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1
Question:

A 33-year-old woman comes to the office due to intermittent dizziness.  The patient describes a sensation of severe spinning accompanied by intense nausea that lasts 1-2 hours.  She feels unsteady during these episodes and lies down with her eyes closed for relief.  The patient has had several similar episodes during the past 2 years but has not noted any factors that precipitate the symptoms.  She also hears a "mechanical humming" sound in her right ear during these episodes.  The patient has no associated headaches, ear pain, or ear discharge.  Temperature is 36.6 C (98 F), blood pressure is 130/84 mm Hg, and pulse is 86/min.  On examination, air conduction is greater than bone conduction in both ears.  When the base of a tuning fork is placed against her forehead, the sound is heard more prominently in the left ear.  Which of the following is the most likely cause of this patient's condition?

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Explanation:

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This patient with episodic vertigo, tinnitus, and sensorineural hearing loss (based on Weber and Rinne tuning fork tests) has Ménière disease, which is characterized by the following triad:

  • Episodic vertigo (20 min to 24 hr), commonly associated with nausea and vomiting

  • Sensorineural hearing loss, fluctuating and varying in severity and usually worsening over time (primarily affecting low frequencies at first and progressing to permanent loss over all frequencies)

  • Low-frequency tinnitus in the affected ear, often accompanied by a feeling of aural fullness

Ménière disease is a disorder of the inner ear characterized by increased volume and/or pressure of endolymph (endolymphatic hydrops) that is thought to be due to defective resorption of endolymph.  The resulting distension of the endolymphatic system causes damage to both the vestibular and auditory components of the inner ear.

The diagnosis of Ménière disease is based primarily on clinical findings; however, audiometry should be performed to fully characterize the hearing loss and follow its course over time, and MRI should be obtained to rule out central nervous system lesions.

(Choice A)  Vestibular schwannomas can cause unilateral sensorineural hearing loss, sometimes with imbalance and tinnitus.  However, symptoms are usually persistent and progressive rather than episodic, and true vertigo is not typical because the slow growth often allows for the development of compensation.

(Choice B)  Multiple sclerosis is characterized by patchy demyelinating lesions in the central nervous system that are disseminated in space and time.  Although this patient's symptoms are disseminated in time (ie, episodic), they are not disseminated in space because all symptoms can be explained by a single lesion impacting the inner ear.  The classic triad of episodic vertigo, hearing loss, and tinnitus is much more consistent with Ménière disease.

(Choice D)  Labyrinthitis is inflammation of the labyrinth and vestibular nerve that causes acute-onset hearing loss and vertigo, often accompanied by nausea and vomiting (if no hearing loss is present, it is called vestibular neuritis).  It usually occurs in a single episode (vs recurrent in this patient) following a viral syndrome; symptoms typically last a few days (vs hours in this patient).

(Choice E)  Benign paroxysmal positional vertigo is thought to be caused by calcium debris within the semicircular canals that alter normal fluid flow.  It causes brief (<1 min), often intense episodes of vertigo triggered by changes in head position.  Patients can have headaches or a feeling of ear fullness, but tinnitus and hearing loss are not typical.

Educational objective:
Ménière disease is a disorder of the inner ear characterized by increased volume and/or pressure of endolymph (endolymphatic hydrops).  Clinical features include episodic vertigo, sensorineural hearing loss, and tinnitus or aural fullness.