47-year-old female who had the onset of persistent vertigo. The first evaluations revealed right beating nystagmus on gaze straight and to the right, unsteady gait with limb ataxia, and positive head thrust to the left. Improvement seemed to be occurring in that gait deviation with eyes closed was no longer present. Vestibular physical therapy exercises were started. On the next evaluations there was paroxysmal positional vertigo from the left ear and minimal neurosensory hearing loss. An audiogram showed minor neurosensory deafness. A follow-up visit showed "crackling with passive movement of the head."
1) Do you confirm the diagnosis?
2) What is the best treatment?
47 years old, female , Italy
Development of symptoms of persistent vertigo since September 2008, for which the patient was evaluated on several occasions at the ENT department of the Pordenone hospital, for example:
- on September 2008: “otoscope examination negative, clinical signs of left vestibular deficit (II-III° nystagmus to the right, tonic deviation of the limbs to the left, Romberg's negative and Hallpike negative overall)" with instructions to take Plasil, Vertiserc and rest for several days;
- on September 2008: “evidence of rapid III° nystagmus to the right, unsteady gait, no clear tonic deviations. Finger-nose test and heel-knee test: negative. Mingazzini I: negative. No signs of cranial nerve deficits. Diagnosis of peripheral Vertigo” with instructions to rest with the use of levosulpiride 25 mg x 3 in consideration of the ineffectiveness of the other drugs taken.
- On September 2008: ENT re-evaluation: “ II° - III° spontaneous nystagmus to the right persists, Head thrust test positive to the left, gait still unsteady with deviation to the right. Suggestion: CT scan of the brain.” CT scan of the brain: within normal limits in relation to age, ventricular system and subarachnoid cavity. No particular supra and subtentorial sensitometric alterations are determined. Midline structures aligned.
- On September 2008: “left peripheral vestibular deficit persists, with initial signs of compensation (no more gait deviations with eyes closed, II° spontaneous nystagmus to the right and positive head thrust test to the left persist). Vestibulo-ocular reflex rehabilitation exercises are assigned (rotation of the head with visual fixation for 1? – 2? Several times a day)”. On October 2008: “at the current check-up PPV (Paroxysmal Positional Vertigo) of the left PSC (posterior semicircular canal). The Epley maneuver is performed to the right.”
- On November 2009, specialist visit “Findings are negative. The audiometric exam shows a minimal neurosensory component for acute tones to the left and the head shaking test is clearly positive for nystagmus to the right. This finding is compatible with left peripheral vestibular deficit, probably ascribable (based on clinical history) to viral labyrinthitis. The persistence of symptoms can be attributed to the fact that the vestibular deficit is unstable and therefore establishment of compensation is delayed. Today I do not detect paroxysmal positional vertigo, for which reason I am not performing any maneuvers. I advise performing auditory brainstem response (ABR) and MRI scan of the brain with contrast medium. I suggest resuming treatment with microsr 24 mg 1 tablet 2 times a day for 30 days. I also advise paying attention to posture in the workplace and ruling out any accompanying head and neck disturbance components.”
- ABR of December 2008: “bilateral traces negative for retrocochlear pathological conditions.”
- Audiometric exam dated October 2008: “ minor neurosensory deafness more noticeable on the left side at around 1000 Hz, pantonal.”
- MRI of the brain and brainstem with and without contrast medium of December 2008: “CSF spaces of normal width. No signs of masses. Median structures in axis. No supra and subtentorial parenchymal sign alterations or pathological permeation foci after contrast. Regular appearance of the acoustic nerves; no expansive formations are seen in their proximity."
- Follow-up visit on January 2009: “left posterior vestibular deficit persists. Cracking with passive movement of the head. I advise cervical spine x-ray and rehabilitation physician visit to exclude possible muscle tension component. Vestibular adaptation exercises are given.”
I agree that the diagnosis is an acute process affecting the left inner ear (left peripheral vestibular deficit). Most probably this was a viral infection of the left inner ear. With involvement of both hearing and vestibular dysfunction I agree with a diagnosis of a viral labyrinthitis. The findings on examinations of right beating spontaneous and gaze nystagmus, positive head thrust to the left and right beating post head shaking nystagmus are all consistent with damage to the left inner ear. The finding of paroxysmal positional vertigo from the affected ear (left) is sometimes seen subsequent to damage to an inner ear from a viral infection. However, it is clear that there is more widespread damage to the left inner ear than can be explained by paroxysmal positional vertigo. There is nothing on the examinations, imaging studies (CT and MRI) or Auditory Brainstem Response testing to suggest a problem in the brain, to the blood supply to the brain or affecting the 8th nerve behind the ear.
2. The best treatment depends upon the symptoms that are persisting. If the patient has unsteadiness of gait and unsteady dizziness that is present much of the time, the best treatment is vestibular physical therapy, which consists of compensation, adaptation and balance training exercises and is best done by a trained physical therapist experienced in treating patients with this type of disorder. Vestibular suppressant medications at this stage of the disease tend to delay the development of central nervous system adaptation for a damaged vestibular system. If the patient is having recurrent episodes of severe spinning dizziness, treatment with low salt diet and diuretics such as hydrochlorthiazide or acetazolamide is sometimes helpful in preventing symptoms. Sublingual lorazepam or other benzodiazepine is often helpful in decreasing the severity of a severe spell of spinning dizziness once it has started. The sublingual route is preferred because medication is rapidly absorbed and the problem of vomiting or gastric atony is avoided. Medication by this route can cause drowsiness. We do not think that vasodilators are helpful for this type of problem although vasodilators such as calcium channel blockers are often used in Europe. There is no good evidence that antiviral medications are helpful.
3. Prognosis- Although some of the improvement in symptoms is from healing, much of the improvement especially weeks to months after the insult is from the brain learning to adapt to a damaged/changed inner ear vestibular system. This type of adaptation occurs at variable rates in different patients and may be incomplete. Thus some patients recover in weeks to months and others take months to years. In some patients there is never complete central nervous system adaptation and therefore some residual symptoms of unsteadiness. However, as months go by almost all patients show considerable improvement. If the damaged inner ear is unstable and changes function there can be recurrent episodes of spinning dizziness. This most commonly occurs in the first few months after the initial episodes. A small percentage of patients go on to develop a Meniere's type picture of recurrent episodes of recurrent severe spells of vertigo plus or minus fluctuating hearing. This usually resolves with time, but the length of time is variable and uncertain.