Cholesteatoma in children

INTRODUCTION — A cholesteatoma (sometimes called a keratoma) is an abnormal growth of squamous epithelium in the middle ear and mastoid. It may progressively enlarge to surround and destroy the ossicles, resulting in conductive hearing loss. Hearing loss also may occur if the cholesteatoma obstructs the Eustachian tube orifice, leading to middle ear effusion. Early diagnosis is crucial to an optimal outcome.

The clinical features and treatment of cholesteatomas in children will be reviewed here. Other complications of otitis media are discussed separately. (See "Acute otitis media in children: Epidemiology, microbiology, and complications", section on 'Complications and sequelae'.)

TERMINOLOGY

●Cholesteatoma – A cholesteatoma is an abnormal accumulation of squamous epithelium within the middle ear and mastoid [1,2].

●Congenital cholesteatoma – The classic definition of a congenital cholesteatoma is a cholesteatoma that develops behind an intact tympanic membrane (TM) in a child with no history of middle ear disease (picture 1A) [3]. However, since most children have some history of middle ear disease during infancy, cholesteatomas are considered to be congenital if they occur in children who have an intact TM and no history of ear surgery [4-6].

●Acquired cholesteatoma – Acquired cholesteatomas develop after birth, usually as a complication of chronic middle ear disease.

EPIDEMIOLOGY — The true incidence of cholesteatoma is not known. Retrospective data suggest a mean annual incidence of 9.2 cases per 100,000 persons of all ages (range 3.7 to 13.9) [7]. In studies limited to children, the incidence ranges from approximately 5 to 15 per 100,000 children [2]. Cholesteatomas are bilateral in approximately 4 percent of cases [7,8].

●Congenital cholesteatoma – Congenital cholesteatomas account for 1 to 5 percent of cholesteatomas in most published series [9]. In a series of 172 cases of congenital cholesteatoma, the average age at diagnosis was five years [6]. Nearly three-quarters of cases occurred in boys. Most cases involved ≥2 quadrants. In cases that confined to one quadrant, 82 percent involved anterosuperior quadrant (figure 1). The ossicular chain and mastoid were involved in 43 percent and 23 percent of cases, respectively.

●Acquired cholesteatoma – Most cases of cholesteatoma are acquired. The posterosuperior quadrant is the most common location (figure 1). In a retrospective study of >45,000 children who had undergone tympanostomy tube placement, 1 percent developed cholesteatoma [10].

●Predisposing factors – Predisposing factors for acquired cholesteatoma include [1,7,10]:

•History of recurrent acute otitis media and/or chronic middle ear effusions

•Older age at first placement of tympanostomy tubes, and increasing number of, and longer interval between, insertions [10]

•Cleft palate [11-13]

•Craniofacial anomalies

•Turner syndrome [14]

•Down syndrome [15]

•Family history of chronic middle ear disease and/or cholesteatoma [16-18]

PATHOGENESIS

●Congenital – The origin of the squamous epithelium in congenital cholesteatomas is a matter of debate [9,19,20]. The most widely held theory suggests that fetal epithelial rests in the middle ear persist after birth and evolve into squamous inclusion cysts (congenital cholesteatomas) behind an intact tympanic membrane (TM) [21]. This theory is supported by the histologic demonstration of single or multiple epidermoid formations in fetal temporal bones that typically disappear before birth [22]. Alternative theories suggest that the middle ear is seeded by squamous cells in the amniotic fluid or from bits of the outer surface of the TM following infection or microperforation [23-25].

●Acquired – Acquired cholesteatomas usually arise from focal retractions of the TM. Less commonly, they can develop as a consequence of squamous epithelium entering the middle ear.

•Retraction pocket – Retraction pockets are invaginations of the TM that form in patients with chronic Eustachian tube dysfunction, negative middle ear pressure, and focal collapse of the TM [19,20]. As the retraction pocket is progressively pulled into the middle ear space, it forms a large pouch with a narrow neck. The pouch traps desquamating cells, forming a cholesteatoma lined by the metabolically active epithelium of the TM and filled with dead keratin debris (figure 2). The metabolically active squamous epithelium has the ability to erode bone: first, the delicate ossicles (picture 2) and then the solid cortices of the temporal bone. The keratin debris may become infected following water exposure or a middle ear infection, resulting in chronic purulent drainage (picture 1C). (See 'Natural history and complications' below.)

Areas of predilection for retraction pockets include old tympanostomy tube sites, the posterosuperior portion of the TM overlying the entrance to the mastoid, and the pars flaccida at the top of the TM (figure 3) [26]. Structural variations in the fibrous layer of the TM may account for weakness in these areas [27].

•Other mechanisms – Less common mechanisms by which squamous epithelium may enter the middle ear include [19]:

-Through a perforation at the outer edge of the TM (secondary acquired cholesteatoma)

-Through a temporal bone fracture (picture 3)

-As a rare complication of myringotomy (with or without tympanostomy tube placement) (picture 4) (see "Overview of tympanostomy tube placement, postoperative care, and complications in children", section on 'Cholesteatoma')

CLINICAL FEATURES

Presentation — Cholesteatomas may be asymptomatic or may present with symptoms attributable to the cholesteatoma (otorrhea, hearing loss) [1,28]. Most affected children have a history of recurrent acute otitis media and/or chronic middle ear effusions.

●Asymptomatic children – In children without symptoms, the lesion may be detected on routine physical examination based upon the following characteristic otoscopic findings (see 'Otoscopic examination' below):

•A white mass behind an intact TM (picture 1A-C), which is the most common presentation for congenital cholesteatomas [6,29]

•A deep retraction pocket with or without granulation and skin debris (picture 5)

•Focal granulation on the surface of the TM, especially at the periphery (picture 6)

●Symptomatic children – Chronic ear drainage is the most common presenting symptom for acquired cholesteatomas [29]. Children who develop cholesteatoma as a complication of prior surgery may present with new onset hearing loss in the affected ear. Children rarely complain of hearing loss, particularly if it is unilateral [2]. Thus, it is important to test hearing periodically in children with a history of recurrent or persistent middle ear disease. Rarely, children with cholesteatoma may present with a late complication (eg, facial nerve palsy). (See 'Natural history and complications' below and "Hearing loss in children: Screening and evaluation".)

Otoscopic findings

Congenital cholesteatomas — Congenital cholesteatomas can occur anywhere in the temporal bone but have a predilection for the anterosuperior quadrant of the middle ear, just above the Eustachian tube opening (picture 1A) [6,30]. Congenital cholesteatomas in this location may obstruct the Eustachian tube opening early in the course, resulting in middle ear effusion, which can complicate the diagnosis [6,31]. Congenital cholesteatomas also occur in the posterosuperior quadrant (picture 7) [4].

In the early stages, a congenital cholesteatoma may appear as a subtle whitish discoloration or spherical, white cyst behind an otherwise normal-appearing TM (picture 1B). As the lesion grows, it makes contact with the underside of the TM and becomes more obvious. With further expansion, the cholesteatoma can replace the middle ear space. The normal variegated appearance of the middle ear space is replaced by a bulging, white mass, which displaces the TM outwardly (picture 8). Perforation of the TM and chronic ear drainage are late findings. They usually follow the onset of hearing loss by months or years [30].

The mean age of diagnosis is four to five years [5,6,32].

Acquired cholesteatomas — Clinical features suggestive of acquired cholesteatomas include deep retraction pockets (picture 5), a white mass behind the TM (picture 1C), focal granulation at the periphery of the TM (picture 6), new-onset hearing loss, and ear drainage for more than two weeks despite treatment.

Retraction pockets are recognized by careful examination of the TM. They are said to be "shallow" when the full extent of the pocket is visible by otoscopy (picture 9). They are "deep" when they disappear into the crevices of the middle ear or into the mastoid air spaces (picture 5).

Deep pockets can collect debris that cannot be seen with otoscopy [33]. They are assumed to be cholesteatomas until proven otherwise. If a retraction pocket expands into the middle ear, its mouth may be visible near the edge of the TM. The cholesteatoma sac, filled with dead keratin debris, may appear as a white mass behind the TM (picture 1C).

NATURAL HISTORY AND COMPLICATIONS — Cholesteatomas that are not surgically excised continue to enlarge and may become secondarily infected.

Complications of cholesteatoma include hearing loss, cranial nerve palsies (facial [seventh cranial nerve], abducens [sixth cranial nerve]), other neurologic signs and symptoms (eg, vertigo), venous thrombosis, and potentially life-threatening infections (eg, brain abscess, meningitis) [4,34-39].

Congenital cholesteatoma may grow for years without causing symptoms, which highlights the importance of careful examination of the middle ear during routine health care maintenance [6,40]. In a review of 172 cases of congenital cholesteatoma from a single institution, the average age at diagnosis was five years. The ossicular chain was involved in 43 percent and the mastoid in 23 percent [6].

In the late stages, cholesteatoma (congenital or acquired) can destroy intratemporal structures, causing sensorineural deafness or paralysis of the facial nerve. It can erode through the walls of the mastoid, compressing the temporal lobe of the brain or presenting as a subcutaneous mass (image 1). (See "Facial nerve palsy in children".)

Advanced cholesteatomas may become secondarily infected [41]. The organisms most commonly cultured from infected cholesteatomas include Pseudomonas aeruginosa, Proteus species, Bacteroides, and Peptococcus/Peptostreptococcus [2]. Chronic bacterial infection may result in retrograde thrombosis of the cerebral veins, leading to formation of epidural abscess, a surgical emergency [42].

EVALUATION

Clinical suspicion — Cholesteatoma should be suspected in children with any of the following:

●Characteristic otoscopic findings on examination (see 'Otoscopic findings' above and 'Otoscopic examination' below)

●New-onset hearing loss in a previously operated ear

●An ear with persistent otorrhea for more >2 weeks despite appropriate treatment

Children with these findings should be referred to an otolaryngologist for confirmation of the diagnosis and definitive management. (See 'Diagnosis' below and 'Surgical treatment' below.)

Early detection of cholesteatoma requires careful otoscopic examination at routine health maintenance visits [1,43]. Early detection may avoid hearing loss and the need for extensive surgery [4,34]. Early lesions are best detected by inspecting the outer edges of the tympanic membrane (TM).

Otoscopic examination — Characteristic otoscopic findings of cholesteatoma include:

●A white mass behind an intact TM (picture 1A and picture 1B and picture 1C)

●A deep retraction pocket (picture 5)

●Focal granulation on the surface of the TM especially at the periphery (picture 6)

Particular attention should be paid to the posterosuperior quadrant and anterosuperior quadrant (figure 1), which are the most frequent locations of acquired and congenital cholesteatomas, respectively. (See 'Acquired cholesteatomas' above and 'Congenital cholesteatomas' above.)

To ensure an optimal examination, the otoscope's light source must have adequate brightness and cerumen removal may be necessary to expose the entire TM. (See "Cerumen", section on 'Cerumen removal'.)

Otoscopy may be difficult if there is middle ear fluid or if the child is not cooperative [2]. In such cases, if cholesteatoma is suspected, referral to otolaryngology for examination with an operating microscope may be necessary [2]. Computed tomography (CT) of the temporal bone is another option but is less helpful if there is fluid in the middle ear since middle ear fluid and cholesteatoma have the same appearance on CT. (See 'Diagnosis' below.)

Hearing evaluation — Children with suspected cholesteatoma should undergo audiometric testing to detect associated hearing loss. Cholesteatoma usually is associated with some degree of conductive hearing loss (maximum threshold of 50 decibels), although hearing may be normal if the ossicular chain is not involved [29]. (See "Hearing loss in children: Screening and evaluation".)

DIAGNOSIS — The diagnosis of cholesteatoma is confirmed by the otolaryngologist using visual examination under an operating microscope, high-resolution computed tomography (CT), diffusion-weighted magnetic resonance imaging (DW-MRI), and/or surgical exploration [28,44-46].

●Examination (with or without tympanostomy tube [TT] placement) – Visual examination and palpation of the TM under an operating microscope is often diagnostic. When an effusion is present, some surgeons prefer to place a TT during the initial examination under anesthesia as a preliminary step before proceeding with temporal bone CT and surgery [29,47]. TT placement enhances the value of CT by introducing air into the middle ear and mastoid (CT does not permit differentiation between fluid and the soft tissue density of the cholesteatoma). Preliminary TT placement also improves the chance of finding a dry, uninflamed middle ear at the time of cholesteatoma removal.

●Temporal bone CT – The evaluation usually includes high-resolution CT of the temporal bone to confirm the nature and extent of the lesion (image 1) and presence of complications. However, an experienced otolaryngologist may sometimes remove a small congenital cholesteatoma without performing CT.

●DW-MRI – DW-MRI can be helpful in the diagnosis of cholesteatoma since it can differentiate between cholesteatoma, middle ear fluid, and granulation tissue; however, it may not identify small volumes of cholesteatoma [48]. DW-MRI has been shown to be both sensitive and specific for detecting cholesteatoma in the temporal bone. Non-echo planar DW-MRI is better for detecting smaller lesions. DW-MRI can provide information on anatomic areas not easily seen on otoscopy and can assist with surveillance for recurrence after primary cholesteatoma surgery [49].

DIFFERENTIAL DIAGNOSIS — The differential diagnosis of cholesteatoma includes other white lesions of the tympanic membrane (TM). The following lesions may be easily confused with cholesteatomas (picture 10).

●Myringosclerosis appears as whitish plaques of calcium and phosphate crystals (picture 11). These plaques are firm on palpation, contained within the middle layer of the TM, typically crescent-shaped, and move with the TM during pneumatic otoscopy [29]. In contrast, cholesteatomas are soft, occupy the middle ear space, and do not move with the TM during pneumatic otoscopy.

●White foreign bodies (picture 12).

●Exostoses, which are outgrowths of bone into the external ear canal (picture 13).

●Prosthetic and graft material in operated ears.

●Inclusion cysts of TM (picture 14). These lesions occupy the middle layers of the TM but do not extend into the middle ear. Similar to myringosclerosis lesions but unlike cholesteatomas, inclusion cysts of the TM move with the TM during pneumatic otoscopy [29].

●Occasionally, a bulging acute otitis media may be hard to differentiate from a large congenital cholesteatoma (picture 15).

The history may be helpful in differentiating cholesteatoma from some of these lesions (eg, foreign body, prosthetic, and graft material). Otomicroscopy with palpation or computed tomography (CT) may be necessary to differentiate these disorders.

SURGICAL TREATMENT — The goals of surgical treatment are eradication of the keratinizing squamous epithelium from the temporal bone and preservation or restoration of hearing [50]. The extent and effectiveness of surgery depends upon the size and stage of the cholesteatoma [51].

Early cholesteatoma

●Congenital – Small, congenital cholesteatomas are usually treated by middle ear exploration and simple excision. Access to the tympanic membrane (TM) is either through the ear canal or via a postauricular incision, reflecting the pinna out of the way for better exposure.

The TM is reflected downward after incision of the ear canal skin (figure 4) and elevation of the top one-half of the tympanic annulus. Usually, the TM is left attached to the end of the malleus (umbo). The cyst is detached from its mucosal connections and delivered intact. The TM is returned to its normal anatomic position.

Excision of congenital cholesteatomas through a more limited exposure is possible using the newer generation of small endoscopes [52]. Endoscopically assisted transcanal surgery without a postauricular incision is often possible, even in young children with small external ear canals (picture 16) [53,54].

●Retraction pocket – Early retraction pocket cholesteatomas may be successfully treated with middle ear ventilation and mechanical reduction of the retraction pocket [47,55-57]. Tympanostomy tube placement (with or without adenoidectomy) releases the vacuum that retracts the TM. Once the middle ear is ventilated, it is often possible to mechanically reduce the pocket by removing debris and sucking the pocket out with a small Frazier ear suction (picture 5). The stretched segment of TM will return to its normal appearance, though it remains thin and will appear hypermobile on pneumatic otoscopy. It may retract again once the tube extrudes, so follow-up is important [55]. (See "Overview of tympanostomy tube placement, postoperative care, and complications in children", section on 'Postoperative care'.)

Retraction pockets that cannot be reduced require excision and grafting of the TM. For lesions limited to the middle ear, exploration of the middle ear and microdissection of the cholesteatoma sac (matrix) is usually sufficient. Endoscopes allow access to recesses of the middle ear not normally visible with microscopy [58]. With endoscopic or endoscopically assisted techniques, it may be possible to preserve involved ossicles and avoid mastoidectomy in some cases without compromising long-term control of disease (picture 17) [59,60].

Because the cholesteatoma sac is a retracted portion of the TM, its removal produces a hole that must be repaired, usually with the thin, strong fascia overlying the temporalis muscle or with cartilage from the pinna or tragus. Tympanostomy tube placement (with or without adenoidectomy) at the time of grafting or at the first sign of retraction may prevent recurrent cholesteatoma formation.

If the ossicles have been damaged by the osteolytic effects of the cholesteatoma or if it was necessary to remove a portion of the ossicles to fully expose the cholesteatoma, the ossicular chain is reconstructed using bone, cartilage, or prosthetic material. Hearing results are generally good if the stapes superstructure can be spared. Conductive hearing losses usually result if only the footplate of the stapes is left [61].

Advanced cholesteatoma — When a cholesteatoma (congenital or acquired) has extended beyond the accessible confines of the middle ear, middle ear exploration is usually inadequate to permit complete removal. To reach the temporal bone spaces above and behind the middle ear, it is necessary to remove the overlying plates of cranial bone (figure 5).

The pneumatized space just superior to the middle ear is called the epitympanum or attic. The attic contains the head of the malleus and the body of the incus. When cholesteatoma enters the attic, it often encircles the ossicular heads and cannot be freed, even when visualized with a microscope or angled endoscope. In such cases, the overlying bone, called the scutum, must be drilled away for access (figure 5) [62]. The defect created by atticotomy is often reconstructed with cartilage from the pinna to prevent postoperative retraction of the TM.

Mastoid involvement — Cholesteatoma involving the mastoid is treated by mastoidectomy (the surgical removal of the mastoid cortical bone and underlying air cells to gain access to the cholesteatoma). There are several variations in surgical approach, but mastoidectomies generally are of two types, canal wall-up and canal wall-down mastoidectomy (the "canal" part of these terms refers to the posterior portion of the external auditory canal that separates the canal from the mastoid air cells):

●Canal wall-up surgery – Canal wall-up surgeries include complete, simple, or cortical mastoidectomy. These are closed-cavity procedures. The posterior portion of the external auditory canal is preserved. In such cases, the healed ear usually looks normal when examined with an otoscope (figure 6).

●Canal wall-down surgery – Canal wall-down surgeries include radical mastoidectomy and modified radical mastoidectomy. These are open-cavity procedures. The posterior portion of the external auditory canal is sacrificed. A common cavity (mastoid bowl) is created between the ear canal and mastoid and, often, the middle ear. Regular otolaryngology follow-up for cleaning of squamous debris from the mastoid bowl is required, usually for life (picture 18). Canal wall-down surgery usually includes enlargement of the soft tissue opening of the ear canal (meatoplasty) to facilitate cleaning. Cleaning of the mastoid bowl may be uncomfortable for young children. Older children and adults sometimes complain of discomfort and dizziness, even when the bowl is cleaned skillfully.

The canal wall-up approach is usually preferred when feasible [63]. On the other hand, canal wall-down surgery is a time-tested, safe alternative for children with large cholesteatomas or destruction of the ear canal, those who fail canal wall-up surgery, or those for whom regular follow-up cannot be assured. Without regular cleaning, a foul-smelling mastoid bowl may result as accumulated squamous debris becomes secondarily infected.

Surgical complications — In addition to the general risks of surgery (eg, bleeding, infection), the risks of ear surgery include hearing loss, facial nerve paralysis, cerebrospinal fluid leak, and TM perforation [9,29].

OUTCOME

●Recurrence – Recurrence is uncommon in children with small, encapsulated congenital cholesteatomas that are removed intact [9,29,64]. In other patients, the risk of recurrence ranges from approximately 10 to 60 percent, depending upon the extent of disease (ie, larger more extensive cholesteatomas have a higher recurrence risk) [6,29,50,65,66]. Lesions associated with ossicular or mastoid involvement are at particularly high risk of recurrence (up to 40 to 60 percent) [6]. Multiple recurrences are not uncommon is such patients and they may require multiple surgeries [67].

Other risk factors for recurrence include age <8 years at diagnosis, higher initial stage of disease, and poor Eustachian tube function [50,66,68-70].

The high risk of recurrence highlights the need for regular postoperative follow-up, as described below. (See 'Follow-up' below.)

●Hearing – Hearing is usually stable or improved if the stapes superstructure is spared [6,61,71]. Factors related to satisfactory hearing outcome include less ossicular damage and better preoperative hearing [61,72,73]. In one series of 349 patients, most of whom underwent canal wall-down mastoidectomy, hearing improved in 30 percent (mean gain 5 decibels), remained unchanged in 55 percent, and worsened in 15 percent at a mean follow-up of 7.3 years; 30 percent of ears had a hearing threshold of 30 decibels or better [72].

●Cranial nerve palsies – Among patients who present with cranial nerve palsies, outcome is related to timeliness of diagnosis [74]. Recovery of function is possible with early intervention.

FOLLOW-UP — Children who have undergone treatment for cholesteatoma require regular follow-up postoperatively to prevent and control recurrent disease [29].

Children treated with excision and grafting require careful monitoring for at least two years after surgery. Even tiny fragments of cholesteatoma matrix can grow into a cholesteatoma (residual cholesteatoma) [68]. In addition, the same forces that caused the first cholesteatoma to form can cause the grafted tympanic membrane (TM) to collapse, predisposing to the development of recurrent cholesteatoma [75].

Clinical manifestations of residual/recurrent cholesteatoma include chronic otorrhea and/or perforation of the TM [72]. (See 'Presentation' above.)

Follow-up evaluation includes:

●Careful visualization of the ear under the operating microscope

●Annual audiograms

●Imaging, if recurrence is suspected

In addition, children who are treated with canal wall-down surgery require regular follow-up for cleaning of squamous debris from the mastoid bowl. (See 'Mastoid involvement' above.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Acute otitis media, otitis media with effusion, and external otitis".)

SUMMARY AND RECOMMENDATIONS

●Definition and predisposing factors – Cholesteatomas are abnormal accumulations of squamous epithelium within the middle ear and mastoid. They can be acquired or congenital. Predisposing factors for acquired cholesteatoma include recurrent acute otitis media and/or chronic middle ear effusions, family history of chronic middle ear disease and/or cholesteatoma, certain syndromes (eg, Down syndrome, Turner syndrome), and congenital anomalies (eg, cleft palate, craniofacial anomalies). (See 'Terminology' above and 'Epidemiology' above.)

●Presentation – Chronic ear drainage is the most common presenting symptom for acquired cholesteatomas. Most affected children have a history of recurrent acute otitis media and/or chronic middle ear effusions. Cholesteatomas also may come to attention based upon abnormal otoscopic findings on routine physical examination or abnormal routine hearing screening. Rarely, children with cholesteatoma present with a late complication (eg, facial nerve palsy). (See 'Presentation' above.)  

●Otoscopic findings – Characteristic otoscopic findings of cholesteatoma include (see 'Otoscopic findings' above):

•A white mass behind an intact tympanic membrane (TM) (picture 1A and picture 1B and picture 1C)

•A deep retraction pocket (picture 5)

•Focal granulation on the surface of the TM especially at the periphery (picture 6)

●Complications – If not surgically excised, cholesteatomas will continue to enlarge and can cause complications including hearing loss, cranial nerve palsies, vertigo, and serious infections (eg, meningitis, brain abscess). Early detection, which requires careful otoscopic examination at routine health maintenance visits, is important to reduce the risk of these complications. (See 'Clinical features' above and 'Clinical suspicion' above.)

●Diagnosis – The diagnosis of cholesteatoma is confirmed by the otolaryngologist using visual examination and palpation under an operating microscope, high-resolution computed tomography of the temporal bone, diffusion-weighted magnetic resonance imaging, and/or surgical exploration. (See 'Diagnosis' above.)

●Differential diagnosis – The differential diagnosis of cholesteatoma includes other white lesions of the TM:

•Myringosclerosis (picture 11)

•Foreign body (picture 12)

•Exostoses (outgrowths of bone into the external ear canal) (picture 13)

•Prosthetic and graft material in operated ears

•Inclusion cysts of TM (picture 14)

•Occasionally, a bulging acute otitis media may be hard to differentiate from a large congenital cholesteatoma (picture 15)

●Treatment – Cholesteatomas are treated surgically. The goals of surgery are eradication of the keratinizing squamous epithelium from the temporal bone and preservation or restoration of hearing. The extent of surgery depends upon the size and stage of the cholesteatoma. (See 'Surgical treatment' above.)

●Recurrence risk – Recurrence is uncommon in children with small congenital cholesteatomas that are successfully removed. In other patients, the risk of recurrence ranges from approximately 10 to 60 percent, depending upon the extent of disease. Lesions associated with ossicular or mastoid involvement are at particularly high risk of recurrence. Signs of recurrence include chronic drainage and/or perforation of the TM. (See 'Outcome' above.)

●Follow-up – Children require regular follow-up postoperatively to monitor for complications and to prevent and control recurrent disease. Follow-up includes annual audiograms, careful visualization of the ear under the operating microscope, and imaging if recurrence is suspected. (See 'Follow-up' above.)