A 34-year-old woman with a history of recurrent acute otitis media reported having hearing difficulties when outdoors and hearing a pulsing sound when exercising. She reported taking antibiotics over the past few years but saw no improvement. Most recently, the patient was told that the infection will not go away and that she needed to see an otolaryngologist. The patient had no history of Eustachian tube dysfunction nor childhood ear infection. Her ear examination is shown in Figure 1.
Diagnosis: Glomus Jugulare
Acute otitis media is uncommon among adults, especially among those without any previous Eustachian tube dysfunction. When an adult patient with no history of Eustachian tube dysfunction presents with this condition, the clinician must consider other possibilities as well. In primary care settings, acute otitis media is often erroneously diagnosed when the physician looks at the patient's external auditory canal wall and thinks that the tympanic membrane is in view. The skin in the canal with its capillaries may appear like a red turbid tympanic membrane.
One condition to be considered in an adult with no Eustachian tube dysfunction history reporting acute otitis media is Wegener's granulomatosis (AKA granulomatosis with polyangiitis). Patients with this condition can present with ear pain and symptoms like those of acute otitis media. However, they generally do not have a history of Eustachian tube dysfunction. Some patients with Wegener's granulomatosis exhibit serous otitis on presentation. Other abnormalities these patients tend to have include chronic sinusitis, progressive sensorineural hearing loss, sudden hearing loss that could involve both sides, lung and/or kidney disease, and vision loss, among others. An anti-neutrophil cytoplasmic antibody (ANCA) panel can help confirm this diagnosis.
Another uncommon condition that can mimic acute otitis media in an adult with no history of Eustachian tube dysfunction is the presence of a middle ear mass. A vascular mass in the middle ear may appear like a red tympanic membrane and present with conductive hearing loss. The differential diagnosis of a red middle ear mass includes glomus tympanicum, middle ear adenoma, and glomus jugulare extending into the middle ear, among others. Biopsy of a middle ear mass protruding through the tympanic membrane is not advisable; imaging should always be obtained before this biopsy. In rare cases, a mass protruding through the tympanic membrane could be the carotid artery, which can appear white. In addition, the jugular bulb, which tends to appear blue, can be high, dehiscent, and in contact with the tympanic membrane. Facial schwannomas when biopsied could cause facial paralysis. Finally, glomus tumors are highly vascular in nature, and controlling bleeding from a glomus tumor biopsy would be difficult.
The imaging to be obtained includes a CT scan of the temporal bone and possibly an MRI of the internal auditory canals with and without contrast. CT produces higher-resolution images of the bony structures of the middle ear. While a CT scan can be done with and without contrast, it does require exposing the patient to radiation twice. To obtain contrast imaging, our preference is to use the MRI to avoid extra radiation exposure.
The patient's imaging was consistent with that of a glomus jugulare tumor that extends into the middle ear (Figs. 2-5). A glomus tumor that arises from the glomus bodies of the middle ear, which lie in the cochlea's promontory, is called glomus tympanicum. A glomus tumor can also arise from the dome of the jugular bulb, which is within a few millimeters of the middle ear. This is called glomus jugulare.
Glomus jugulare tumors can expand silently and break through the floor of the middle ear, becoming visible behind the tympanic membrane. They can fill the middle ear and be indistinguishable from a glomus tympanicum on examination. During pneumatic otoscopy, air pressure on the tympanic membrane can cause blanching of the tumor, which is called Brown's sign. This patient was diagnosed with a glomus jugulare tumor based on the imaging results.
Glomus jugulare tumors are generally diagnosed via CT and MRI scans. On CT of temporal bones, the tumor is seen infiltrating the bone surrounding the jugular bulb (the top-most portion of the jugular vein). Due to their highly vascular nature, these tumors take up contrast intensely on MRI and appear white on post-contrast images. Glomus tumors are generally benign; aggressive/malignant forms do occur but are rare.
Treatment of these tumors has evolved over the past decade. Surgical resection is generally done, although surgery for total tumor resection has an increased likelihood of causing injury to the lower cranial nerves (IX to XI) due to their proximity to the jugular bulb, which can affect swallowing and shoulder motion as well as the patient's voice.
While nearly all these tumors were surgically resected in the past, there has been a shift in management strategy away from surgery and toward stereotactic radiation, which is done in one to five sessions. This treatment modality has been found to be highly effective, with a pooled control rate of 95 percent (Otol Neurotol. 2018 Apr;39:488-493). Stereotactic radiosurgery aims to stop further growth of the tumor, but it does not cause the tumor to disappear. In some cases, depending on the extent and location of the tumor, patients can be treated with a combination of surgery and radiosurgery.
When tumors extend to the middle ear, radiating the tumor presents a challenge. Radiation to the cochlea can cause sensorineural hearing loss and be disadvantageous. With middle ear or ear canal extension, the middle ear component of the tumor is often resected surgically, and the jugular foramen portion of the tumor is radiated. If the tumor is significantly extended into the intracranial cavity, the clinician may need to resect the intracranial portion of the tumor as well. The portion of the tumor around the jugular bulb is then radiated, allowing for a higher degree of cranial nerve preservation.
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 temporal bones showing infiltration of the tumor around the jugular bulb.
- Video 2. Coronal (parallel to face) CT of the temporal bones showing the area where the tumor extends from the jugular bulb into the middle ear.
- Video 3. Sagittal (vertical, parallel to ear) CT of the temporal bones showing the extension of the tumor below the internal auditory canal.
- Video 4. Axial (horizontal) T1 post-contrast MRI of the internal auditory canals showing the extent of the tumor.
- ideo 5. Coronal (parallel to face) T1 post-contrast MRI of the internal auditory canals showing the tumor in the coronal plane.
- Video 6. Sagittal (vertical, parallel to ear) T1 post-contrast MRI of the internal auditory canals demonstrating the anatomy and tumor involvement in the sagittal plane.
Watch the patient videos online at thehearingjournal.com.