Approach
Patients suspected of having cholesteatoma should be referred to an ear, nose, and throat surgeon on a semi-urgent basis. Infants and children should be referred to a paediatric otolaryngologist or a neurotologist with paediatric experience.[39] If facial palsy is present with suspected cholesteatoma, those patients should be referred urgently because early treatment is associated with better outcomes, and treatment delays can result in a poorer prognosis.[22][32][33]
The definitive treatment of cholesteatoma is surgery. The main goal is to remove the disease; provide a dry, safe ear that does not discharge; and prevent potential complications. Treatment is also aimed at improving the hearing threshold. This may not always be possible at initial surgery but may be possible following a second procedure.
There are different surgical approaches: the canal wall up mastoidectomy or the canal wall down mastoidectomy. There are no randomised controlled trials comparing these approaches.
Microscopic and endoscopic techniques are both in use. One systematic review showed that rates of residual and recurrent disease were lower with endoscopic ear surgery for middle ear cholesteatoma.[40] There was insufficient evidence, however, that operative time, uptake of the graft, or hearing results were improved with endoscopic versus microscopic techniques. Utilisation of high definition visualisation, such as a 4K magnification endoscope and narrow band imaging filter, has been shown to improve visualisation of tissue based on varying degrees of vascularity; this allows for better differentiation between pathology and normal anatomy, which may reduce risk of disease recurrence after surgery.[41]
Pre-surgical care
On initial presentation, prior to definitive surgical treatment, it may be appropriate to treat the aural discharge with topical antibiotics. Agents containing quinolone (e.g., ciprofloxacin and ofloxacin) are effective in both adults and children, either alone or in combination with a topical corticosteroid.[1] Aural cleaning may also reduce symptomatic discharge. The ear canal may need to be cleaned of any debris or wax prior to the use of topical ear drops.
Patients who have severe swelling of the ear canal may have difficulty applying ear drops. A wick should be inserted in the ear canal to allow for drug delivery. In some patients, debridement of granulation tissue may be necessary.
Canal wall up mastoidectomy (combined approach tympanoplasty)
This procedure allows removal of cholesteatoma but leaves the canal wall intact. It involves removal of the mastoid air cells lateral to the facial nerve and otic capsule, leaving the posterior and superior parts of the external canal wall intact.[42] It is often preferred for children, as it avoids the long-term complications of a mastoid cavity. However, it necessitates a second-look procedure after 9 to 12 months to examine for residual/recurrent disease. A meta-analysis found an increased incidence of postoperative cholesteatoma when using an intact wall approach rather than canal wall down approach.[43]
Alternatively, a non-echo-planar diffusion-weighted MRI may be used in some patients to check for recurrence.[28][29][30] There continues to be debate regarding which type of MRI is best to examine for recurrent cholesteatoma. Some authors advocate routine MRI scans (such as non-echo-planar, fast-spin echo, or diffusion weighted sequences) for follow-up, but caution that a negative scan may not be completely accurate as residual or recurrent disease may not yet be detectable. Non-echo-planar imaging was found to be more reliable compared with echo-planar imaging in identifying residual/recurrent cholesteatoma in one systematic review.[44] Another systematic review found that non-echo-planar diffusion-weighted MRI is highly sensitive and specific in identifying middle-ear cholesteatoma.[30] Patients will require continued follow-up.[45][46][47]
Canal wall down mastoidectomy
This procedure aims to remove the disease by drilling from the attic wall posteriorly. The size of the resultant cavity will depend on the extent of the cholesteatoma. A less invasive procedure resulting in a minimal cavity is called an atticotomy or atticoantrostomy; a more invasive procedure resulting in a larger cavity is called a modified radical mastoidectomy. A canal wall down procedure may allow direct examination of the cavity for recurrence, but if the attic has been reconstructed, a second-look procedure or a non-echo-planar diffusion-weighted MRI may be necessary to examine the middle ear for recurrent disease.
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