Osteoma with cholesteatoma of the external auditory canal: neck manifestation of this rare association
A 24-year-old female with Rett syndrome developed recurrent unilateral neck abscesses over a 6-month period.\nRett syndrome is a severe neurodevelopmental disorder characterized by the progressive loss of intellectual functioning, fine and gross motor skills and communicative abilities, deceleration of head growth and the development of stereotypic hand movements, occurring after a period of normal development [].\nAt initial consult, a 2 cm abcess overlying the left mastoid tip and a bony swelling arising from the EAC with overlying normal skin (Fig. ) obstructing the view of the left ear canal and tympanic membrane was noted. Following incision and drainage of the abscess in theatre, the patient returned 2 weeks later with a self-draining left neck wound. As she was otherwise systemically well, the patient was treated as an outpatient with a course of oral antibiotics and listed for excision of the EAC osteoma and exploration of her left mastoid cavity. Due to the patient's global learning disabilities, audiological assessment and imaging of her ears were only possible under general anaesthesia. A combined decision was taken to proceed to surgical exploration, as it was felt that these adjunctive tests will not impact on her immediate management and would subject the patient to an additional anaesthetic challenge.\nAt surgery, transmeatal exicision of the EAC osteoma revealed an underlying cholesteatoma that has caused bony erosion of the inferior canal wall (Fig. ) and formed a fistulous connection to the overlying neck skin (Fig. ). The post-aural sinus and fistula were excised through a post-auricular incision. A cortical mastoidectomy was performed to explore the mastoid cavity, which was found to be disease free. The temporalis fascia was harvested and used to reconstruct the inferior canal wall defect. Histopathologic examination of the excised tissues confirmed the synchronous dual pathology involving the EAC (Figs and ).\nThe patient's symptoms settled after surgery and the neck abscess healed completely. There was no clinically detectable neck disease or cholesteatoma formation in the left ear canal at her 12-month clinic review.