently reduces glutamate containing foods, which tend to have high sodium content. Alcohol restriction inadvertently reduces tyramine intake as beer and wine are high in tyramine. In our practice at University of California Irvine, we do not restrict sodium in patients with Meniere’s disease but ask patients to drink more than 2 liters of water per day if they eat more salt. In addition, we do not restrict highly distilled alcohol (e.g., vodka) as it does not contain tyramine. Patients drinking highly distilled alcohol are asked to drink 140 mL of water for every ounce of highly distilled alcohol (e.g., vodka) as alcohol causes a loss of water through its diuretic effect. We do not recommend lipoflavinoids but rather supplement the patients with magnesium and riboflavin (vitamin B2), which can help prevent migraine. Nortriptyline, verapamil, or topiramate can be trialed for pharmacologic migraine prophylaxis as well if initial treatment is not effective. In this patient, a retrospective review of his axial T2-weighted image showed T2 white matter changes, which are a sign of chronic migraine (Figure 6). BONUS ONLINE VIDEOS: VISUAL DIAGNOSIS Read this month’s Clinical Consultation case, then watch the accompanying videos from Hamid R. Djalilian, MD, to review the patient’s imaging for yourself. Video 1. Axial (horizontal) CT of the left temporal bone showing the intact posterior semicircular canal ampullated end. Video 2. Coronal (parallel to the face) CT of the left temporal bone demonstrating that the vestibule was not opened completely during surgery. Video 3. Sagittal CT of the left temporal bone showing the anatomy in the sagittal plane. Video 4. Axial (horizontal) T2 MRI showing T2 white matter changes in the brain indicating long-term migraine. Video 5. Coronal (parallel to the face) T2 MRI showing intact fluid in the cochlea indicating the patient is a cochlear implant candidate. Video 6. Axial (horizontal) CT showing defect of endolymphatic sac surgery prior to the labyrinthectomy. Watch the patient videos online at thehearingjournal.com.