PP1605 - Not All Cases of Dizziness and Tinnitus Are Ménière’s Disease: A Need for Physician Education

Menière's disease (MD), or idiopathic endolymphatic hydrops, results in a tetrad of symptoms; episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. Despite MD being a rare inner ear disorder with specific clinical criteria, it is frequently misdiagnosed/over-diagnosed by primary/ENT physicians. This is a case of a 27-year-old woman presenting with left-sided pulsatile tinnitus and short episodes of “imbalance” who was initially diagnosed with MD. Audiologic and MRI results that led to the correct diagnosis will be presented. The role of audiologists in evaluating patients with suspected MD and educating physicians about this condition will be discussed.

Summary:

In 1861, French physician, Prosper Ménière, identified a rare disorder that was named after him. Pathophysiology of idiopathic Menière's disease (MD) includes distension of the endolymphatic space and saccular ducts in the inner ear leading to endolymphatic hydrops, causing damage to hair cells and vestibular neuro-epithelium. Disease onset is typically between 40-60 years, affecting about 0.2 percent of the U.S. population. Majority of cases are at least initially unilateral, with women and men affected equally.

A definitive diagnosis of MD requires >2 spontaneous episodes of vertigo >20 minutes, documented sensorineural hearing loss, tinnitus, and aural fullness, with other causes excluded. In the early stages, episodes may occur in clusters, often with symptom-free periods of many years between. Despite these diagnostic guidelines, MD is frequently misdiagnosed/over-diagnosed by both primary and ENT physicians (Syed & Aldren, 2012). This case exemplifies the common problem of over-diagnosing MD, need for better physician education, and importance of correctly identifying etiology for optimum patient management.  

In November, 2021, a 27-year-old female reported her first episode of dizziness described as a “feeling of imbalance” that lasted about five minutes and resolved with rest; true vertigo was ruled out. She had a history of frequent childhood ear infections requiring (pressure equalization (PE) tubes. Current medical history was significant for anxiety and attention deficit hyperactivity disorder (ADHD) for which she was on medication. No genetic, personal, or family history of audio-vestibular problems was reported. She experienced a similar second imbalance episode in February, 2022. Since then, she reported constant pulsatile left-sided tinnitus but no further episodes of dizziness. She reported that the tinnitus mimicked her heartbeat, was exacerbated by physical activity, and improved but did not resolve when she lay flat.

In May 2022, the patient’s primary care provider (PCP) recommended a consult with an ENT physician. The PCP mentioned MD as a possible diagnosis despite a clinical presentation not consistent with this disorder. Menière's disease is rarer in young adults and inconsistent with short episodes of “imbalance,” or pulsatile tinnitus. This potential diagnosis resulted in significant anxiety/apprehension for the patient who already suffered from anxiety.

The patient consulted an ENT physician in June, 2022, when pure-tone air-conduction threshold assessment and speech audiometry was performed. Hearing sensitivity was within normal limits with normal tympanograms bilaterally. She was referred for an MRI with/without contrast. The MRI revealed “small loops of the bilateral anterior inferior cerebellar artery extended into the internal auditory canals” and “possible osseous thinning/dehiscence along the bilateral superior semicircular canals,” (superior semicircular canal dehiscence-SSCD).

As evident from the clinical presentation and imaging studies, this was not a case of MD. The role of audiologists in evaluating patients with suspected MD and educating physicians about this condition will be discussed. We also will address: (a) the anterior inferior cerebellar artery abnormality possibly causing the pulsatile tinnitus, (b) whether SSCD was an incidental finding or played a role in the presenting symptoms, and (c) management of this patient. Recent audiometric and imaging results will be presented. It is often bilateral but can be unilateral