---

BLEEDING FROM THE EAR (OTORRHAGIA) AND GLOMUS TYMPANICUS

---

PART I: BLEEDING FROM THE EAR (OTORRHAGIA)

---

CAUSES OF BLEEDING FROM EAR

---

A. TRAUMATIC CAUSES (Most common overall)

Longitudinal temporal bone fracture is the most common cause of traumatic otorrhagia. Blood exits via a TM tear or EAC laceration. CSF otorrhoea may accompany it if the dura is torn — glucose testing distinguishes blood-stained CSF (Scott-Brown, 8th ed., Vol. 3, p. 3147).

---

B. INFECTIVE CAUSES

---

C. NEOPLASTIC CAUSES (Clinically very important)

---

D. VASCULAR CAUSES

• Aberrant internal carotid artery in middle ear — pulsatile bluish-red mass, catastrophic haemorrhage if traumatised
• High-riding jugular bulb — bluish mass in hypotympanum, mistaken for effusion
• Arteriovenous malformation (AVM) of temporal bone
• Aneurysm of petrous carotid artery (Cummings, 7th ed., p. 2094)

---

E. BLOOD DYSCRASIAS AND SYSTEMIC CAUSES

• Haemophilia, von Willebrand disease
• Thrombocytopenic purpura (ITP)
• Anticoagulant therapy (Warfarin, NOACs)
• Leukaemia (gingival and mucosal bleeds including ear)
• Osler-Weber-Rendu syndrome (hereditary haemorrhagic telangiectasia)

---

F. INTRACRANIAL / MISCELLANEOUS

• Encephalocele into middle ear — CSF otorrhoea may be haemorrhagic
• After mastoid surgery — reactionary or secondary haemorrhage
• Glomus tumour post-biopsy — catastrophic bleed

---

CLINICAL APPROACH TO OTORRHAGIA — DIAGNOSTIC FLOWCHART

BLEEDING FROM EAR
        │
        ├── History: Trauma? Infection? Hearing loss? Pulsatile?
        │
        ├── Traumatic → Otoscopy → TM tear? → Audiogram + HRCT Temporal Bone
        │                                     → Exclude CSF with β2-transferrin test
        │
        ├── Infective → Otoscopy → EAC/TM pathology → Culture + Sensitivity
        │                                             → Treat accordingly
        │
        ├── Pulsatile bleeding → Glomus tumour / Vascular anomaly
        │                     → Pneumatic otoscopy (Brown's sign)
        │                     → HRCT + MRI + MRA/DSA
        │
        └── No obvious cause + Persistent → Biopsy after imaging
                                          → Exclude carcinoma

---

---

PART II: GLOMUS TYMPANICUS

---

INTRODUCTION AND HISTORICAL BACKGROUND

• Rosenwasser (1945) first described the jugulotympanic glomus body.
• Guild (1953) described the tympanic plexus (glomus tympanicum) and jugular adventitia (glomus jugulare) as the sites of origin.
• Glomus Tympanicus arises from the Jacobson's nerve (tympanic branch of CN IX) along the cochlear promontory (Scott-Brown, 8th ed.).
• Glomus Jugulare arises from the jugular fossa/adventitia of the jugular bulb.

Zakir Hussain's ENT classifies tympanojugular paragangliomas separately as they represent a continuum of the same pathological entity.

---

ANATOMY OF THE GLOMUS BODY

1. Along the tympanic branch of CN IX (Jacobson's nerve) on the cochlear promontory → gives rise to Glomus Tympanicus
2. Along the auricular branch of CN X (Arnold's nerve) in the jugular fossa → gives rise to Glomus Jugulare

---

PATHOLOGY

• Gross: Reddish-brown, lobulated, highly vascular mass
• Microscopy: Nests of epithelioid chief cells (Zellballen pattern) in a richly vascular fibrous stroma; surrounded by sustentacular (S100+) cells
• Immunohistochemistry: Chief cells positive for chromogranin A, synaptophysin, NSE; sustentacular cells positive for S-100
• Malignancy: Rare (<5%); diagnosed by metastasis (to lymph nodes, lung, liver) NOT by histology alone (Cummings, 7th ed., Chapter 120)
• Genetics: 30% are hereditary; mutations in succinate dehydrogenase (SDH) genes — SDHB, SDHC, SDHD (Recent advances)

---

CLASSIFICATION

FISCH CLASSIFICATION (Most widely used — Hazarika, Dhingra, Cummings)

GLASSCOCK-JACKSON CLASSIFICATION (for Glomus Tympanicus specifically)

Glomus Tympanicus = Fisch Type A / Glasscock-Jackson Type I or II, confined to the middle ear, best prognosis (Scott-Brown, 8th ed., p. 3278).

---

EPIDEMIOLOGY

---

CLINICAL FEATURES

SYMPTOMS (Dhingra, 7th ed.; Hazarika; Stell & Maran)

• Rhythmic, synchronous with pulse
• Low-frequency, roaring quality
• Aggravated by exertion, relieved by carotid compression ipsilaterally (reduces arterial pressure) — Aquino's sign
• May cease with jugular vein compression — Branham's sign (more specific for AV fistula but noted)

• Progressive, unilateral
• Due to mass effect on ossicular chain or filling middle ear cavity
• Rarely sensorineural if cochlea is invaded (Fisch C/D)

• Spontaneous haemorrhagic discharge
• Brisk, pulsatile
• May occur after attempted biopsy → catastrophic haemorrhage if biopsy is taken without prior imaging (Zakir Hussain's ENT — strong caution against biopsy)

• Sensation of blockage in the ear

• Mild; deep-seated if erosion of bone present

• CN VII (facial palsy) — indicates temporal bone extension
• CN IX, X, XI, XII — Jugular foramen syndrome (Vernet's syndrome) in advanced Glomus Jugulare

• Episodic hypertension, palpitations, sweating, headache
• Must be excluded before surgery or embolisation

---

SIGNS

• Reddish-blue or cherry-red pulsatile mass seen through the intact tympanic membrane in the anterior-inferior quadrant (over the cochlear promontory / hypotympanum)
• The mass may be visible as a vascular blush or complete tympanic filling

On pneumatic otoscopy: Applying positive pressure causes blanching (pallor) of the reddish mass as blood is squeezed out; on releasing pressure, it re-flushes red (pulsatile). This blanching and pulsation with pneumatic pressure = Brown's sign (Stell & Maran, 5th ed.; Hazarika, 4th ed.).

• In hypotympanic tumour, a reddish glow visible below the annulus — like a rising sun — as the mass pushes up from below (Cummings, 7th ed.)

• Pulsatile tinnitus decreases / ceases on compressing the ipsilateral carotid artery

---

IMAGING OF GLOMUS TYMPANICUS

---

INVESTIGATIONS

FLOWCHART OF INVESTIGATIONS

SUSPECTED GLOMUS TYMPANICUS
(Pulsatile tinnitus + reddish middle ear mass + Brown's sign)
              │
              ├─── AUDIOMETRY
              │    ├── Pure Tone Audiogram → Conductive hearing loss
              │    └── Tympanometry → Pulsatile movements on tympanogram (Type B/As)
              │
              ├─── IMAGING (Mandatory BEFORE biopsy)
              │    ├── HRCT Temporal Bone (First line)
              │    │    └── Soft tissue mass on promontory
              │    │        No bony erosion in Tympanicus
              │    │        Bony erosion suggests Jugulare
              │    │
              │    ├── MRI + Gadolinium (Second line)
              │    │    ├── "Salt and pepper" appearance on T1 (large tumours)
              │    │    │   [Flow voids = pepper; haemorrhage = salt]
              │    │    └── Intense enhancement with contrast
              │    │
              │    └── MRA / DSA (if vascular anatomy needed pre-op)
              │         └── Identifies feeding vessels
              │             Permits pre-operative embolisation
              │
              ├─── BIOCHEMICAL (Exclude functional tumour)
              │    ├── 24-hour urine: VMA, metanephrines, catecholamines
              │    └── Plasma metanephrines (more sensitive — Recent advances)
              │
              ├─── NUCLEAR MEDICINE
              │    ├── Octreotide scan (In-111 DTPA octreotide)
              │    │   Paragangliomas express somatostatin receptors
              │    └── 68-Ga DOTATATE PET-CT (Recent — Gold Standard)
              │        Superior sensitivity for multicentric/metastatic disease
              │
              ├─── GENETIC TESTING (Hereditary cases)
              │    └── SDH gene mutations: SDHB, SDHC, SDHD
              │        (Indicated in: young patients, bilateral, multicentric, FH)
              │
              └─── BIOPSY
                   CONTRAINDICATED without prior imaging
                   Only after vascular lesion excluded

AUDIOLOGICAL INVESTIGATIONS (in detail)

IMAGING CHARACTERISTICS

---

DIFFERENTIAL DIAGNOSIS

Zakir Hussain's ENT specifically emphasises that aberrant ICA must always be excluded before any middle ear exploration or biopsy.

---

MANAGEMENT OF GLOMUS TYMPANICUS

TREATMENT ALGORITHM

CONFIRMED GLOMUS TYMPANICUS (Fisch Type A / G-J Type I–IV)
                    │
          ┌─────────┴──────────┐
    Functional?           Non-functional
    (catecholamine         (majority)
    secreting)                │
          │                   │
    Alpha-blocker         Assess:
    preoperatively        - Age/fitness
    (Phenoxybenzamine     - Tumour size/extent
    10–14 days)           - Hearing status
                          - Patient preference
                                │
              ┌─────────────────┼──────────────┐
           Type I/II        Type III/IV       Poor surgical
           (Small/Medium)   (Extended)        candidate/elderly
              │                 │                    │
         SURGICAL            SURGICAL +          RADIOTHERAPY
         (Transcanal/        Pre-op              (Primary or
         Tympanotomy)        Embolisation        adjuvant)
              │              → Infratemporal
              │              fossa approach
              │              (Fisch Type A/B)
              │
        COMPLETE EXCISION → CURE

---

SURGICAL MANAGEMENT (Mainstay of Treatment)

PRE-OPERATIVE PREPARATION

1. Exclude functional tumour — 24-hr urine VMA + plasma metanephrines
   • If positive: Phenoxybenzamine (alpha-blocker) 10–14 days preoperatively
   • Beta-blocker NEVER given first (risk of hypertensive crisis)
2. Pre-operative embolisation (24–72 hours before surgery)
   • Reduces intraoperative blood loss by 40–60%
   • Via ascending pharyngeal artery (main feeder)
   • Technique: Seldinger approach, superselective catheterisation, PVA particles / coils (Cummings, 7th ed., p. 2099)
3. Cross-matching blood, ICU availability
4. Audiometric baseline
5. Facial nerve monitoring setup intraoperatively

---

SURGICAL APPROACHES (Based on Fisch Classification)

TRANSCANAL APPROACH (Glomus Tympanicus — Step by Step)

1. General anaesthesia (hypotensive preferred — MAP 60–65 mmHg)
2. Injection of 1% Lignocaine with 1:100,000 adrenaline — EAC infiltration
3. Tympanomeatal flap elevation — posteriorly based, 6 o'clock to 12 o'clock
4. Middle ear exploration — identify tumour on cochlear promontory
5. Bipolar diathermy to coagulate feeding vessels (inferior tympanic artery = Jacobson's artery)
6. Tumour dissection from the promontory — suction dissection + microsurgical technique
7. Ossicular chain assessment and preservation where possible
8. Haemostasis with Surgicel/Gelfoam
9. TM flap repositioning + canal packing

Key surgical principle: Glomus Tympanicus is usually completely resectable with transcanal approach alone, with cure rates >95% (Cummings, 7th ed.; Stell & Maran).

---

RADIOTHERAPY

Indications:

• Elderly/medically unfit patients
• Residual/recurrent tumour post-surgery
• Unresectable disease (Fisch C3/D)
• Patient refusal for surgery
• Bilateral tumours (preserve hearing on better side)

Technique:

• Conventional external beam RT: 45–50 Gy in 25 fractions
• Stereotactic Radiosurgery (SRS) — Gamma Knife / CyberKnife (Recent advance)
  • Marginal dose: 12–15 Gy to 50% isodose line
  • Local control: 90–95% at 5 years
  • Cranial nerve preservation superior to surgery
  • Preferred for Fisch C/D tumours and elderly patients (Cummings, 7th ed., p. 2105)

Mechanism:

---

WATCHFUL WAITING (Observation)

• Elderly patients with small, asymptomatic Fisch Type A tumours
• Serial HRCT/MRI every 1–2 years
• Tumours are slow-growing (mean growth rate 0.8 mm/year)
• Re-evaluate if symptoms progress (Hazarika, 4th ed.)

---

COMPLICATIONS OF SURGERY

---

PROGNOSIS

---

RECENT ADVANCES (Important for RGUHS)

1. Genetics and Hereditary Paraganglioma Syndromes

• SDH gene mutations (SDHB, SDHC, SDHD) identified in 25–35% of cases
• SDHB mutations associated with malignancy (sympathetic paraganglioma)
• Genetic counselling + testing of first-degree relatives now recommended (Cummings 7th ed.; Baysal et al., NEJM 2000)

2. 68-Ga DOTATATE PET-CT

• Gold standard for imaging paragangliomas (sensitivity >95%)
• Identifies multicentric disease (up to 40% in hereditary)
• Replaces Octreotide scintigraphy (Timmers et al., JCEM 2012)

3. Stereotactic Radiosurgery (SRS) / Gamma Knife

• Local control 92–97% at 5 years for Glomus Tympanicus/Jugulare
• Cranial nerve preservation significantly better than open surgery for C/D
• Increasingly preferred as primary treatment for Fisch C/D (Cummings 7th ed.; Liscak et al., 2016)

4. Endoscopic Ear Surgery (EES) for Glomus Tympanicus

• Fully endoscopic transcanal approach (4 mm, 0° and 30° rigid endoscopes)
• Advantages: Superior visualisation of hypotympanum, no external incision
• Growing evidence for Glasscock-Jackson Type I–III (Marchioni et al., Otolaryngol Head Neck Surg, 2017)

5. PRRT (Peptide Receptor Radionuclide Therapy)

• 177Lu-DOTATATE for malignant/metastatic paraganglioma
• Targets somatostatin receptor subtype 2 (SSTR2)
• Approved for neuroendocrine tumours; trials ongoing for head and neck paragangliomas (Strosberg et al., NEJM 2017)

6. Targeted Systemic Therapy

• Sunitinib, Cabozantinib (VEGFR inhibitors) — phase II trials
• For metastatic SDHB-mutated paragangliomas
• Temozolomide-based chemotherapy for rapidly progressive disease

7. Intraoperative Neuromonitoring

• Continuous facial nerve EMG monitoring now standard for all skull base surgery
• Reduces permanent facial palsy rates significantly

---

SUMMARY TABLE: GLOMUS TYMPANICUS vs GLOMUS JUGULARE

---

KEY EXAMINATION POINTS FOR RGUHS

1. Brown's sign is pathognomonic of glomus tumour of the middle ear
2. Never biopsy a pulsatile reddish middle ear mass without prior imaging (HRCT + MRI)
3. Aberrant ICA must be excluded before any middle ear procedure
4. Fisch classification is the most used globally; Glasscock-Jackson for tympanicus specifically
5. Pre-operative alpha-blockade (Phenoxybenzamine) is mandatory for functional paragangliomas
6. Pre-operative embolisation 24–72 hrs before surgery reduces blood loss
7. Treatment of choice for Glomus Tympanicus is transcanal surgical excision
8. Stereotactic radiosurgery is preferred for elderly/unfit and Fisch C/D
9. SDH gene mutations — hereditary; genetic counselling recommended
10. 68-Ga DOTATATE PET-CT is the modern gold standard for staging/multicentricity

---

REFERENCES

1. Scott-Brown's Otorhinolaryngology, Head & Neck Surgery, 8th ed. — Gleeson et al. — Chapter on Paragangliomas of the temporal bone
2. Cummings Otolaryngology Head and Neck Surgery, 7th ed. — Flint et al. — Chapter 120: Paragangliomas of the Head and Neck
3. Stell & Maran's Head and Neck Surgery, 5th ed. — Watkinson & Gilbert — Temporal bone tumours
4. Diseases of Ear, Nose and Throat, 7th ed. — P.L. Dhingra — Chapter on Tumours of the Ear
5. Textbook of ENT and Head & Neck Surgery, 4th ed. — Hazarika et al. — Glomus tumours
6. ENT — Zakir Hussain — Practical ENT examination guide
7. Baysal et al. (2000) — Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma — Science, 287:848
8. Timmers et al. (2012) — Comparison of 18F-DOPA, 18F-FDG, and 18F-FDA PET/CT — J Clin Endocrinol Metab
9. Marchioni et al. (2017) — Endoscopic management of glomus tympanicum — Otolaryngol Head Neck Surg
10. Liscak et al. (2016) — Gamma knife radiosurgery of glomus jugulare tumours — Stereotact Funct Neurosurg

---