Title: Tinnitus With an Underlying Jugulotympanic Paraganglioma
Authors: Amira Shaikh, OMS3; Jacob Templin, DO
Introduction
This case study focuses on a common presentation of a rare disease: Jugulotympanic paraganglioma. Paragangliomas are rare tumors that commonly present with tinnitus and hearing deficits. These tumors pose a significant challenge in determining the correct treatment strategies due to their location. Discovering these tumors in the early stages can help optimize patient outcomes and treatment options.
Case Description
A 53-year-old female with a past medical history of TMJ and otitis media presented with pulsatile tinnitus and perceived hearing deficits on the left side. Additional symptoms included left sided pre and post auricular pain radiating down the left neck and into the shoulder, as well as occasional headaches. Physical exam revealed an erythematous left tympanic membrane without bulging or masses. Audiometry suggested mild sensorineural hearing loss on the right side without significant findings on the left. Otomicroscopy exam showed a red hue in the middle ear near the promontory. CTA Head and Neck revealed findings consistent with a jugulotympanic paraganglioma. Patient opted for surgical therapy, resulting in resolution of tinnitus. Pathology was consistent with left jugular foramen paraganglioma.
Discussion
Jugulotympanic paragangliomas often present with tinnitus, a common chief complaint from patients. Persistence of painful and pulsatile tinnitus prompted further investigation despite the patient’s past medical history of ear infections and TMJ which commonly cause tinnitus. CTA revealed this rare tumor, which is challenging to treat. Mutations in Succinate Dehydrogenase Subunit B and the amount of tumor invasion can be significant determining factors on if it is more beneficial to use the “wait and scan” method with the risk of detrimental mass effect from the tumor, or to proceed and remove the tumor with the risk of causing damage to surrounding structures resulting in consequences such as cranial nerve deficits.
Thank you for this interesting case report, Student Dr. Shaikh. I have been assigned as one of the judges for your project. How long had the patient been struggling with her symptoms? Did she have a history of otitis media in childhood, and is that a risk factor for this condition? Thanks again – very interesting case!
Dr. Briggs Early, thank you so much for your interest and your comment! The patient had been struggling with symptoms of pulsatile tinnitus and otalgia intermittently for about 18 months. Her symptoms would occur about once every 1-2 weeks. The patient had been treated for otitis media a few months prior to the onset of her symptoms and she attributed her symptoms to post- otitis media residual effects. She did not pursue further evaluation of her symptoms until they worsened over time, with the addition of headaches and perceived hearing deficits. Our patient did not have a history of childhood otitis media. Analysis of the current literature on this condition does not suggest that childhood otitis media is a risk factor for development of this tumor. However, studies do show that at least 30% of paragangliomas are inheritable. The most common gene mutations associated with paragangliomas are Succinate Dehydrogenase B (SDHB), Von Hippel-Lindau syndrome (VHL), Phenolic glycolipid – 1 (PGL-1), Phenolic glycolipid – 2 (PGL-2), Phenolic glycolipid – 3 (PGL-3), Phenolic glycolipid – 4 (PGL-4), and Transmembrane Protein 127 (TMEM127), with SDHB being most strongly associated with increased malignancy. Therefore, it is important to make sure that all patients diagnosed with paragangliomas have genetic screening, as these gene mutations have shown to be the greatest known risk factor for development of these tumors thus far. I hope this addresses your questions well. Thank you!
Yes, thank you – I appreciate your thorough response to my questions!
Judge: Could you hypothesize about the possible relationship and underlying causes between the rash and the paraganglioma?
Dr. Novack, thank you so much for your question and interest. Many paragangliomas are associated with Succinate Dehydrogenase B (SDHB) mutations. When a mutation in this gene is present, this is associated with a complete loss of mitochondrial complex II activity, thus creating pseudo-hypoxic conditions and prompting stimulation of angiogenesis. The rash our patient described resembled a hemagioma-like cutaneous lesion. Hemangiomas are a benign proliferation of blood vessels either on or just deep to the skin. Therefore, my hypothesis is that patients with a SDHB mutation places them in a state where they are more susceptible to vascular proliferation, which could thus explain the relationship between the hypervascular skin rash described by our patient and the proliferation of the jugulotympanic paraganglioma, a hypervascular tumor. I hope this addresses your question well. Thank you again!
Thanks for your presentation. In the background information, you note that CN XII is sometimes involved with jugulotympanic paragangliomas. Where does this involvement typically occur?
Dr. Walser, thank you so much for your question and your interest. The involvement of Cranial Nerve XII usually occurs during later stages of tumor development. Because this tumor typically originates in the Jugular Foramen, the nerves that are commonly impacted initially are Cranial Nerves IX, X, and XI. As time progresses and the tumor expands, encroachment of Cranial Nerve XII can occur at the location where it exits the Hypoglossal Canal. This will then leave the patient susceptible to paralysis or weakness of the tongue. I hope this addresses your question well. Thank you again!