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Ruck Sigmoid sinus x-rayPenn Otorhinolaryngology – Head and Neck Surgery has developed a program under the direction of otologist Jason Brant, MD, and endovascular neurosurgeon Omar Choudhri, MD devoted to determining the source of and optimal treatment plan for a rare, highly individualized condition, pulsatile tinnitus.

The condition known clinically as tinnitus (and as ringing in the ears elsewhere) is defined as the auditory perception of sound in the absence of an external source. While all illnesses are personal, tinnitus has a unique immediacy in that the sound can only be perceived by its possessor.

About 10% of individuals with tinnitus have a subtype known as pulsatile tinnitus. Unlike standard tinnitus, which is generally benign and does not have specific treatment, pulsatile tinnitus often has an identifiable source, may signal a more serious underlying disorder, and may be curable if correctly diagnosed. Pulsatile tinnitus is not associated with the classic ringing or whirring sounds typical of standard tinnitus, but with one far more intimate—the heartbeat.

At Penn Otorhinolaryngology – Head and Neck Surgery, otologist Jason Brant, MD, and vascular neurosurgeon Omar Choudhri, MD, have gathered a team of like-minded specialists to confront the unique needs of pulsatile tinnitus patients – starting with identifying its cause.

The importance of evaluation: the potentially serious causes of pulsatile tinnitus

The cause of pulsatile tinnitus can be venous, arterial, arteriovenous, a result of tumors in the bone surrounding the ear or bone missing from certain portions of the ear, from elevation of the pressure surrounding the brain, or even transmitted from pathology of the heart or in the neck. For about a third of patients, the source of the condition can be unknown but it is important to rule out the serious possible causes. As a consequence of the condition’s rarity and intimate manifestations, pulsatile tinnitus is often under-diagnosed, an error that has the potential for untoward consequences in affected persons. A thorough evaluation is necessary to determine the best course of treatment, and to identify potentially more serious underlying issues that could be the source.

Giant carotid aneurysmPulsatile tinnitus can be the signal for some rather severe conditions, including vascular malformations, paraganglioma, aneurisms, bony pathologies and other tumors,” Dr. Brant says, noting that while these anomalies are uncommon, they must be ruled out to preclude the risk of bleeding or tumor progression.

This concern around the rare, but potentially lethal ramifications of a missed diagnosis is the crux of pulsatile tinnitus diagnosis.

“There’s little consensus at the moment on which tests are suitable for which populations,” Dr. Brant says. “Which complicates things somewhat, because we need to both ensure we’re not missing anything and that we’re not over-testing.”

To address these complexities, Drs. Brant and Choudhri have developed a comprehensive program to streamline the evaluation and potential treatment of pulsatile tinnitus. A third component will initiate research processes to identify the types of care best suited to each of the condition’s venous and arterial etiologies.

Penn’s plan for pulsatile tinnitus care

As always, the process of resolving disease begins with diagnosis. Occasionally, according to Dr. Brant, the source of pulsatile tinnitus is obvious.

“From the ENT side, we’re always aware of things like superior canal dehiscence and sigmoid sinus dehiscence—basically missing portions of bone in the structure of the ear that can cause pulsatile tinnitus—and can see if there’s a tumor in the middle ear.”

Pulsatile tinnitus imagingHowever, if early investigations yield nothing conclusive, a more insidious etiology may be present. Determining the source of pulsatile tinnitus in these cases may involve evaluation for elevation in pressure around the brain or creating a rendering of blood flow turbulence within vascular structures—an effort greatly enhanced by recent developments in MRI technology.

Termed 4D Time-of-Flight (TOF) MRI, the modality can provide comprehensive visualization of cardiovascular hemodynamics and intravascular blood flow patterns through the body.

“We have been able for some time to image the interior caliber and tortuosity of veins and arteries with catheter-based, CT and MR angiography,” says Dr. Choudhri. “4D-flow MRI provides a more versatile and comprehensive depiction of flow fields.”

If a vascular or osteological source of pulsatile tinnitus is identified (e.g. tumors, malformations, etc.), otological or neurological surgery is most often needed to correct, according to Dr. Brant. This is occasionally combined with adjunct therapy (i.e., stenting for aneurysms). Other potential causes like elevated intracranial pressure, hyperthyroidism, anemia and other systemic contributors can be treated medically.

Very few medical centers treat pulsatile tinnitus, and fewer have devoted programs to the condition with the expertise and resources needed for optimal evaluation and treatment.

“Having a specific focus on pulsatile tinnitus and coming up with better techniques and algorithms for who would benefit from surgery and who wouldn’t is what sets Penn ENT apart,” says Dr. Brant.

Dr Choudhri concurs. “Pulsatile tinnitus is a condition of decisions—which imaging modality to choose for which underlying pathology,” he says. “But one must first understand that pathology—the bony dehiscences and vascular anomalies—and their subtle effects in the vasculature to address the condition. This can’t be achieved casually.”

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