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BLEEDING FROM THE EAR (OTORRHAGIA) AND GLOMUS TYMPANICUS

RGUHS University — 50 Marks Long Answer

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PART I: BLEEDING FROM THE EAR (OTORRHAGIA)

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CAUSES OF BLEEDING FROM THE EAR

A. CLASSIFICATION

┌─────────────────────────────────────────────────────────────┐
│                   OTORRHAGIA — CAUSES                       │
├─────────────────┬───────────────────────────────────────────┤
│   LOCAL CAUSES  │          SYSTEMIC CAUSES                  │
├─────────────────┼───────────────────────────────────────────┤
│  Traumatic      │  Bleeding diatheses                       │
│  Inflammatory   │  Leukaemia                                │
│  Neoplastic     │  Anticoagulant therapy                    │
│  Vascular       │  Thrombocytopenia                         │
└─────────────────┴───────────────────────────────────────────┘

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B. LOCAL CAUSES — DETAILED

1. TRAUMATIC (Most Common Group)

           Temporal Bone Fractures
                    │
         ┌──────────┴──────────┐
    Longitudinal              Transverse
    (80–90%)                  (10–20%)
         │                         │
  - Along EAC axis          - Perpendicular to EAC
  - TM tear + blood         - Haemotympanum
  - Conductive hearing loss - SNHL / total deafness
  - Facial nerve rare       - Facial nerve 50%
  - CSF otorrhoea ++        - Vertigo severe

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2. INFLAMMATORY / INFECTIVE

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3. NEOPLASTIC (Very Important for RGUHS)

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4. VASCULAR CAUSES

• Aberrant internal carotid artery — pulsatile mass behind TM; risk of catastrophic haemorrhage if biopsied or myringotomised
• High jugular bulb — bluish-red mass in floor of middle ear
• Aneurysm of ICA — very rare
• Arteriovenous malformation

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5. MISCELLANEOUS

• Vicarious menstruation (Endometriosis of EAC) — cyclical bleeding; extremely rare
• Spontaneous TM rupture in pressure change (diving, flying)

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C. SYSTEMIC CAUSES

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D. APPROACH TO A PATIENT WITH OTORRHAGIA

                    OTORRHAGIA
                        │
          ┌─────────────┴──────────────┐
       H/O Trauma?                 No Trauma
          │                            │
   Temporal bone                  Otoscopy
   fracture W/U                       │
   (CT temporal bone)      ┌──────────┴──────────┐
                        Blood in EAC          Haemotympanum
                           │                       │
                    ┌──────┴───────┐           (No TM
                 Active         Old/dry         perforation)
                bleeding       blood            CT temporal bone
                    │                        Rule out:
               Inspect EAC               - Glomus tumour
               under microscope          - Aberrant ICA
               Rule out:                 - High JB
               - Laceration EAC
               - Foreign body
               - Furunculosis
               - Carcinoma EAC
               - Glomus tumour

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PART II: GLOMUS TYMPANICUS (GLOMUS TYMPANICUM)

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1. INTRODUCTION AND DEFINITION

• Synonyms: Chemodectoma, paraganglioma of middle ear, non-chromaffin paraganglioma
• Eponym: First described by Guild (1941) as glomus body; Rosenwasser (1945) described first case
• Histology: Identical to carotid body tumour — chief cells (type I) + sustentacular cells (type II) in Zellballen pattern

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2. ANATOMY OF PARAGANGLIA OF THE EAR

     PARAGANGLIA RELEVANT TO EAR
     ┌─────────────────────────────────────┐
     │  Jacobson's nerve (Tympanic br IX)  │ → Glomus tympanicum
     │  Arnold's nerve (Auricular br X)    │ → Glomus tympanicum
     │  Jugular bulb / adventitia of ICV   │ → Glomus jugulare
     │  Along CN IX/X/XI in jugular fosse  │ → Glomus jugulare
     └─────────────────────────────────────┘

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3. EPIDEMIOLOGY

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4. AETIOLOGY AND PATHOGENESIS

Normal paraganglionic chief cells (Jacobson's nerve on promontory)
                    │
          Neoplastic transformation
          (exact stimulus unknown)
                    │
        ┌───────────┴────────────┐
   Sporadic (75%)           Hereditary (25%)
        │                        │
   Unknown                 SDH gene mutations
                        (SDHB, SDHC, SDHD)
                        SDHD — most common
                        for head & neck PGLs
                             │
                      Autosomal dominant
                      (genomic imprinting—
                       paternal transmission)

• SDHD mutations — multiple head and neck paragangliomas; low malignancy risk
• SDHB mutations — higher risk of malignancy and metastasis (Neuroendocrine Neoplasms, p. 101)
• SDHC mutations — mostly head and neck; low malignancy
• Genetic counselling and cascade screening recommended for all first-degree relatives

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5. PATHOLOGY

• Reddish-brown, highly vascular, lobulated mass
• Sits on cochlear promontory
• Bleeds profusely on manipulation

• Zellballen pattern — nests/clusters of chief cells (type I) surrounded by sustentacular cells (type II)
• Chief cells: round-polygonal, granular eosinophilic cytoplasm, vesicular nuclei
• Rich fibrovascular stroma
• Neurosecretory granules on electron microscopy
• IHC: Chromogranin A (+), Synaptophysin (+), S-100 for sustentacular cells (+)

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6. CLASSIFICATION

a) GLASSCOCK-JACKSON CLASSIFICATION (Most Widely Used)

┌────────┬────────────────────────────────────────────────────┐
│ Type   │ Description                                        │
├────────┼────────────────────────────────────────────────────┤
│ Type I │ Small mass on promontory                           │
│ Type II│ Completely filling the middle ear space            │
│ Type III│ Fills middle ear + extends into mastoid           │
│ Type IV│ Fills middle ear, mastoid, extends through TM     │
│        │ into EAC; may involve ICA anteriorly               │
└────────┴────────────────────────────────────────────────────┘

b) FISCH CLASSIFICATION

┌────────┬────────────────────────────────────────────────────┐
│ Class  │ Description                                        │
├────────┼────────────────────────────────────────────────────┤
│ A      │ Tumour limited to middle ear (promontory)          │
│ B      │ Tumour in middle ear + mastoid; no bone destruction│
│ C      │ Involves infralabyrinthine compartment; ICA canal  │
│ D      │ Intracranial extension                             │
│  D1    │ < 2 cm intracranial extension                      │
│  D2    │ > 2 cm intracranial extension                      │
└────────┴────────────────────────────────────────────────────┘

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7. CLINICAL FEATURES

a) SYMPTOMS

         GLOMUS TYMPANICUM — SYMPTOM TRIAD
         ┌──────────────────────────────────┐
         │ 1. Pulsatile tinnitus (Most       │
         │    common symptom; 80–90%)        │
         │ 2. Conductive hearing loss        │
         │ 3. Bleeding from ear              │
         └──────────────────────────────────┘

b) SIGNS

CRITICAL: Never biopsy a pulsatile middle ear mass without prior imaging — risk of catastrophic haemorrhage!

• Check for carotid body tumour (neck mass at carotid bifurcation)
• Check for other paragangliomas
• BP monitoring (catecholamine secretion)
• Lower cranial nerve assessment

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8. DIAGNOSTIC INVESTIGATIONS

FLOW CHART — INVESTIGATIONS

                 Suspected Glomus Tympanicum
                          │
              ┌───────────┴────────────┐
           Clinical                Imaging
           Tests                      │
              │         ┌─────────────┼──────────────┐
       ┌──────┴──────┐  CT            MRI           Angio
       │Audiometry   │  Temporal      Head/Neck     -graphy
       │Tympanometry │  Bone          with          (if needed)
       │PTA          │  (HRCT)        Contrast
       │ABR          │     │              │
       │24hr urine   │  Bony          'Salt &
       │VMA/catechol │  anatomy       Pepper'
       │Chromogranin │  extent        pattern
       │Genetic      │
       │testing SDHB │
       └─────────────┘
                │
          If secreting:
          Octreotide scan
          (Ga-68 DOTATATE PET)

a) AUDIOLOGICAL INVESTIGATIONS

b) IMAGING

• Soft tissue mass on cochlear promontory (mesotympanum)
• Isodense to muscle on non-contrast
• Intense enhancement on contrast (confirming vascularity)
• Assess: ossicular chain involvement, tegmen integrity, bony erosion, jugular foramen, ICA canal
• No bony erosion in early glomus tympanicum (distinguishes from glomus jugulare which erodes jugular spine)

• Required for large tumours or pre-operative embolisation
• Shows: highly vascular blush; feeding vessels (ascending pharyngeal artery, inferior tympanic artery)
• Pre-operative embolisation 24–72 hours before surgery to reduce intraoperative bleeding
• Also used for:
  • Identifying aberrant ICA
  • Balloon test occlusion

c) BIOCHEMICAL TESTS

Important: In secreting glomus tumours, a hypertensive crisis can be precipitated by induction of anaesthesia — always check before surgery!

d) GENETIC TESTING (Recent Advances)

• SDH gene panel (SDHB, SDHC, SDHD, SDHA, SDHAF2) recommended for all patients
• Especially important if: young age, multiple tumours, family history
• SDHB mutation → screen for renal cell carcinoma (paraganglioma-renal cell carcinoma syndrome)
• Neuroendocrine Neoplasms (p. 101): "Management should ideally be in specialist centres with multidisciplinary input. The risk of aggressive behaviour is higher in patients with germline SDHB mutations."

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9. DIFFERENTIAL DIAGNOSIS

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10. MANAGEMENT

MANAGEMENT FLOW CHART

              Confirmed Glomus Tympanicum
                        │
           ┌────────────┴────────────┐
      Small tumour               Large/advanced
      (Fisch A/B,                (Fisch C/D,
   Glasscock I/II)            Glasscock III/IV)
           │                         │
      MDT discussion           MDT discussion
           │                         │
    ┌──────┴──────┐           ┌───────┴───────┐
    │             │           │               │
  Surgery    Observation   Pre-op         Radiotherapy
  (curative) (elderly/     Embolisation   ± Surgery
             comorbid/         │
             small/       Surgery
             slow)       (complex/
                          staged)

A. OBSERVATION / ACTIVE SURVEILLANCE

• Elderly patients (> 70 years) with small tumours
• Significant comorbidities
• Single hearing ear
• Slow-growing tumour on serial imaging (6-monthly MRI)
• Patient preference

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B. SURGICAL MANAGEMENT (Treatment of Choice for Glomus Tympanicum)

1. Complete audiological workup
2. HRCT temporal bone + MRI with contrast
3. Biochemical tests (rule out secretion)
4. Pre-operative embolisation for large tumours (24–72 hours before surgery)
5. Informed consent — risk of bleeding, hearing loss, facial palsy

┌─────────────────┬──────────────────────────────────────────┐
│ Classification  │ Surgical Approach                         │
├─────────────────┼──────────────────────────────────────────┤
│ Fisch A / GJ I  │ Transcanal approach (under microscope)   │
│                 │ + Tympanotomy                             │
├─────────────────┼──────────────────────────────────────────┤
│ Fisch B / GJ II │ Transcanal + Extended tympanotomy        │
│                 │ or Endaural approach                      │
├─────────────────┼──────────────────────────────────────────┤
│ GJ III / Fisch B│ Postaural approach + Mastoidectomy       │
│ with mastoid    │                                           │
├─────────────────┼──────────────────────────────────────────┤
│ GJ IV / Fisch C │ Infratemporal fossa approach (Fisch)     │
│                 │ + ICA management                          │
├─────────────────┼──────────────────────────────────────────┤
│ Fisch D         │ Combined otological + neurosurgical       │
│ (intracranial)  │ approach; staged if needed               │
└─────────────────┴──────────────────────────────────────────┘

• Under general anaesthesia (hypotensive anaesthesia reduces bleeding)
• Endaural or post-aural incision
• Elevation of tympanomeatal flap
• Exposure of promontory
• Bipolar cautery of feeding vessels
• Excision of tumour from promontory (coagulate, dissect, remove)
• Haemostasis with oxidised cellulose/Gelfoam
• Replace TM flap; pack EAC
• Cure rate: > 95% for Type I/II

• Mastoidectomy
• Blind sac closure of EAC
• Rerouting of facial nerve anteriorly
• Exposure of infralabyrinthine and jugular compartments
• Control of sigmoid sinus and IJV
• Gross total resection

• Facial nerve — must be identified and preserved
• Carotid artery — aberrant ICA; dehiscent ICA
• Jugular bulb — torrential venous bleeding
• Ossicular chain — hearing preservation
• Cochlea/labyrinth — sensorineural hearing loss

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C. RADIOTHERAPY

• Elderly / unfit for surgery
• Residual or recurrent tumour
• Unresectable disease (large Fisch C/D)
• Patient refusal of surgery

• Local tumour control: 90–97% at 5 years
• Symptom improvement (tinnitus): 60–70%
• Low complication rate
• Considered equivalent to surgery for local control in Fisch A/B

• Proton beam therapy — superior dose conformation; less scatter to cochlea, brain
• Ga-68 DOTATATE PET-guided radiation planning for precise target delineation

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D. EMBOLISATION (Adjunctive)

• Pre-operative embolisation: 24–72 hours before surgery
• Reduces intraoperative blood loss by 40–60%
• Performed via selective catheterisation of inferior tympanic artery (branch of ascending pharyngeal artery)
• Materials: PVA particles, coils, Onyx
• Risks: stroke (if embolic material enters ICA), cranial nerve palsy

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E. MEDICAL / PHARMACOLOGICAL MANAGEMENT (Limited Role)

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11. COMPLICATIONS

Post-operative Complications:

          POST-OPERATIVE COMPLICATIONS
                     │
        ┌────────────┴──────────────┐
   Early                       Late
     │                           │
  - Haemorrhage             - Conductive HL
  - Facial palsy (temp)     - SNHL (if cochlea)
  - CSF leak                - Recurrence (5–15%)
  - Infection               - Permanent facial palsy
  - Meningitis              - Cholesteatoma
  - Pneumocephalus          - EAC stenosis

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12. PROGNOSIS AND FOLLOW-UP

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13. RECENT ADVANCES (Crucial for RGUHS)

1. Endoscopic ear surgery (EES) — Fully endoscopic transcanal approach for Fisch A/B glomus tympanicum using 0° and 45° endoscopes; better visualisation of hypotympanum; less morbidity; published in Otology & Neurotology (2018–2023)
2. Ga-68 DOTATATE PET-CT — Replaced conventional octreotide scan; near 100% sensitivity for paragangliomas; identifies multifocal disease and occult lesions; changes management in 30% of cases
3. Next-generation sequencing (NGS) gene panels — Comprehensive SDH gene panel testing allows identification of hereditary cases, risk stratification, and cascade family testing
4. PRRT (Lu-177 DOTATATE — LUTATHERA) — FDA approved (2018) for somatostatin-receptor positive gastroenteropancreatic NETs; now used off-label and in trials for head & neck paragangliomas with promising results (Neuroendocrine Neoplasms, p. 101)
5. Robotic surgery — Emerging role; reduced EAC approach feasibility studies
6. Gamma Knife radiosurgery — Long-term data (> 10 years) confirms 95%+ local control for glomus tympanicum with < 3% complication rate; now considered first-line alternative to surgery in many European centres
7. Image-guided navigation — Intraoperative CT/MRI navigation for complex Fisch C/D tumours; reduces risk to ICA and posterior fossa
8. Proton beam therapy — Cochlear dose reduction compared to photon IMRT; preserves hearing in young patients

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14. OTOENDOSCOPIC AND IMAGING FINDINGS

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15. SUMMARY TABLE: GLOMUS TYMPANICUM AT A GLANCE

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REFERENCES (Standard Textbooks per RGUHS Curriculum)

1. Scott-Brown's Otorhinolaryngology, Head & Neck Surgery (8th Ed.) — Chapter on Middle Ear Tumours
2. Cummings Otolaryngology — Head and Neck Surgery (7th Ed.) — Glomus Tumours of Temporal Bone
3. Stell and Maran's Head and Neck Surgery and Oncology (5th Ed.) — Paragangliomas
4. Dhingra's Diseases of Ear, Nose and Throat (8th Ed.) — Middle Ear Tumours, Otorrhagia
5. Hazarika P — Textbook of Ear, Nose and Throat — Glomus Tympanicum, Bleeding from Ear
6. Zakir Hussain — Textbook of ENT and Head & Neck Surgery — Otorrhagia, Middle Ear Tumours
7. Harrison's Principles of Internal Medicine (21st Ed., p. 1055) — Ear pain and related conditions
8. Neuroendocrine Neoplasms (p. 101) — Management of head and neck paragangliomas; SDH mutations; PRRT
9. Fisch U, Mattox D — Microsurgery of the Skull Base — Infratemporal fossa approaches
10. Glasscock ME, Shambaugh GE — Surgery of the Ear — Classification of glomus tumours
11. Boedeker CC et al. — Paragangliomas in SDH mutations — Head Neck 2009
12. Marchetti M et al. — Gamma Knife for glomus tympanicum — Neurosurgery 2018
13. Marchioni D et al. — Endoscopic approach for glomus tympanicum — Otol Neurotol 2020

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Examiner's Tip (RGUHS): In a 50-mark question, always: (1) define and classify, (2) discuss embryology/anatomy briefly, (3) cover all clinical features with signs, (4) give a structured investigation algorithm, (5) discuss management with surgical approach based on classification, (6) add recent advances. Diagrams of "Rising Sun sign," Glasscock-Jackson classification, and the investigation flowchart are frequently expected and carry significant marks.