Overview
Definition:
Cholesteatoma is a non-cancerous skin growth that occurs in the middle ear behind the eardrum
It is typically an accumulation of stratified squamous epithelium and keratin debris within the epitympanum or mastoid
In children, it can be congenital or acquired, with acquired forms often arising from chronic eustachian tube dysfunction and squamous migration into the middle ear space
Congenital cholesteatomas are thought to arise from trapped epidermal rests during fetal development
They can erode surrounding bone and soft tissues, leading to hearing loss, dizziness, facial nerve paralysis, and intracranial complications.
Epidemiology:
Cholesteatoma accounts for approximately 3% of all perforations of the tympanic membrane
While it can occur at any age, pediatric cholesteatoma is less common than in adults, though it can present with significant sequelae
Congenital cholesteatoma is rare, accounting for an estimated 0.09% to 0.35% of all cholesteatomas
Acquired cholesteatomas are more common and are often associated with recurrent otitis media and poor eustachian tube function
Boys are slightly more commonly affected than girls
There is no significant racial predilection for congenital cholesteatoma, but acquired forms are more prevalent in populations with higher rates of otitis media.
Clinical Significance:
Cholesteatoma poses a significant clinical challenge in pediatrics due to its potential for progressive destruction of the middle ear and mastoid structures
Early diagnosis and appropriate management are crucial to prevent irreversible hearing loss, chronic otorrhea, and potentially life-threatening complications such as labyrinthitis, facial nerve palsy, meningitis, and brain abscess
For DNB and NEET SS candidates, understanding the diagnostic nuances, management strategies, and referral pathways for pediatric cholesteatoma is essential for competent patient care and successful examination performance.
Clinical Presentation
Symptoms:
Most common symptom is chronic or recurrent ear discharge (otorrhea), often foul-smelling and purulent
Hearing loss is common, typically conductive, and may be progressive
Ear fullness or pressure
Tinnitus may be present
Vertigo or dizziness can occur if the labyrinth is involved
Facial nerve weakness or paralysis (rare but serious)
Pain (otalgia) is less common unless there is superimposed infection.
Signs:
Otorrhea emanating from a tympanic membrane perforation
Granulation tissue or debris visible in the middle ear or attic through the perforation
Retraction pockets of the tympanic membrane, especially in the pars flaccida
Evidence of ossicular erosion on otoscopy or audiometry
In advanced cases, signs of complications like facial nerve palsy, post-auricular swelling (indicating mastoiditis), or nystagmus.
Diagnostic Criteria:
There are no strict diagnostic criteria for cholesteatoma itself, but a high index of suspicion is raised with chronic otorrhea and hearing loss in the presence of a tympanic membrane perforation or significant retraction pocket
Definitive diagnosis is usually made intraoperatively or via high-resolution CT imaging
Histopathological examination of surgical specimens confirms the diagnosis
For congenital cholesteatoma, diagnosis is often made incidentally during evaluation for other conditions, and it is characterized by its location behind an intact tympanic membrane.
Diagnostic Approach
History Taking:
Detailed history of ear discharge: onset, duration, character (color, odor), and response to antibiotics
History of recurrent otitis media or previous ear surgery
Assessment of hearing loss: subjective reports of difficulty hearing
Any history of ear trauma or previous ear infections
Family history of hearing problems or ear conditions
Red flags: sudden onset of severe pain, facial droop, vertigo, or significant hearing loss suggestive of complications.
Physical Examination:
Thorough otoscopic examination is paramount
Assess the tympanic membrane for perforations (marginal vs
central), retraction pockets, attic perforations, and the presence of cholesteatoma matrix or debris
Examine the external auditory canal for discharge, granulation tissue, or signs of external otitis
Assess for cranial nerve deficits, particularly facial nerve function
Palpate the mastoid for tenderness or post-auricular swelling
Evaluate nasal and oral mucosa for signs of inflammatory conditions.
Investigations:
Audiometry: Essential to assess the type and degree of hearing loss (conductive, sensorineural, or mixed)
Tympanometry can assess middle ear status and eustachian tube function
High-resolution computed tomography (HRCT) of the temporal bone is the investigation of choice for suspected cholesteatoma, especially before surgery
It can delineate the extent of the cholesteatoma, bony erosion (ossicles, scutum, tegmen), and involvement of the facial nerve canal and mastoid
Pure tone audiometry is crucial for baseline assessment and post-operative monitoring
MRI may be useful in cases of suspected intracranial extension or to differentiate cholesteatoma from other soft tissue masses.
Differential Diagnosis:
Chronic suppurative otitis media (CSOM) with simple perforation
Otomycosis (fungal ear infection)
Foreign body in the ear canal
Middle ear granulation tissue without cholesteatoma
Tympanosclerosis
Cholesterol granuloma
Benign or malignant middle ear tumors (rare in children)
External otitis with secondary middle ear involvement.
Management
Initial Management:
For suspected cholesteatoma with active otorrhea, initial management involves thorough cleaning of the ear canal and middle ear under magnification
A course of topical antibiotic drops (e.g., ofloxacin, ciprofloxacin) with or without systemic antibiotics may be initiated to control infection and inflammation
The goal is to create a dry ear and a clear view for further assessment and definitive management
Referral to an ENT specialist is mandatory.
Medical Management:
Medical management is primarily supportive and aimed at controlling infection and inflammation in the initial stages
Topical antibiotic drops are the mainstay for managing otorrhea
Systemic antibiotics are used for acute exacerbations or signs of spreading infection
Nasal decongestants and intranasal corticosteroids may be prescribed to improve eustachian tube function in cases of associated Eustachian tube dysfunction
However, medical management alone does not resolve cholesteatoma.
Surgical Management:
Surgical intervention is the definitive treatment for cholesteatoma
The goals are to remove the cholesteatoma matrix, eradicate infection, preserve or reconstruct hearing, and create a safe, dry ear
Surgical options include: Tympanoplasty: Reconstruction of the eardrum and ossicles
Mastoidectomy: Removal of diseased mastoid bone
Bondy-Ritter procedure: Conservative mastoidectomy with preservation of the posterior canal wall
Canal wall-up or canal wall-down mastoidectomy: Depending on the extent of disease, surgeon preference, and patient factors
Minimally invasive techniques and endoscopic approaches are increasingly used
Congenital cholesteatomas are typically removed surgically through a mastoidectomy or middle ear exploration, often with preservation of the posterior canal wall.
Supportive Care:
Post-operative care includes regular ear canal cleaning, dry ear precautions, and topical/oral antibiotic therapy as prescribed
Pain management with analgesics is important
Careful follow-up with regular otoscopic examinations and audiometry is crucial to monitor for recurrence and assess hearing outcomes
Patient and caregiver education on ear care and warning signs of complications is vital.
Complications
Early Complications:
Post-operative infection (wound infection, otitis externa)
Facial nerve injury (partial or complete paralysis)
Vestibular dysfunction (vertigo, nystagmus)
Conductive hearing loss post-surgery
Chorda tympani injury leading to taste disturbance
Granuloma formation at the surgical site.
Late Complications:
Cholesteatoma recurrence is the most significant late complication, occurring in up to 15-30% of cases, depending on surgical technique and follow-up
Hearing loss (conductive or sensorineural)
Chronic otorrhea
Mastoid cavity obliteration or collapse
Chronic otitis media
Labyrinthitis (inflammation of the inner ear)
Meningitis
Brain abscess
Sigmoid sinus thrombosis
Dural abscess.
Prevention Strategies:
Prompt and effective management of acute otitis media to prevent tympanic membrane perforation and subsequent acquired cholesteatoma
Vigorous management of eustachian tube dysfunction
Careful surgical technique to ensure complete removal of cholesteatoma matrix and avoid damage to vital structures
Regular, long-term post-operative surveillance to detect recurrence early.
Prognosis
Factors Affecting Prognosis:
The prognosis for pediatric cholesteatoma is generally good with complete surgical eradication and appropriate follow-up
Factors influencing prognosis include the size and location of the cholesteatoma, the extent of bony erosion, the presence of complications, the surgical technique employed, and the diligence of post-operative follow-up
Recurrence rates are higher in cases with marginal perforations or attic cholesteatomas.
Outcomes:
Successful surgical treatment typically leads to a dry ear and cessation of otorrhea
Hearing outcomes vary depending on the extent of ossicular damage and the success of reconstruction
Many children achieve significant hearing improvement or stabilization
Long-term hearing preservation or restoration is a key goal of surgical management.
Follow Up:
Lifelong, regular follow-up is recommended for patients with cholesteatoma, even after successful surgery
This includes frequent otoscopic examinations (e.g., every 6-12 months for several years) to monitor for recurrence or complications
High-resolution CT scans may be performed periodically if recurrence is suspected
Audiometry should be performed regularly to track hearing status.
Key Points
Exam Focus:
Cholesteatoma is a destructive process requiring surgical intervention
Differentiate congenital from acquired forms
HRCT temporal bone is key for pre-operative planning
Recurrence is common
hence, long-term follow-up is mandatory
Complications can be life-threatening
Referral to ENT specialist is crucial for any suspected case.
Clinical Pearls:
Suspect cholesteatoma in any child with persistent or recurrent foul-smelling ear discharge, even with an intact eardrum (congenital)
A "pear-like" whitish mass seen behind the eardrum or in a retraction pocket is highly suspicious
Always perform a formal audiogram
Do not rely solely on otoscopy for diagnosis of extent or recurrence.
Common Mistakes:
Delaying referral to an ENT specialist
Inadequate cleaning of the ear canal before otoscopic examination, obscuring findings
Relying on antibiotics alone for chronic otorrhea without investigating for cholesteatoma
Incomplete surgical removal of the cholesteatoma matrix
Insufficient post-operative follow-up leading to missed recurrences.