Objectives: To illustrate the diagnostic value of progressive unilateral hearing loss, pulsatile tinnitus, and a vascular retrotympanic mass in recognizing an advanced jugular paraganglioma after an initial diagnosis of chronic Eustachian tube dysfunction.
Methods: We retrospectively describe the clinical, audiological, and computed tomographic findings of a 53-year-old woman evaluated at Windhoek Central Hospital in 2010 and interpret the historical case in the context of contemporary temporal-bone paraganglioma literature.
Results: The patient had approximately 3 years of progressive left hearing loss and intermittent ipsilateral pulsatile tinnitus. Otoscopy demonstrated a reddish vascular retrotympanic mass. Audiometry showed left mixed hearing loss, and tympanometry showed a type B tracing. Contrast-enhanced CT/CT angiography demonstrated a hypervascular mass centered at the left jugular bulb/foramen with irregular skull-base erosion, superior extension toward the hypotympanum and middle ear, jugulosigmoid involvement, close relationship to the carotid canal, deep skull-base extension, and probable intracranial involvement. Referral to a tertiary skull-base center was recommended.
Conclusions: A vascular retrotympanic mass associated with unilateral hearing loss and pulsatile tinnitus should prompt imaging for temporal-bone paraganglioma. Advanced tympanojugular lesions require careful definition of skull-base and neurovascular extension and multidisciplinary management.
This case is particularly noteworthy because it involves a female engineer who was initially essentially asymptomatic, presenting only with symptoms suggestive of Eustachian tube dysfunction. The underlying diagnosis was therefore initially missed. Several years later, while working in Africa, she was reevaluated for persistent and progressive otologic symptoms, leading to the identification of an advanced jugular paraganglioma. The case highlights how these generally slow-growing tumors may present with subtle and nonspecific otologic symptoms in their early stages, potentially delaying diagnosis until substantial skull-base extension has occurred.
Table of Contents
1. Abstract
2. Introduction
3. Case Report
4. Discussion
5. Conclusion
Objectives & Topics
The primary objective of this case report is to illustrate the critical diagnostic pitfalls associated with unilateral otologic symptoms and highlight the diagnostic value of progressive hearing loss, pulsatile tinnitus, and a vascular retrotympanic mass in identifying an advanced jugular paraganglioma that was initially misattributed to chronic Eustachian tube dysfunction.
- Clinical presentation of temporal bone and tympanojugular paragangliomas
- Diagnostic misattribution of progressive unilateral hearing impairment to Eustachian tube dysfunction
- Role of pure-tone audiometry and tympanometry in assessing middle-ear masses
- Cross-sectional imaging via computed tomography (CT) and CT angiography for skull-base erosion and vascular mapping
- Staging considerations using the Fisch classification system and contemporary multidisciplinary management
Excerpt from the Book
Case Report
A 53-year-old Cuban woman was evaluated at Windhoek Central Hospital, Namibia, in 2010 after approximately 3 years of progressive left-sided hearing impairment. She had previously been evaluated in Ciego de Ávila, Cuba, where her symptoms were attributed to chronic Eustachian tube dysfunction. During a professional assignment in the Kavango region of Namibia, the hearing loss worsened and intermittent ipsilateral pulsatile tinnitus developed. Otoscopy demonstrated a reddish, vascular-appearing retrotympanic mass protruding toward the tympanic membrane. Pure-tone audiometry showed essentially preserved right-ear thresholds and mixed hearing loss in the left ear, with an air-bone gap and greater deterioration of air-conduction thresholds at higher frequencies (Figure 1). Tympanometry showed a type B tracing on the affected side. In this setting, the type B tracing was interpreted as markedly reduced tympano-ossicular mobility associated with the middle-ear occupying lesion rather than as a specific sign of Eustachian tube dysfunction.
Contrast-enhanced CT and CT angiographic evaluation demonstrated a hypervascular soft-tissue mass centered in the left jugular bulb and jugular foramen with irregular erosion of the adjacent skull base. Superior extension toward the hypotympanum and middle-ear cavity was evident. The lesion involved the ipsilateral jugulosigmoid region and had a close anatomical relationship to the carotid canal. Deep skull-base extension was also present, and the available historical images raised concern for an intracranial component (Figures 2 and 3).
The original radiology report described a “left glomus jugulare, stage IV”; however, the staging system was not specified. Because only historical photographs of the CT study are available rather than the original DICOM dataset, a precise retrospective Fisch subclassification cannot be assigned. The most defensible contemporary description is an advanced left jugular/tympanojugular paraganglioma with middle-ear and skull-base extension and probable intracranial involvement.
The case was reviewed at a departmental staff meeting. Given the advanced skull-base extent and anticipated neurovascular complexity, tertiary skull-base evaluation was recommended. The patient was subsequently referred to Cuba for further evaluation and definitive management.
Summary of Chapters
1. Abstract: Summarizes the clinical presentation, audiologic findings, computed tomographic evaluation, and multidisciplinary referral of a 53-year-old patient diagnosed with an advanced jugular paraganglioma.
2. Introduction: Provides an overview of temporal bone paragangliomas, detailing their neuroendocrine origin, anatomical pathways of spread, hallmark clinical symptoms, and primary imaging modalities.
3. Case Report: Describes the chronological clinical course, otoscopic evaluation, audiological testing, and CT angiographic findings demonstrating extensive skull-base and middle-ear involvement.
4. Discussion: Contextualizes the case within current literature, examining diagnostic differentiation, radiological staging via the Fisch system, modern multimodal management options, and genetic screening guidelines.
5. Conclusion: Synthesizes the core diagnostic takeaways, emphasizing the clinical triad of unilateral hearing loss, pulsatile tinnitus, and a retrotympanic mass to prevent delayed referral.
Keywords
Paraganglioma, Glomus Jugulare, Tympanojugular Paraganglioma, Eustachian Tube Dysfunction, Pulsatile Tinnitus, Mixed Hearing Loss, Skull Base, Jugular Foramen, Computed Tomography, Angiography, Retrotympanic Mass, Fisch Classification, Temporal Bone
Frequently Asked Questions
What is the central focus of this case report?
The case report focuses on documenting the clinical evaluation and delayed diagnosis of an advanced jugular paraganglioma that was mistakenly treated as chronic Eustachian tube dysfunction for several years.
What are the primary thematic areas covered in the report?
The main themes include neuro-otologic symptom recognition, audiometric and tympanometric interpretation, high-resolution cross-sectional radiological assessment, and skull-base surgical referral planning.
What is the primary objective of the publication?
The objective is to highlight the diagnostic significance of progressive unilateral hearing loss, pulsatile tinnitus, and a vascular retrotympanic mass to avoid diagnostic delay and inappropriate interventions.
Which scientific methodology was utilized?
The author employed a retrospective single-case study methodology, reviewing clinical records, audiological evaluations, and radiographic findings from 2010 while correlating them with modern scientific literature.
What does the main body of the case report describe?
The main section details the patient's three-year symptomatic progression, otoscopic findings of a reddish vascular mass, type B tympanometry, pure-tone audiometry results, and contrast-enhanced CT scans demonstrating bone erosion and extensive skull-base involvement.
Which keywords best characterize this scientific report?
Key terms characterizing the publication include Paraganglioma, Glomus Jugulare, Pulsatile Tinnitus, Mixed Hearing Loss, Jugular Foramen, Temporal Bone, and Retrotympanic Mass.
Why was the patient's condition initially mistaken for Eustachian tube dysfunction?
The insidious onset of unilateral hearing impairment and a flat type B tympanogram were initially misinterpreted as Eustachian tube hypofunction and fluid effusion, obscuring the presence of a vascular mass restricting ossicular mobility.
Why is a biopsy strictly contraindicated when evaluating a vascular retrotympanic mass?
Biopsy of a suspected vascular middle-ear mass presents severe risks of catastrophic hemorrhage and complications from potential vascular anatomical variants, necessitating cross-sectional imaging prior to any tissue sampling.
What radiological features confirmed the advanced stage of this paraganglioma?
Contrast-enhanced CT and angiography revealed permeative erosion of the jugular foramen and adjacent skull base, extension into the hypotympanum and middle ear, jugulosigmoid involvement, proximity to the carotid canal, and deep skull-base extension with probable intracranial involvement.
- Quote paper
- Manuel Suárez (Author), 2026, Delayed Diagnosis of an Advanced Jugular Paraganglioma Mimicking Chronic Eustachian Tube Dysfunction. A Case Report, Munich, GRIN Verlag, https://www.grin.com/document/1768160
